Other rare kidney tumors

1. Nephroblastomatosis

Summarize

Nephroblastomatosis, which is just one word away from nephroblastoma, is a different disease. The two diseases are somewhat related, but nephroblastomatosis is primarily born in much younger infants and toddlers, and the vast majority of the time it is a benign disease.

Nephroblastomatosis is a rare precursor tumor of nephroblastoma, which, as mentioned before, filters blood, mainly through "renal units". The term "renal unit" generally refers to the renal corpuscle (glomerulus + capsule) + tubule. Each of these units can be considered as a workshop for filtering blood wastes.

During the first 36 weeks of a mother's pregnancy, they develop from a mass of tissue called the "posterior renal blastoderm". Normally, they disappear after 36 weeks of pregnancy. If, for whatever reason, it doesn't disappear, these clusters are called nephrogenic renal remnants. These nephrogenic renal remnants are called nephroblastomatosis and may malignantly transform into nephroblastoma.

Epidemiological

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Etiolog & Risk factors

The risk of developing nephroblastomatosis is also associated with the following diseases:

:: WAGR syndrome (manifested by aniridia, genitourinary abnormalities and mental retardation)

:: Beckwith-Wiedemann syndrome (characterized by abnormal enlargement of one side of the body or part of the body, large tongue, umbilical hernia at birth and genitourinary abnormalities)

:: Isolated lateralized hyperplasia (manifested by abnormally large size on one side of the body or part of the body)

Classification & Stage

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Clinical manifestations

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Clinical Department

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Examination & Diagnosis

diagnostic criteria

The diagnostic criteria for nephroblastomatosis are:

● Multiple abnormal foci of nephrogenic rests (nephrogenic renal remnants) or persistent metanephric blastema (metanephric blastema) in the fetus after 36 weeks of gestation;

● Nephrogenic renal remnants are categorized into two types depending on where they are located: peripheral renal lobe remnants (90% of cases) versus intralobular remnants (10% of cases), the latter being more associated with nephroblastoma.

Screening for nephroblastomatosis is recommended every 3 months to detect conversion to malignancy. Ultrasound can detect the mass, requires no anesthesia, and is relatively inexpensive. However, magnetic resonance is more accurate and is usually used for follow-up.

routine checkup

Doctors make the initial diagnosis through kidney and blood tests, including a thorough physical examination, history taking, complete blood count, blood biochemistry, kidney function tests, and urinalysis. These tests help doctors determine the child's general condition and kidney function.

When doctors suspect the presence of a kidney tumor in a child, imaging is often used to determine the location of the lesion and whether it has spread. Commonly used diagnostic imaging modalities include ultrasound, chest and abdominal X-rays, CT, dynamic CT scans, magnetic resonance imaging (MRI), and PET-CT. Specific further confirmation of the diagnosis needs to be combined with pathological examination of the tumor tissue.

In imaging presentation, renal lymphoma resembles nephroblastomatosis, but the former is rarely seen in infants and young children.

Clinical Management

Current treatment options are controversial. Some researchers recommend chemotherapy, while others believe in close continuous radiation therapy for enlarged masses.

1.4.1 Radiotherapy

There are two types of radiation therapy: external radiation therapy and internal radiation therapy.

Exactly which form of radiotherapy is used depends on the type and stage of cancer suffered and whether a biopsy has been performed prior to surgery.

1.4.2 Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy (see Glossary for more information). Treatment is usually with combination chemotherapy, which involves the use of two or more anticancer drugs.

Prognosis

Nephroblastomatosis is usually considered a benign condition but carries a risk of malignancy. Nephrogenic renal remnants are present in about 1% of infants, and less than 1% of these develop malignancy.

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References

1. Sethi AT, Narla LD, Fitch SJ, Frable WJ. Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics. 2010;30 (5):1421-5.
2. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5345535/
3. https://radiopaedia.org/articles/nephroblastomatosis
4. https://www.medicinenet.com/script/main/art.asp?articlekey=26379

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2. Multi-atrial cystic nephroma

Summarize

Multilocular Cystic Nephroma (MCCN) is a very rare renal tumor that is usually benign and tends to present as a solitary tumor, but may occur in both kidneys at the same time, commonly in the upper part of the kidney.

The disease is most common in male children under 2 years of age or in middle-aged or elderly women.

Epidemiological

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Etiolog & Risk factors

The development of multilocular cystic nephromas is associated with mutations in a gene called DICER1.

It is worth noting that patients with multilocular cystic nephromas often require concomitant chest radiographs to rule out pleuropulmonary blastoma because cystic nephromas and pleuropulmonary blastomas share similar loss-of-function and site-specific mutations in DICER1.

Classification & Stage

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Clinical manifestations

Patients with multilocular cystic nephroma may not always have clinically significant symptoms. In pediatric patients, abdominal or flank masses are the most common, and may be accompanied by nonspecific signs or symptoms, such as hematuria, urinary tract infections, high blood pressure, fever, or other physical complaints.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

Renal ultrasound is often the first step in the diagnosis, and if a multilocular cystic nephroma is indeed present, it can be found in the ultrasound findings, which show a unilateral mass of the kidney with irregular cysts and septa of variable thickness. In addition, common imaging tests include CT and MRI.

However, on imaging, multilocular cystic nephroma shows multilocular cystic features but does not accurately differentiate it from other complex cystic renal masses and thus is not specific. Therefore, the diagnosis of this disease relies on pathologic examination with the following diagnostic criteria:

● The tumor consists entirely of a cyst and its septum;

● A cystic nephroma is a discrete well separated mass;

● The septum is the only solid component of the tumor and conforms to the contours of the cyst without dilated nodules;

● Pathology shows that the cyst consists of an arrangement of flat epithelial cells, cuboidal epithelial cells, and nail epithelial cells;

● Pathology shows fibrous tissue in the septum and possibly well-differentiated renal tubules.

In addition, since multilocular cystic nephroma shares the same genetic mutation as pleuropulmonary blastoma, and thus children with this tumor may also have pleuropulmonary blastoma, imaging of the lungs for cysts or solid tumors may be performed. Also, multilocular cystic nephroma is a heritable disease, so genetic counseling and genetic testing may be considered.

2. Other routine examinations

Clinical Management

As a benign tumor, multilocular cystic nephroma can be cured by surgery without the need for adjuvant other treatments (e.g. radiotherapy, chemotherapy, etc.). Currently, total nephrectomy is the most common surgical procedure, but for tumors smaller than 40mm, partial nephrectomy with some preservation of renal function is an option.

Prognosis

Multilocular cystic nephroma is a benign tumor with a good prognosis.

Follow-up & Review

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Cutting-edge Therapeutic & Clinical Research

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References

1. https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq (Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)) PDQ® -Patient Version)
2. https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq (Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)-Patient Version) Health Professional Version)
3. Granja MF et al.: Multilocular Cystic Nephroma A Systematic Literature Review of the Radiologic and Clinical Findings. ajr Am J Roentgenol. 2015 Dec;. 205(6):1188-93.
4. Dong B et al.: Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: a case report. Can Urol Assoc J. 2014 Jul;8(7-8):E545 -7.
5. Ozturk H, Karaaslan S.: Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant. Int J Clin Exp Pathol. 2014 Jul 15;7(8):5271-9.

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3. Cystic partially differentiated nephroblastoma

Summarize

Cystic Partially Differentiated Nephroblastoma (CPDN) is a rare cystic variant of nephroblastoma. CPDN is a rare cystic variant of nephroblastoma, which often presents as a painless abdominal mass and occurs more frequently in infants and children under 2 years of age, and is more common in males than females.CPDN has unique pathologic and clinical features, and pathological findings are the diagnostic criterion for the disease.The mainstay of treatment for CPDN is surgery. The prognosis of the disease is good, but there is a tendency of aggressiveness and malignancy.

Epidemiological

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Etiolog & Risk factors

Most cases of cystic partially differentiated nephroblastoma, a type of variant nephroblastoma, have no family history or other known cause. In a few cases, a genetic mutation puts the child at increased risk for the disease. Unlike multilocular cystic nephroma, CPDN is often free of mutations at the DICER1 locus, but CPDN is often associated with chromosome 12-chromosome trisomy.

Classification & Stage

CPDN staging is the same as for nephroblastoma, see:" Clinical Staging of Nephroblastoma "

Clinical manifestations

Common clinical manifestations of CPDN are abdominal mass, painless hematuria is uncommon

Clinical Department

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Examination & Diagnosis

1 Diagnostic strategy

The imaging findings of this disease are only suggestive and cannot be differentiated from other renal tumors, and a pathological diagnosis is the basis for a definitive diagnosis of CPDN.

Diagnostic criteria for this disease include a well-defined multicystic mass, the presence of a thin septum whose septal portion is the only solid portion of the tumor, and the absence of a distended mass or solid areas within the tumor. The septum consists of blastocytes and may contain variable numbers of embryonic mesenchymal or epithelial cells. This pathologic feature distinguishes this tumor from standard nephroblastoma.

CPDN is very close to presentations including cystic mesodermal nephroma, cystic renal dysplasia, cystic nephroma (CN) and Wilms' tumor with cystic changes. These differential diagnoses are important due to differences in treatment and need to be confirmed by histopathology.

2 Other routine examinations

Clinical Management

The standard treatment for CPDN is based on surgery. Stage I patients have a 100% survival rate after undergoing tumor resection for nephron preservation alone. Combined chemotherapy with vincristine and cenomycin after tumor resection in patients with stage II disease has shown good efficacy.

Prognosis

Cystic partially differentiated nephroblasts are usually benign and have a good prognosis. However, they have a malignant tendency to recur after surgery because of the presence of embryonic cells. Incomplete resection of the tumor or spillage from tumor breakage during surgery can lead to recurrence after tumor removal.

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References

References

1. http://www.pathologyoutlines.com/topic/kidneytumorcysticnephroblastoma.html. Accessed June 27th, 2018.
2. https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq#section/_150
3. Blakely, Martin L et al. Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy. Journal of Pediatric Surgery , Volume 38 , Issue 6 , 897 - 900
4. Luithle, Tobias et al. Treatment of Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma-A Report From the SIOP/GPOH Study Group. The Journal of Urology , Volume 177 , Issue 1 , 294 - 296

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4. Renal cell carcinoma

Summarize

Basic Information

Renal Cell Carcinoma (also known as kidney cancer or renal cell adenocarcinoma) is a disease that occurs in the tubules of the kidneys.

Renal cell carcinoma is the most common kidney tumor in adults, accounting for about 85% of cases, but it is rare in children under 15 years of age and more common in adolescents 15 to 19 years of age. Children The development of renal cell carcinoma is associated with other genetically inherited conditions. Common symptoms include hematuria, abdominal mass, abdominal pain, and weight loss. Renal cell tumors can metastasize to the lungs, bones, liver, and lymph nodes. Treatment and prognosis are related to the stage of the disease

Epidemiological

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Etiolog & Risk factors

Etiology and Risk Factors

The exact cause of renal cell carcinoma is unknown and is generally thought to be related to the accumulation of DNA mutations, which may be inherited or acquired. A number of risk factors are thought to increase the risk of renal cell carcinoma, but it is not clear whether these factors contribute to renal cell carcinoma. These factors include:

● Family history of renal cell carcinoma;

● Certain genetic conditions, such as Von Hippel-Lindau disease (a genetic disorder that causes abnormal growth of blood vessels), tuberous sclerosis (an inherited disease marked by noncancerous fat cysts in the kidneys), adult neuronal neoplasms, and sickle cell disease;

● Smoking;

:: Prolonged abuse of certain painkillers, including over-the-counter painkillers;

:: Overweight;

● Hypertension.

Classification & Stage

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After a diagnosis of renal cell carcinoma is made, follow-up tests are performed to determine if the cancer has spread within the kidney or to other parts of the body, a process also known as staging, which is done by methods that include CT scans, magnetic resonance imaging, chest X-rays, and bone scans.

Renal cell carcinoma is staged as follows:

● Stage I: Tumor is 7 cm or smaller and is seen only in the kidneys;

● Stage II: Tumor greater than 7 cm, seen only in the kidneys;

● Stage III: A tumor in the kidney can be of any size but has spread to: a) nearby lymph nodes, or b) has spread to nearby blood vessels in the kidney (renal vein or vena cava), or c) has spread to the renal structures that collect urine, or has spread to d) layers of adipose tissue around the kidney;

● Stage IV: The cancer has spread:

a) exceeds the layer of adipose tissue surrounding the kidney and may have spread to the adrenal gland above the kidney, or

b) Spread to other parts of the body, such as the brain, lungs, liver, adrenal glands, bones, or distant lymph nodes.

Clinical manifestations

● Hematuria;

:: Abdominal mass;

:: Constant pain on the side of the body;

● Loss of appetite;

:: Unexplained weight loss;

:: Anemia.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

Definitive diagnosis of the disease requires a combination of imaging and pathology of the tumor tissue. Commonly used diagnostic imaging modalities include ultrasound, chest and abdominal X-rays, CT, dynamic CT scans, magnetic resonance imaging (MRI), and PET-CT.

2. Other routine examinations

Clinical Management

treatment plan

There are different treatment options for renal cell carcinoma depending on the stage and type of disease, including standard treatment and clinical trials.

4.6.1 Standard treatment

There are currently five standard treatments for renal cell carcinoma, including surgery, radiation therapy, chemotherapy, biologic therapy, and targeted therapy.

(1) Surgery

:: Partial nephrectomy: removal of the cancer in the kidney and some of the tissue around it, which can be done to prevent loss of kidney function when the kidney has been damaged or has already been removed;

:: Simple nephrectomy: a surgical procedure in which only the kidney is removed;

:: Radical nephrectomy: a surgical procedure that removes the kidneys, adrenal glands, surrounding tissues, and nearby lymph nodes.

(2) Radiation therapy

There are two types of radiation therapy: external radiation therapy and internal radiation therapy.

(3) Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy. The type of chemotherapy depends on the type and stage of cancer being treated.

(4) Biotherapy

The following biologic treatments are being used or studied to treat renal cell carcinoma:

● Nivolumab: Nivolumab is a monoclonal antibody that enhances the body's immune response to renal cell carcinoma cells;

:: Interferon: Interferon affects the division of cancer cells and can slow tumor growth;

● Interleukin-2 (IL-2): IL-2 promotes the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells.

(5) Targeted therapy

Antiangiogenesis-targeted therapies stop the formation of blood vessels in the tumor, causing the tumor to stop growing or shrinking, and are used to treat advanced renal cell carcinoma. Antiangiogenic agents include monoclonal antibodies and kinase inhibitors:

:: Monoclonal antibodies: Laboratory-made antibodies that come from a single type of immune system cell. These antibodies recognize substances on cancer cells or substances that help cancer cells grow. The antibody attaches to the substance and kills the cancer cell, stops it from growing, or stops it from spreading. Monoclonal antibodies are usually given by infusion.

● Kinase inhibitor: stops cells from dividing and may stop the growth of new blood vessels in tumors. m TOR inhibitors are a type of kinase inhibitor. Everolimus and temsirolimus are two mTOR inhibitors used to treat advanced renal cell carcinoma.

4.6.2 Treatment Options for Patients with Different Stages of Staging

● Treatment of stage I renal cell carcinoma :

- Surgery (radical nephrectomy, simple nephrectomy or partial nephrectomy);

- Radiation therapy is used as palliative care to relieve symptoms in inoperable patients;

- Arterial embolization as palliative care;
- A clinical trial.

● Treatment of stage II renal cell carcinoma:

- Surgery (radical nephrectomy or partial nephrectomy);
- Surgery (nephrectomy), before or after radiation therapy;
- Radiation therapy as palliative care to relieve symptoms in inoperable patients;
- Arterial embolization as palliative care;
- Clinical Trials.

● Treatment of stage III renal cell carcinoma:

- Surgery (radical nephrectomy) with possible removal of blood vessels to the kidney and some lymph nodes;
- Artery embolization followed by surgery (radical nephrectomy);
- Radiation therapy as palliative care to relieve symptoms and improve quality of life;
- Artery embolization as palliative care;
- Surgery (nephrectomy) as palliative care;
- Radiation therapy before and after surgery;
- Clinical trials of post-surgical biologic therapy.

● Treatment of stage IV and recurrent renal cell carcinoma may include:

- Surgery (radical nephrectomy);
- Surgery (nephrectomy) to reduce the size of the tumor;
- Targeted therapy;
- Biological therapy;
- Radiation therapy as palliative care to relieve symptoms and improve quality of life;
- Clinical trials.

Prognosis

The prognosis and treatment options for renal cell carcinoma depend on the stage of the disease as well as the age and general health of the patient.

Follow-up & Review

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clinical trial

For more information, please refer to the Clinical Trials section of Renal and Kidney Tumor Basics.
Clinical trials for pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. american society of clinical oncology. kidney cancer: introduction. 2018.
2. Kidney Cancer UK. childhood kidney cancer. 2018.
3. National Cancer Institute. Renal Cell Cancer Treatment. 2018.

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5. Congenital mesodermal nephroma

Summarize

Basic Information

Congenital Mesoblastic Nephroma (CMN) is a rare, low malignancy risk tumor that accounts for 3% to 5% of renal tumors in children, and is the most common renal tumor in infants within the first month of life. The median age at diagnosis of patients is 0 to 2.9 months. 16% of affected children are identified during prenatal screening. The male to female sex ratio is 1.5:1.Children tend to present with an abdominal mass or hematuria.The prognosis of the disease is related to the stage of the tumor.

Epidemiological

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Etiolog & Risk factors

A common cause of the disease is genetic defects, and gene mutations commonly found in patients include somatic chromosome 11 trisomy, ETV6, and NTRK3 gene fusions.

Classification & Stage

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Congenital mesodermal nephroma cancer cells can spread through tissues, the lymphatic system, and the bloodstream. Doctors often use lymph node biopsies and imaging tests to find out if the cancer has spread to parts of the body other than the kidneys. Staging is important and determines how treatment is given.

Congenital mesodermal nephroma is staged as follows:

● Stage I: Tumor is 7 cm or smaller and is seen only in the kidneys;

● Stage II: Tumor greater than 7 cm, seen only in the kidneys;

● Stage IV: The cancer has spread a) beyond the layer of fatty tissue surrounding the kidney and may have spread to the adrenal gland above the kidney, or b) to other parts of the body, such as the brain, lungs, liver, adrenal glands, bone, or distant lymph nodes.

Most congenital mesodermal nephromas are found to be stage I/II tumors, and a few are stage III

Clinical manifestations

Common clinical manifestations include abdominal mass, hypertension, hematuria, hyperglycemia, hypercalcemia, and hyperreninemia. Most children present with an abdominal mass or hematuria, and the symptoms of hypercalcemia and hyperreninemia disappear completely after removal of the tumor.

Clinical Department

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Examination & Diagnosis

1 Diagnostic Strategies

Most of the disease is initially diagnosed using imaging tests, but the final diagnosis needs to be combined with the pathological findings of the tumor tissue. Commonly used diagnostic imaging modalities include ultrasound, chest and abdominal radiographs, CT, dynamic CT scan, magnetic resonance imaging (MRI), and PET-CT.

2 Other routine examinations

Clinical Management

Standard treatment

Most patients with congenital mesangial nephroma have localized lesions, and standard treatment consists of total nephrectomy, with adjuvant chemotherapy or radiotherapy also used in infants older than 3 months of age.

(1) Surgery

Two types of surgery are included: total nephrectomy and partial nephrectomy.

Some patients may receive chemotherapy or radiation therapy after surgery to kill all the cancer cells. Treatment given after surgery, called adjuvant therapy, is used to reduce the risk of the cancer coming back. Sometimes, a second surgery is needed with the goal of seeing if the cancer cells remain after chemotherapy or radiation.

(2) Radiation therapy

Exactly which form of radiation therapy is used depends on the type and stage of the cancer in question and whether or not a biopsy has been performed prior to surgery. External radiation therapy is usually used to treat nephroblastoma.

(3) Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy.

Treatment is usually with combination chemotherapy, which involves the use of two or more anti-cancer drugs. The type of chemotherapy depends on the type and stage of cancer being treated.

Prognosis

Prognosis and follow-up

The outcome of the disease and survival after treatment are collectively referred to as prognosis. The prognosis of the disease is related to the specific tumor type, stage, treatment regimen, and individual response to specific treatments.

In a review of 200 CMN research papers (270 cases in total), all patients underwent complete or partial renal removal, with a 5-year survival rate of approximately 95% and recurrence in 4% of patients. The relationship between subtype, stage, and prognosis is shown in the following table (in units of 1).

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References

References

1. Furtwaengler R, Reinhard H, Leuschner I, Schenk JP, Goebel U, Claviez A, et al. Mesoblastic nephroma-a report from the Gesellschaft fur P ädiatrische Onkologie und Hämatologie (GPOH). Cancer. 2006;106(10):2275-83.
2. Gooskens S, Houwing M, Vujanic G, Dome J, Diertens T, Coulomb-l'Herminé A, et al. Congenital mesoblastic nephroma 50 years after its recognition: a narrative review. pediatric blood & cancer. 2017.
3. pathology Outlines. congenital mesoblastic nephroma 2018.

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6. Clear cell sarcoma of the kidney

Summarize

Basic Information

Cell Clear Sarcoma of the Kidney (CCSK) is a rare malignant tumor. The age at diagnosis ranges from 2 months to 14 years, with the highest incidence between 2 and 3 years of age, decreasing sharply in children over 3 years of age, and rare in infants under 6 months of age and young adults. The male to female ratio of incidence is about 2:1.

Renal clear cell sarcoma is aggressive and the most common site of metastasis is the bone, followed by the lungs, abdomen, retroperitoneum, brain and liver. Common symptoms are abdominal mass and abdominal pain. The prognosis of the disease is related to the stage.

Epidemiological

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Etiolog & Risk factors

The etiology of renal clear cell sarcoma is unclear. It does not appear to be associated with nephroblastoma, and no specific chromosomal translocations associated with renal clear cell sarcoma have been identified.

Classification & Stage

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Kidney-type clear cell sarcoma cells can spread through tissues, the lymphatic system, and the blood. Staging is used to find out if the cancer has spread to parts of the body other than the kidneys. Staging is important and determines how treatment will be given. Staging usually involves a lymph node biopsy and imaging tests.

Staging of clear cell sarcoma of the kidney is the same as staging of nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

Patients with renal clear cell sarcoma present similarly to patients with nephroblastoma. Common symptoms include an abdominal mass, abdominal pain, which may be accompanied by hematuria of the naked eye, fever, and high blood pressure. Often, parents seek medical attention when they notice abdominal swelling or an abdominal mass while bathing or changing their child's clothes.

Clinical Department

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Examination & Diagnosis

1 Diagnostic strategy

The diagnosis of renal clear cell sarcoma relies primarily on imaging tests. Imaging tests are important in the diagnosis and staging of the disease and include urography, ultrasonography, and CT of the chest and abdomen.Pathologic biopsy is the definitive diagnostic tool for the disease.

Also, because renal vein tumor thrombosis is present in approximately 5% of patients with renal clear cell sarcoma, abdominal ultrasound should be performed to assess the status of the inferior vena cava and renal veins.

:: Abdominal ultrasound (ultrasound): in clear cell sarcoma of the kidney, ultrasound may show a solid mass in the renal parenchyma with an irregularly echogenic border and a heterogeneous, unevenly elevated internal echogenicity. The entire renal shape is distorted, and there is a localized mass protruding beyond the normal field contour.

● CT scan or CAT (computerized axial tomography): in renal clear cell sarcoma, the plain scan shows localized elevation of the kidney, with uneven and low internal density, within which patches or small dots of calcification are seen, sometimes in the form of eggshells; enhancement scans show marked enhancement of the normal portion of the kidney, while enhancement within the carcinoma is low, with an irregular degree of internal enhancement, and necrotic zones of even lower density are present. The cancer may penetrate the peritoneum into the perinephric fat layer, and in the advanced stage, it may penetrate the renal fascia and spread to extrarenal tissues.

:: Pathologic examination: renal clear cell sarcoma usually presents as a large, unicentric mass that is markedly distorted or almost completely occupies the kidney. The tumor is commonly found in the renal medulla. Tumor sections are brownish-gray, soft, mucinous, and may contain discrete foci of necrosis and hemorrhage.

Histologically, renal clear cell sarcoma shows three components including:

(1) Sole cells, which are small round or oval cells with distinct cytological features

(2) Spacer cells, which are spindle-shaped cells spaced along the fiber vessels

(3) An intercellular matrix composed of mucopolysaccharides that range from tiny indistinguishable droplets to large pools that give renal clear cell sarcomas their well-defined appearance. Depending on the number, distribution, and morphologic variations of the 3 components, the tumor may present either a typical renal clear cell sarcoma or a variety of different histologic features.

2 Other routine examinations

Clinical Management

Treatment of renal clear cell sarcoma includes standard therapy and clinical trials.

Standard treatment

:: Surgical treatment

Two types of surgery are included: total nephrectomy and partial nephrectomy.

If the lesion is resectable, radical nephrectomy is the treatment of choice. If the size or extension of the lesion is uncertain, biopsy and chemotherapy are performed, and surgical resection is performed after chemotherapy has been effective.

:: Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy.

Patients with renal clear cell sarcoma may receive combination chemotherapy. The addition of doxorubicin to chemotherapy regimens has been shown to improve disease-free survival.

Prognosis

The prognosis of this disease is related to the specific tumor type, stage, treatment regimen, and individual response to specific treatments.

The 8-year recurrence-free survival rate for renal stage I-III localized clear cell sarcoma is 88%, but patients with advanced disease often recur after treatment.A subgroup of patients with stage I tumors in patients aged 2 to 4 years, and renal clear cell sarcoma not complicated by tumor necrosis, have a better prognosis.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

For more information, please refer to the Clinical Trials section of the Kidney and Kidney Tumor Basics.

Clinical trials in pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. https://emedicine.medscape.com/article/993245-overview
2. https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
3. https://www.kcuk.org.uk/kidneycancer/what-is-kidney-cancer/childhood-kidney-cancer/

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7. Renal Ewing sarcoma

Summarize

Basic Information

Primary neuroectodermal tumor /Ewing sarcoma of the kidney (formerly known as neuroepithelial tumor) is a type of Ewing sarcoma, a rare tumor type. Ewing sarcoma usually occurs in the bones and soft tissues of young people and rarely in the kidneys.

Renal Ewing sarcoma accounts for less than 1% of renal tumors. The tumor grows and spreads rapidly and is highly aggressive. Patients often present with localized pain, which may be accompanied by hematuria and large renal masses. The prognosis of this disease is related to the specific tumor type, staging, treatment options, and individual response to specific treatments.

Epidemiological

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Etiolog & Risk factors

Primary renal Ewing sarcoma is primarily a genetic disease, with most patients having a chromosomal translocation in which the EWS gene on chromosome 22 is fused to FLI1 on chromosome 11 t(11; 22)(q24; Q12), forming a powerful transcription factor. The source of the cells is unknown and is currently thought to be derived from neuronal cells and neural crest cells.

Classification & Stage

Tumor cells from this disease can spread through tissues, the lymphatic system, and the bloodstream. Staging is used to find out if the cancer has spread to parts of the body other than the kidneys. Staging is important and determines how treatment is given.

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

Patients often present with localized pain, usually involving the flanks, subcostal or abdominal area, which may be accompanied by hematuria and large kidney masses, and in a few cases, difficulty urinating, fever, weight loss, fatigue and abdominal swelling.

Clinical Department

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Examination & Diagnosis

1 Diagnostic strategy

The diagnosis of this disease is complex and relies heavily on imaging studies as well as pathologic findings to confirm the diagnosis. The disease requires differential diagnosis with a variety of tumors such as William's tumor, rhabdomyosarcoma, neuroblastoma, lymphoma, clear cell sarcoma, small cell carcinoma, synovial sarcoma, and connective tissue-promoting hyperplastic small round cell tumor. The presence of vascular thrombi has been reported as a unique feature of the disease.

Commonly used diagnostic imaging modalities include ultrasound, chest and abdominal x-rays, CT, dynamic CT scans, magnetic resonance imaging (MRI), and PET-CT.

The median diameter of the longest ESFT/PNET tumors was 13 cm. Histologically, the cells were small and rounded, and more than 80% of them expressed the following molecular markers: vimentin (82%), neuron specific enolase (88%), CD99 (99%), and in some cases FLI 1, PGP9.5, GFAP, CD57, and others. etc.

In situ hybridization and PCR testing revealed the presence of the EWS/FLI 1 gene translocation in 70% to 90% of patients.

2 Other routine examinations

Clinical Management

There is no standardized approach to the treatment of this disease, and there are no studies on the effectiveness of different treatment modalities on outcomes.

Previously used treatments include surgical resection and adjuvant chemotherapy with or without neoadjuvant chemotherapy and radiotherapy. Chemotherapy may be a combination of drugs such as doxorubicin, etoposide, isocyclophosphamide, vincristine or cyclophosphamide.

Standard treatment

:: Surgery

Two types of surgery are available depending on the progression of the disease: total nephrectomy and partial nephrectomy.

:: Radiotherapy

There are two types of radiation therapy: external radiation therapy and internal radiation therapy.

Exactly which form of radiotherapy is used depends on the type and stage of cancer suffered and whether a biopsy has been performed prior to surgery.

:: Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy.

The type of chemotherapy depends on the type and stage of cancer being treated.

Prognosis

The prognosis of this disease is related to the specific tumor type, stage, treatment regimen, and individual response to specific treatments.

The prognosis for patients who develop metastases from this disease is very poor, and patients with non-metastatic disease have a high rate of recurrence. The tumor has a high response rate to chemotherapy. The median disease-free survival is 5 months.

Patients diagnosed with metastatic disease (M1) or without metastatic disease (M0) had a 60% and 85% probability of survival at 18 months, respectively.The median overall survival for patients with stage M1 tumors was 24 months. Survival to 12 months was 93% in patients with M0 disease who received chemotherapy compared with 75% in patients who did not receive treatment. Among patients with treated M1 disease, the median progression-free survival was 22.0 months, with a clinical benefit rate of 74%.

Follow-up & Review

not have

Routine

not have

Cutting-edge Therapeutic & Clinical Research

For more information, please refer to the Clinical Trials section of the Kidney and Kidney Tumor Basics.

Clinical trials in pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5645000/
2. https://www.ncbi.nlm.nih.gov/pubmed/23800653
3. https://www.humanpathol.com/article/S0046-8177(06)00533-8/fulltext
4. https://www.goldjournal.net/article/S0090-4295(06)00272-X/fulltext

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8. Rhabdomyosarcoma-like tumor of the kidney

Summarize

Basic Information

Rhabdoid Tumor of the Kidney is a rare childhood tumor, with 20 to 25 new cases of the tumor identified each year in the United States. This tumor is most common in infants and young children, and the average age of diagnosis is 15 months.

Malignant rhabdomyosarcoma-like tumors typically occur in the kidneys, but can also occur in other soft tissues or in the brain, and those that occur in the brain are also known as atypical teratoid rhabdomyosarcoma-like tumors (ATRTs).

Rhabdomyosarcoma-like tumors of the kidneys are more malignant as they progress more rapidly and the tumors are very likely to spread rapidly after they appear, often metastasizing to the lungs or brain. The primary symptom in pediatric patients is one or more masses found in the child's abdomen. The prognosis and treatment options for the child depend on many factors.

Epidemiological

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Etiolog & Risk factors

The cause of rhabdomyosarcoma-like tumors is inconclusive. Mutations in the SMARCB1 gene (also known as Ini1, Snf5, and Baf47) are found in all rhabdomyosiform tumors, and such mutations sometimes occur in patients' normal non-tumor cells and increase the risk of patients developing rhabdomyosiform tumors in other locations.

Classification & Stage

Tumor cells can spread through tissues, the lymphatic system, and the bloodstream. Staging is used to find out if the cancer has spread to parts of the body other than the kidneys. Staging is important and determines how treatment is given.

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

Usually, the first sign that a child has a malignant rhabdomyosiform tumor is the discovery of one or more lumps in the child's abdomen. Since malignant rhabdomyosarcoma occurs most often in infants, the infant may not have any unusual signs of pain or discomfort. Additionally, some children with malignant rhabdomyosarcoma may experience difficulty urinating or hematuria.

Clinical Department

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Examination & Diagnosis

1 Diagnostic strategy

Your doctor will perform diagnostic tests for malignant rhabdomyosarcoma tumors such as imaging and pathology tests to confirm the diagnosis.

The doctor will also test to see if the tumor has spread to the brain. These tests include:

:: Children younger than 1 year old have an abdominal ultrasound every 2 to 3 months and a monthly head ultrasound;

● Abdominal ultrasound every 2 to 3 months and MRI of the brain and spine every 3 months for children aged 1 to 4 years.

Imaging

When a doctor suspects that a patient has a kidney tumor, imaging tests are often used to determine the location of the lesion and whether it has spread. Commonly used diagnostic imaging modalities include ultrasound (ultrasound), chest and abdominal x-rays, computed tomography (CT), dynamic CT scans, magnetic resonance imaging (MRI), and nuclear scans (PET-CT).

pathological examination

This is a more backward test, usually preceded by non-invasive or less invasive tests. When there are still unclear cases, the doctor will remove some cells or tissues from the child's body through surgery or minor operations, and a pathologist will make a specimen to look for signs of cancer under a microscope.

Biopsies can be taken before treatment, after chemotherapy, or after surgical removal.

Genetic counseling

For malignant rhabdomyosarcoma-like tumors, the tumor tissue can be tested to see if it contains mutations in SMARCB1 as described above, which generally results in this gene expression being turned off in all rhabdomyosarcoma-like tumors.

If a mutation is found, the doctor may recommend additional testing to determine if the mutation is a germline mutation, which means any detectable, heritable mutation that is in the germ cells to see if it can occur. Such mutations may be present throughout the patient's body and present and inherited throughout the family. If these results are positive, the doctor may recommend mutation testing or genetic counseling for other members of the patient's family.

2 Other routine examinations

Clinical Management

Treatment of this disease includes surgery, radiotherapy and chemotherapy, as well as a combination of methods. However, because this tumor is rare and progresses rapidly, there are no definitive standards of treatment. Treatment options are patient-specific. Prompt medical treatment and aggressive therapy are important for recovery after treatment.

1 Standard treatment

:: Surgical treatment

In most cases, the first step in treating a rhabdomyosarcoma-like tumor is surgery. Usually, the surgeon will attempt to remove the entire tumor at the time of the biopsy. Depending on the size and location of the tumor, the surgeon may remove only part of the kidney or the entire kidney.

:: Chemotherapy

After surgery, children with rhabdomyosarcoma-like tumors receive further chemotherapy with drugs that interfere with the growth and proliferation of cancer cells. Different groups of chemotherapy drugs work in different ways, and doctors often use a combination of chemotherapy drugs. Some chemotherapy drugs may also be given at different times of the cancer depending on the type of cancer they are used to treat.

Systemic chemotherapy in children with rhabdomyosarcoma-like tumors is often achieved by intramuscular or intravenous injection. Typically during the course of chemotherapy, children may experience adverse side effects. Understanding these side effects can help children, their parents, and medical staff prepare for and, in some cases, prevent these symptoms from occurring.

:: Radiotherapy

Children over 6 months of age with malignant rhabdomyosiform tumors may also receive radiation therapy as part of their treatment.

:: Stem cell transplantation therapy

Treatment for rhabdomyosarcoma often involves very high doses of chemotherapy to temporarily remove some of the child's healthy blood cells, as well as radiation therapy to destroy the rhabdomyosarcoma, and these treatments can strongly affect the child's body's ability to produce normal blood and immune cells. As a result, doctors may recommend that the child receive an autologous stem cell transplant. Once the radiotherapy is complete, the patient will be repopulated with previously stored healthy blood cells to promote normal body and organ function.

:: Supportive care

Supportive care is a way to prevent treatment infections, side effects of treatment and complications. The goal is to keep the child comfortable during treatment. It is also an important part of preventing short- and long-term complications of the disease and treatment.

Prognosis

In general, malignant rhabdomyosiform tumors are highly aggressive and difficult to treat. Children initially respond well to treatment, but recurrence during or shortly after treatment remains a risk. Children with germline mutations are also at high risk for secondary tumors. Prompt medical treatment and appropriate therapy often improve the prognosis.

Follow-up & Review

not have

Routine

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Cutting-edge Therapeutic & Clinical Research

For more information, please refer to the Clinical Trials section of the Kidney and Kidney Tumor Basics.

Clinical trials for pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. Dana-Farber Cancer Insititute. Rhabdoid Tumor
2. Jackson EM, Sievert AJ, Gai X, et al. (March 2009). "Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors". Clin. Cancer Res. 15 (6): 1923-30.
3. Dana-Farber & Boston Children's cancer and blood disorders center. Malignant Rhabdoid Tumor in Children

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9. Pro-connective tissue proliferative small round cell tumor

Summarize

Basic Information

Desmoplastic Small Round Cell Tumour (DSRCT) is a very rare and rapidly progressive soft tissue tumor. It affects men and women 4:1 and is usually found in the abdominal cavity, but may occur in other parts of the body. The tumor is formed by small, round cancer cells surrounded by scar-like connective tissue. The tumor may not be easily detected until it becomes larger. As a result, the disease has often spread to the liver, lymph nodes, lungs, or bones by the time it is diagnosed. The prognosis is poor because the disease can be misdiagnosed or detected at an advanced stage.

Epidemiological

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Etiolog & Risk factors

The pathogenic mechanism of the disease is not clear. It is generally believed that the tumor originates from primitive cells in infancy. It is associated with chromosomal translocations, and such translocations produce mutations that result in the failure of a transcription factor that inhibits tumor growth. Therefore, the diagnosis of this cancer also uses the presence or absence of a mutation in this gene as a secondary diagnostic modality.

Classification & Stage

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

The disease is often asymptomatic in the early stages, and an abdominal mass is only detected when the tumor becomes larger, making it easier to misdiagnose or ignore. Common symptoms include: abdominal mass, abdominal pain, cramps, nausea, vomiting, diarrhea, constipation, difficulty in defecation, abdominal swelling.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

If there are corresponding clinical symptoms, the doctor will further determine whether the patient has DSRCT and whether metastatic dissemination has occurred through imaging and pathological examination of tumor tissues.

Imaging

pathological examination

Biopsies can be taken before treatment, after chemotherapy, or after surgical removal.

2. Other routine examinations

Clinical Management

Because DSRCT is particularly rare, there is no standard treatment. The general treatment is as follows:

9.6.1 Surgery

It is used to remove as much of the cancer as possible. But usually the cancer has spread too far to be completely removed, but surgeons still try to remove at least 90 percent of it.

Meanwhile, hyperthermic intraperitoneal chemotherapy (HIPEC), also known as intraperitoneal heat infusion chemotherapy, strengthens the killing effect on cancer cells by combining heat and chemotherapy in a comprehensive way through the use of heated sterile chemical solution and the thermodynamic effect of heat therapy that can increase the efficacy of anticancer drugs. In addition, it also avoids the side effects brought by standard chemotherapy to patients.

9.6.2 Chemotherapy

Use powerful drugs to kill cancer cells or stop them from growing and making more.

Some chemotherapy drugs can be taken by mouth or used intravenously. In specific cases, doctors may also use methods that use more than one type of chemotherapy at a time.

9.6.3 Radiotherapy

DSRCT located outside the abdomen is less likely to show dissemination and is often easier to treat than DSRCT in the abdomen or DSRCT that has spread to other parts of the body.

Prognosis

Because the disease can be misdiagnosed or detected at an advanced stage, patients often begin treatment only when they develop extensive abdominal tumor growth that has metastasized to other parts of the body. As a result, the 5-year survival rate for patients with this tumor is only 15%.

Typically patients initially respond well to first-line chemotherapy, but relapse is common. Additionally it has been shown that some patients with remission or inoperable tumors can benefit from long-term low-dose chemotherapy, converting DSRCT into a chronic disease.

Follow-up & Review

not have

Routine

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Cutting-edge Therapeutic & Clinical Research

For more information, please refer to the Clinical Trials (link here) section of the Kidney and Kidney Tumor Basics.
Clinical trials for pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. St Jude Children's research hospital. Desmoplastic Small Round Cell Tumor
2. Memorial Salone Kettering Cancer Center. Desmoplastic Small Round Cell Tumors (DSRCT) Diagnosis/Treatment
3. Murphy AJ, Bishop K, Pereira C, et al. (December 2008). "A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity". Hum. Pathol. 39 (12): 1763-70.
4. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP (January 2005). "Results of multimodal treatment for desmoplastic small round cell tumors". J. Pediatr. Surg. 40 (1): 251-5.

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10. Primary renal synovial sarcoma

Summarize

Basic Information

Primary Renal Synovial Sarcoma (PRS) is very rare and is a subtype of embryonal sarcoma of the kidney. Primary renal synovial sarcoma consists of cystic structures derived from dilated, involved renal tubules. It is most commonly seen in young adults and common clinical manifestations include flank pain, abdominal mass, and hematuria. These tumors grow and spread rapidly and have a poor prognosis.

Epidemiological

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Etiolog & Risk factors

The patient's chromosome is characterized by the t(X;18)(p11;q11) SYT-SSX translocation. It is histologically similar to monophasic spindle cell synovial sarcoma and is considered an aggressive tumor with a poor prognostic profile in more than 50% of cases.

Classification & Stage

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

Common clinical manifestations include flank pain, abdominal mass, hematuria, and occasionally fever and fatigue.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

In general, there are no clinical or imaging features that can be used as the basis for a definitive preoperative diagnosis of primary renal synovial sarcoma, and the condition can only be diagnosed by pathologic examination. Positive expression results of synovial sarcoma cytokeratins, waveform proteins, bcl-2, and epithelial membrane antigens are usually used as the basis for diagnosis.

2. Other routine examinations

Clinical Management

The main treatments for renal synovial sarcoma include surgical resection and chemotherapy with isocyclophosphamide.

:: Surgery

Two types of surgery are available depending on the progression of the disease: total nephrectomy and partial nephrectomy (see for more information ).

:: Chemotherapy

Chemotherapy with isocyclophosphamide is commonly used for renal synovial sarcoma.

Prognosis

The prognosis of primary renal synovial sarcoma is unknown due to the limited number of reported cases. Previously published data suggest that renal synovial sarcomas tend to spread and have a poor prognosis.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

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References

References

1. B. A. Shannon, A. Murch, and R. J. Cohen, "Primary renal synovial sarcoma confirmed by cytogenetic analysis: a lesion distinct from sarcomatoid renal cell carcinoma," Archives of Pathology and Laboratory Medicine, vol. 129, no. 2, pp. 238-240, 2005.
2. Argani P, Faria PA, Epstein JI, et al.: Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol 24 (8): 1087-96, 2000.
3. Gulum, M., Yeni, E., Savas, M., Ozardali, I., Ozdemir, I., & Mil, D., et al. (2011). Primary renal synovial sarcoma. Case Reports in Urology, 2011, (2011-8-11), 2011(3), 11-2.
4. C. E. Kampe, G. Rosen, F. Eilber et al. "Synovial sarcoma: a study of intensive chemotherapy in 14 patients with localized disease. " Cancer, vol. 72, no. 7, pp. 2161-2169, 1993.
5. C. H. Schaal, F. C. Navarro, and F. A. Moraes Neto, "Primary renal sar- coma with morphologic and immunohistochemical aspects compatible with synovial sarcoma," International Braz J Urol, vol. 30, no. 3, pp. 210-213, 2004.
6. Schoolmeester JK, Cheville JC, Folpe AL: Synovial sarcoma of the kidney: a clinicopathologic, immunohistochemical, and molecular genetic study of 16 cases. Am J Surg Pathol 38 (1): 60-5, 2014.
7. https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
8. https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq#section/_438

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11. Infantile ossifying renal tumor

Summarize

Basic Information

Ossifying Renal Tumor of Infancy (ORTI) is a very rare benign renal tumor with typical clinical, imaging and pathological features. Flesh-eye hematuria is common in children with this disease. MRI and CT findings can be used for diagnosis, but the final diagnosis should be made with reference to pathologic findings.

Epidemiological

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Etiolog & Risk factors

The mass is thought to be derived from the uroepithelium and is attached to the renal medulla, specifically the papillary region of the renal cones. The mass extends from this location in a polypoid fashion into the renal collecting system. Its diameter rarely exceeds 2 to 3 cm.

Classification & Stage

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Clinical manifestations

It occurs in infants up to 1 year of age and is often characterized by hematuria.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

The main basis for the diagnosis of ORTI is as follows:

● Age of the child;

:: Main clinical manifestations: hematuria of the naked eye;

● Imaging (IVP, CT): on imaging, the renal contour is usually intact; however, the collecting system is often observed with partial obstruction. Due to the location of the tumor within the collecting system and its characteristic ossification, ossifying renal tumors in infants may resemble antler stones, but are very rare. The echogenicity of the mass is shaded, and hydronephrosis may be present.CT demonstrates a well-defined mass, often with calcification and poor enhancement;

● Pathology: The pathology of the disease is characterized by the presence of 3 basic components: osteoid nuclei, osteoblasts, and spindle cells;

● Immunohistochemical findings: spindle cells expressed waveform protein in large quantities, and osteoblast-like cells expressed waveform protein and EMA;

● Electron microscopy shows polygonal cells characteristic of epithelial differentiation.

2. Other routine inspections

Clinical Management

It is a benign renal tumor and is best treated by preserving the renal tissue intraoperatively. Surgical methods include partial nephrectomy and tumor resection after incision of the pelvis, which should be based on the site of the tumor. In order to improve the diagnostic accuracy, physicians should make the initial diagnosis of ORTI in children based on clinical presentation and imaging features to reduce the possibility of nephrectomy.

Prognosis

Infantile ossifying renal tumors are benign, no cases of malignant spread have been reported during follow-up, and disease-free survival after surgery ranges from 4 months to 23 years.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

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References

References

1. Chatten J, Cromie WJ, Duckett JW. Ossifying tumor of infantile kidney: report of two cases. Cancer 1980; 45:609-612. crossref, Medline
2. Hu, J., Wu, Y., Qi, J., Zhang, C., & Lv, F. (2013). Ossifying renal tumor of infancy (orti): a case report and review of the literature. Journal of Pediatric Surgery, 48(2), e37-e40.
3. Ito J, Shinohara N, Koyanagi T, Hanioka K. Ossifying renal tumor of infancy: the first Japanese case with long-term follow-up.Pathol Int 1998; 48:151- 159. Crossref, Medline
4. Sotelo-Avila C, Beckwith JB, Johnson JE. Ossifying renal tumor of infancy: a clinicopathologic study of nine cases. Pediatr Pathol Lab Med 1995; 15. 745-762. Crossref, Medline
5. Sotelo-Avila C, Beckwith JB, Johnson JE. Ossifying renal tumor of infancy: a clinicopathologic study of nine cases. Pediatr Pathol Lab Med 1995; 15: 745-762. 745-762. crossref, Medline
6. https://pubs.rsna.org/doi/full/10.1148/radiographics.20.6.g00nv051585

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12. Renal vascular smooth muscle lipoma

Summarize

Basic Information

Renal angiomyolipoma (Angiomyolipoma) is a rare tumor consisting of a disorganized arrangement of blood vessels, smooth muscle and fat. The tumor is a misshapen tumor and is currently considered benign. It develops in 80% of children with tuberous sclerosis by the age of 10 years, but is rare in children without tuberous sclerosis.

Epidemiological

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Etiolog & Risk factors

The tumor is found in 40% to 80% of patients with tuberous sclerosis. The tumor is also associated with neurofibromatosis and vascular Hippel-Lindau syndrome. It is rarer in children without tuberous sclerosis. Eighty percent of children with tuberous sclerosis will develop the disease by age 10. Renal vascular smooth muscle lipomas in patients with tuberous sclerosis are more often bilateral, multifocal, and larger.

Classification & Stage

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Clinical manifestations

The clinical manifestations of the disease are closely related to the size of the tumor. Tumors with a diameter of less than 4 cm usually do not show symptoms; tumors with a diameter of more than 4 cm are more likely to bleed spontaneously, leading to lumbar or abdominal pain, hematuria, and even life-threatening bleeding.

The reason for this is that the tumor is rich in abnormal blood vessels that lack elastin, and these vessels may develop aneurysms, and the clinical manifestations of patients with this disease are associated with bleeding within the aneurysm.

Severe retroperitoneal bleeding is known as Wunderlich syndrome.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

Renal vascular smooth muscle lipoma can be diagnosed by imaging. Its imaging presentation varies widely depending on the number and type of histologic components.

● CT scans and MRI images showing the presence of fat within the mass can be diagnostic;

● Angiography may show the dilated curved vessels characteristic of aneurysms. There are fewer blood vessels in some tumors;

● Although fat is occasionally seen in nephroblastomas and renal cell carcinomas, the diagnosis of renal angiosmooth muscle lipoma is usually clear in the appropriate clinical setting. Renal angiosmooth muscle lipomas are rarely locally aggressive and invade adjacent tissues. Cases of tumor spread to the inferior vena cava and regional lymph nodes have been documented;

● Children with this tumor may also be found positive for Act and Malo by immunohistochemistry;

● The most recent literature advocates a single ultrasound examination of patients with tuberous sclerosis before puberty and annual ultrasound examinations after puberty to identify possible lesions.

2. Other routine inspections

Clinical Management

Renal vascular smooth muscle lipomas larger than 4 cm in diameter should be treated with partial nephrectomy or selective catheter embolization to prevent the development of potentially life-threatening bleeding. However, preservation of functional renal tissue during partial nephrectomy is an important consideration in patients with tuberous sclerosis because renal angiomyolipomas and cysts replace renal parenchyma in large numbers and may lead to end-stage renal disease.

Prognosis

Typical angiosmooth muscle lipomas are benign, but because of their nuclear pleomorphism and mitotic activity, the tumors may extend into the vena cava or spread to regional lymph nodes without malignant progression. The most common serious complication of renal angiosmooth muscle lipoma is hemorrhage.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

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References

References

1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol 1998; 15:21-40.
2. Ewalt DH, Sheffield E, Sparagana SP, Delgado MR, Roach SE. Renal lesion growth in children with tuberous sclerosis complex. J Urol 1998; 160:141-145.
3. Geller E, Smergel EM, Lowry PA. Renal neoplasms of childhood. Radiol Clin North Am 1997; 35:1391-1413.
4. Hennigar RA, Beckwith JB. Nephrogenic adenofibroma: a novel kidney tumor of young people. Am J Surg Pathol 1992; 16:325-334.
5. Lemaitre L, Robert Y, Dubrulle F, et al. Renal angiomyolipoma: growth followed up with CT and/or US. Radiology 1995; 197:598-602.
6. https://pubs.rsna.org/doi/full/10.1148/radiographics.20.6.g00nv051585
7. https://emedicine.medscape.com/article/376848-overview

Audit specialists

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13. Primary renal myoepithelial carcinoma

Summarize

Basic Information

Primary Renal Myoepithelial Carcinoma (PRMC) is a rare cancer that forms in the kidneys. Primary Renal Myoepithelial Carcinoma usually grows and spreads rapidly and occurs in about 20% of cases in children. The common symptom is an abdominal mass. There is no standard treatment and the prognosis is poor.

Epidemiological

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Etiolog & Risk factors

Cancer cells may have a genetic change called translocation. There are cases where the EWSR1 translocation partner is Kruppel-like factor 15 (KLF15).KLF15 is a kidney-specific transcription factor. The new gene fusion transcript EWSR1-KLF15 can be validated and aided in diagnosis using polymerase chain reaction (PCR) and gene sequencing.

Classification & Stage

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

A common symptom of this disease is an abdominal mass.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

The definitive diagnosis of the disease relies primarily on pathologic findings.

Primary renal myoepithelial carcinoma cancer cells have unique morphological features and immunophenotypes, including co-expression of cytokeratins, S-100 and smooth muscle markers, and rearrangement of the EWSR1 gene.

2. Other routine inspections

Clinical Management

There is no standard treatment for this disease. Treatment consists of a combination of surgical removal of the tumor, chemotherapy and radiation therapy.

:: Surgery

Two types of surgery are available depending on the progression of the disease: total nephrectomy and partial nephrectomy (see for more information ).

:: Radiotherapy

There are two types of radiation therapy: external radiation therapy and internal radiation therapy (see Glossary for more information).

Which form of radiotherapy is taken depends on the type and stage of cancer suffered and whether a biopsy has been taken prior to surgery.

:: Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy (see Glossary for more information).
The type of chemotherapy depends on the type and stage of cancer being treated.

Prognosis

Primary renal myoepithelial carcinoma has a very poor prognosis, with frequent metastases and short overall survival.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

:: Clinical trials

References

References

1. National Cancer Institute
2. Cajaiba MM, Jennings LJ, Rohan SM, et al.: Expanding the Spectrum of Renal Tumors in Children: Primary Renal Myoepithelial Carcinomas With a Novel EWSR1-KLF15 Fusion. Am J Surg Pathol 40 (3): 386-94, 2016. [PUBMED Abstract]
3. Gleason BC, Fletcher CD: Myoepithelial carcinoma of soft tissue in children: an aggressive neoplasm analyzed in a series of 29 cases. Am J Surg Pathol 31 Am J Surg Pathol 31 (12): 1813-24, 2007. [PUBMED Abstract]

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14. Renal neuroepithelial tumors

Summarize

Basic Information

Neuroepithelial Tumors of the Kidney (NETKs) are rare, frequent renal tumors in adolescents and young adults with a median age of onset of 18 years.

The tumor grows and spreads quickly and is highly malignant. By the time most children are diagnosed, these tumors have often spread to the outer layer of the kidney, renal veins, and other parts of the body. Neuroepithelial tumors of the kidney are pediatric renal tumors unrelated to Wilms' tumor. It most commonly presents as an abdominal mass and has a poor prognosis.

Epidemiological

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Etiolog & Risk factors

The etiology of NETKs is unclear. Nearly one-third of NETKs have an EWS/FLI1 fusion gene caused by a t(11;22)(q24;q12) chromosomal heterozygote, and the majority of NETKs cells are CD-positive.The pathologic features of NETKs are similar to those of some soft-tissue malignancies.

Classification & Stage

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

The most common presentation is an abdominal mass and most often metastases have occurred before diagnostic findings.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

The disease consists of a group of highly malignant tumors with histologic diversity that are not well characterized histologically or clinically, and the diagnosis relies on histopathology.NETKs may be present in a wide range of ages, including children; they are difficult to diagnose without immunohistochemistry and cytogenetics/molecular biology. Chromosomal abnormalities as well as the EWS/FLI1 fusion gene may aid in diagnosis.

Renal neuroepithelial tumors are pathologically diverse, with features usually consisting of dense clusters of primitive, mostly undifferentiated cells; the cells have round to oval polychromatic nuclei and light-stained eosinophilic cytoplasm, and loosely form fuzzy rosette-like cellular aggregates. Depending on the differentiation of the cells, renal neuroepithelial tumors may present different nuclear and cytoplasmic morphologies.

The vast majority of renal neuroepithelial tumor cells showed CD99 positivity and NSE positivity.

2. Other routine inspections

Clinical Management

There is no standard and effective treatment for renal neuroepithelial tumors.

Renal neuroepithelial tumors are pediatric renal tumors unrelated to Wilms' tumor, so treatment of this tumor differs from that of Wilms' tumor. However, because it is located near the kidney, its counterpart has been tested in a clinical trial developed by the Wilms Tumor Study Group in the United States.

If a child is unfortunate enough to be diagnosed with a renal neuroepithelial tumor, a clinical trial treatment for Ewing sarcoma/primitive neuroectodermal tumor (PNET) should be considered.

Children with recurrent renal neuroepithelial tumors should be considered for treatment in established phase I and phase II clinical trials.

For more information, please refer to the Clinical Trials section of the Kidney and Kidney Tumor Basics.

Clinical trials in pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

Prognosis

Renal neuroepithelial tumors are highly malignant and have a poor prognosis.

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

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References

References

1. http://www.stanfordchildrens.org
2. Parham DM, Roloson GJ, Beckwith JB, et al: Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms' Tumor Study Group Pathology Center. Am J Surg Pathol 25 (2): 133-46, 2001.
3. National Cancer Institute

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15. Post-renal adenoma

Summarize

Basic Information

Metanephric adenomas (Metanephric Adenomas) are rare benign tumors of the kidney.The mean age at onset of Metanephric Adenomas is about 40 years, with an age range of 5-83 years; the mean size of the tumors is 5.5 cm, with a range of sizes from 0.3 to 15 cm.Erythrocytosis and calcification are common in Metanephric Adenomas. Most retrorenal adenomas do not require treatment and have a favorable prognosis.

Posterior renal adenomas should not be confused with mesonephric adenomas (Mesonephric Adenoma). Mesonephric adenoma is pathologically unrelated, although it shares a similar name.

Epidemiological

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Etiolog & Risk factors

The pathogenesis of retrorenal adenomas is unknown. It has a microscopic appearance similar to Nephroblastoma (Wilms tumor) or papillary renal cell carcinoma.

Classification & Stage

not have

Clinical manifestations

Posterior renal adenomas may have typical symptoms associated with renal cell carcinoma, or they may have symptoms such as erythrocytosis, abdominal pain, hematuria, and a palpable mass. Posterior renal adenoma is the type of kidney tumor that most often presents with calcifications and erythrocytosis.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

Posterior renal adenomas can be diagnosed by histopathologic testing.

Posterior renal adenomas may be located in the upper pole, lower pole, and middle lobe regions of the kidney; they are usually confined but unencapsulated. The tumors are brownish-pink and may have cystic and hemorrhagic foci.

The tumor tissue shows a homogeneous structure consisting of tightly packed glandular vesicles or tubular structures. These vesicular or tubular structures have a mature and bland appearance and a small amount of inserted stroma. Tumor cells are small in size and have deeply stained nuclei and inconspicuous nucleoli. No primitive cells are present, while calcified vesicles may be present. Tissue resembling early fetal mesentery may be present. The lumens of the follicles sometimes contain epithelial protrusions or fibrous material, but in most cases are vacuolated. Mitosis is evident.

Tumor cells Leu7+ (3/5), Vimentine+ (4/6), Cytokeratin+ (2/6), Epithelial Membrane Antigen+ (1/6), and Muscle Specific Antigen positive (1/6). In addition, strong positivity and diffuse staining for alpha-methylacyl-coenzyme A racemase (AMACR) can be used to differentiate renal cell carcinoma from retro-renal adenoma, and the combination of serial immunoassays, including AMACR, CK7, and CD57, provides a better differential diagnosis. The differential diagnosis is quite difficult.

2. Cytogenetic testing

Genetic analysis of chromosomes 7, 17, and Y may help to differentiate retrorenal adenomas from papillary renal cell carcinomas in difficult cases. It was shown that retrorenal adenomas lack the increase in chromosomes 7 and 17 and the decrease in Y chromosome frequently seen in typical papillary renal cell tumors, suggesting that retrorenal adenomas are not associated with renal cell carcinomas or papillary adenomas.

3. Other routine inspections

Clinical Management

Since retrorenal adenomas are considered benign, they do not require treatment.

Prognosis

Posterior renal adenomas have a good prognosis, with no evidence of metastasis or local recurrence to date.

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References

References

1. https://en.wikipedia.org/wiki/Metanephric_adenoma
2. Bastos Netto JM, Esteves TC, Mattos RD, Tibiriçá SH, Costa SM, Vieira LJ (August 2007). "Metanephric adenoma: A rare differential diagnosis of renal tumor in children". J Pediatr Urol. 3 (4): 340-341.
3. Davis Cj jr, Barton JH, Sesterhenn IA, Mostofi FK (1995) Metanephric adenoma. Clinicopathologic study of fifty patients. Am J Surg Pathol 19(10):1101 -Kovacs, G. (1995) Metanephric adenoma.
4. Kovacs, G.; Akhtar, M.; Beckwith, BJ.; Bugert, P.; Cooper, CS.; Delahunt, B.; Eble, JN.; Fleming, S.; et al. (Oct 1997). "The Heidelberg classification of renal cell tumours". J Pathol. 183 (2): 131-3.
5. Brisigotti, M.; Cozzutto, C.; Fabbretti, G.; Sergi, C.; Callea, F. (Oct 1992). "Metanephric adenoma". Histol Histopathol. 7 (4): 689-92.
6. Jones, E. C.; Pins, M.; Dickersin, G. R.; Young, R. H. (1995). "Metanephric adenoma of the kidney. A clinicopathological, immunohistochemical, flow cytometric, cytogenetic, and electron microscopic study of seven cases". The American journal of surgical pathology. 19 (6): 615-626.
7. Bostwick DG, Eble JN (2008) Urologic Surgical Pathology. St Louis, Mosby, p118.
8. Grignon, D. J.; Eble, J. N. (1998). "Papillary and metanephric adenomas of the kidney". Seminars in diagnostic pathology. 15 (1): 41-53.
9. Jones EC, Pins M, Dickersin GR, Young RH (1995) Metanephric adenoma of the kidney. A clinicopathological, immunohistochemical, flow cytometry, cytogenetic and electron microscopic study. cytogenetic and electron microscopic study of seven cases. Am J Surg Pathol 19(6):615-626.
10. Olgac S, Hutchinson B, Tickoo SK, Reuter VE (2006) Alpha-methylacyl-CoA racemase as a marker in the differential diagnosis of metanephric adenoma. Mod Pathol 19:218-224.
11. Pins MR, Jones EC, Martul EV, Kamat BR, Umlas J, Renshaw AA (1999) Metanephric adenoma-like tumours of the kidney. report of three malignancies with emphasis on discriminating features. Arch Pathol Lab Med. 123:415-420.
12. Brunelli M, Eble JN, Zhang S, Martignoni G, Cheng L (2003) Metanephric adenoma lacks the gains of chromosomes 7 and 17 and loss of Y that are typical of papillary renal cell carcinoma and papillary adenoma. Mod Pathol 16(10):1060-1063.
13. Galmiche L, Vasiliu V, Poirée S, Hélénon O, Casanova JM, Brousse N (October 2007). "[Diagnosis of renal metanephric adenoma: relevance of immunohistochemistry and biopsy]". Ann Pathol (in French). 27 (5): 365-8.
14. Nagashima Y, Arai N, Tanaka Y, Yoshida S, Sumino K, Ohaki Y, Matsushita K, Morita T, Misugi K 81991) Case record: two cases of renal epithelial tumor resembling immature nephron. Virchow Arch A Pathol Anat 418:77-81.
15. Pages A, Granier M (1980) Le néphrome néphronogène. Arch Anat Cytol Pathol 28:99-103.

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16. Renal mesenchymal sarcoma

Summarize

Basic Information

Renal Anaplastic Sarcoma is a rare pediatric renal tumor. Before the disease was formally defined, it was widely included among other renal tumors. The disease is characterized as an embryonal sarcoma of the kidney. It is most common in children or adolescents under the age of 15 years, with a mean age of onset of 12 years and a median age of 5 years, and the age of onset ranging from 1 to 41 years, with a female predominance. It often presents as an asymptomatic renal mass, with a prevalence in the right kidney. The most common sites of metastasis are the lungs, liver and bones. It is usually treated by surgical resection and the prognosis is related to the stage of the disease.

Epidemiological

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Etiolog & Risk factors

Renal mesenchymal sarcomas have been reported to have cytogenetic abnormalities, such as rearrangements between 10q21 and 18p11.2, and are associated with mutations in the DICER1 gene.

Recent reports suggest that renal interstitial metaplastic sarcoma is associated with germline DICER1 mutations and should be included in the DICER1 syndrome. Because one or more associated tumors can be present in people with germ cell DICER1 mutations and because different affected members of a family can present with different tumors, genetic counseling and testing for germ line DICER1 mutations should be considered when patients are diagnosed.

DICER1 is an important gene encoding micro RNA (mi RNA), and mutations in it cause the homonymous syndrome (DICER syndrome) that has been associated with tumorigenesis in a variety of organs, including pleuropulmonary histiocytomas, as well as the lungs, kidneys, ovaries, and thyroid.

Classification & Stage

Renal interstitial metaplastic sarcoma tumor cells can spread through tissues, the lymphatic system, and the bloodstream. Staging is used to find out if the cancer has spread to parts of the body other than the kidneys. Staging is important and determines how treatment is given.

The specific staging of this disease is the same as for nephroblastoma, see " Clinical Staging of Nephroblastoma ".

Clinical manifestations

The disease often presents as an asymptomatic renal mass, most commonly in the right kidney. The most common sites of metastasis are the lungs, liver and bones.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

The diagnosis of renal interstitial metaplastic sarcoma relies on a combination of imaging and histopathologic evidence, combined with DICER1 mutations, and the presence of other sites of consolidated tumors.
Renal interstitial metaplastic sarcoma often appears as a large renal mass shadow with an average size of 12 cm, often with a cystic component, and often involving the renal pelvis. If the lungs are involved, it may appear as a space-occupying lesion in the lungs of a solid or cystic nature.

The clinical presentation, imaging, and pathology of renal mesenchymal sarcomas have similarities to other renal tumors. The differential diagnosis includes interstitial nephroblastoma, various renal synovial sarcomas, various malignant mesenchymal tumors, and stasis tumors.

Renal mesenchymal sarcoma is characterized by the presence of cystic degeneration in the area of the lesion. The pathology consists of bundles of undifferentiated spindle cells showing marked cellular atypical proliferation and the presence of large areas lacking embryonic cell-like cells, usually accompanied by TP53 overexpression. Hyaline cartilage or chondrocyte-like material is often present in these tumors, with no epithelial structures. Immunostaining is often vementin + (5/5), desmin + (4/6), P53 (strongly positive 3/6), cytokeratin -, NB84a -, MyoD1 -, CD34 -, CD99 -, WT1 -. Renal mesenchymal sarcoma presented similar pathologic features to pleuropneumoblastoma in children and undifferentiated embryonal sarcoma of the liver.

2. Other routine inspections

Clinical Management

Due to the rarity of this tumor, the best treatment is unknown. It is usually treated by surgical removal. In addition to surgery, radiotherapy and chemotherapy are used. In the past, these tumors were identified as mesenchymal nephroblastoma and treated accordingly.

:: Surgery

Two types of surgery are available depending on the progress of the disease: total nephrectomy and partial nephrectomy.
Some patients may receive chemotherapy or radiation therapy after surgery to kill all the cancer cells. Treatment given after surgery, called adjuvant therapy, is used to reduce the risk of the cancer coming back. Sometimes, a second surgery is needed with the goal of seeing if the cancer cells remain after chemotherapy or radiation.

:: Radiotherapy

There are two types of radiation therapy: external radiation therapy and internal radiation therapy.
Exactly which form of radiotherapy is given depends on the type and stage of cancer suffered and whether a biopsy has been taken prior to surgery.

:: Chemotherapy

Chemotherapy is divided into two modalities: systemic chemotherapy and localized chemotherapy.
The type of chemotherapy depends on the type and stage of cancer being treated.

Prognosis

Renal interstitial metaplastic sarcoma is rare, and in the available follow-up data, children with stage I renal interstitial metaplastic sarcoma have a good 2-year disease-free survival rate, and metastases and recurrences occur more often in children with stage II and above.

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:: Clinical trials

For more information, please refer to the Clinical Trials section of the Kidney and Kidney Tumor Basics.
Clinical trials for pediatric renal tumors currently open in the United States can be found at https://clinicaltrials.gov/ .

References

References

1. https://en.wikipedia.org/wiki/Renal_anaplastic_sarcoma
2. Vujanić GM, Kelsey A, Perlman EJ, Sandstedt B, Beckwith JB (2007) Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. Am J Surg Pathol 31(10):1459-1468
3. Wu MK, Vujanic GM, Fahiminiya S, Watanabe N, Thorner PS, O'Sullivan MJ, Fabian MR, Foulkes WD (2017) Anaplastic sarcomas of the kidney are characterized by DICER1 mutations. Mod Pathol. 2018 Jan;31(1):169-178.
4. Watanabe N, Omagari D, Yamada T, Nemoto N, Furuya T, Sugito K, Koshinaga T, Yagasaki H, Sugitani M. (2013) Anaplastic sarcoma of the kidney: case report and literature review. Pediatrics International 55, e129-e132.

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17. Renal lymphoma

Summarize

Basic Information

Renal lymphoma (RML) can be categorized into primary renal lymphoma and secondary renal lymphoma:

1. Primary renal lymphoma (Prenatal lymphoma)

Because there is no lymphatic system per se in the kidneys, primary renal lymphomas are extremely rare, accounting for about 0.7% of extranodal lymphomas. Primary renal lymphoma in children is very rare and most often presents with bilateral renal involvement (single kidney involvement has also been reported), renal failure and anemia. Almost all patients with primary renal lymphoma develop extrarenal lymphoma shortly after diagnosis, and few cases survive more than 1 year after diagnosis.

2. Secondary renal lymphoma

The vast majority of renal lymphomas are secondary to renal lymphoma. More than 1/3 of lymphoma patients are diagnosed with renal lymphoma at autopsy, and only about 12% of surviving patients with known lymphoma will have renal involvement detected by imaging such as MRI, CT, and ultrasound at staging. In pediatric patients, renal involvement occurs in a similar proportion as in adults, mostly in the non-Hodgkin's lymphoma (NHL) and Burkitts subtypes, most often in older children, and manifests as unilateral or bilateral renal masses on imaging, often in combination with extra-renal lymphadenopathy, which can rapidly improve renal function with systemic therapy, such as radiotherapy or chemotherapy.

Epidemiological

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Etiolog & Risk factors

Systemic lymphoma as a risk factor for renal lymphoma.

Classification & Stage

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Clinical manifestations

Although the majority of cases present with bilateral renal involvement, most patients with renal lymphoma do not have significant signs of impaired renal function, and symptoms such as acute renal failure, anemia, hematuria, fatigue, and musculoskeletal pain have been rarely reported. None of the cases with unilateral renal involvement had systemic symptoms or manifestations of renal failure.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

The diagnosis of renal lymphoma relies on CT and renal biopsy.

Because renal lymphoma is similar to other renal tumors in both pathology and imaging, the age of the child, renal biopsy pathology, the presence of bilateral renal non-obstructive enlargement, the presence of other organ or lymph node involvement, the presence of renal failure without any other cause, and the rapid improvement of renal function after radiation therapy or systemic chemotherapy should be considered comprehensively for the specific diagnosis.

2. Other routine inspections

Clinical Management

The treatment of this disease is based on chemotherapy. Because excretion of tumor metabolites may lead to renal obstructive or uric acid nephropathy, careful monitoring of renal function is required during treatment .

Prognosis

Kidney involvement may be present at the time of lymphoma diagnosis and does not necessarily portend a poor prognosis. Adult patients with renal lymphoma rarely survive more than one year, and children with renal lymphoma have a better prognosis.

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References

References

1. https://emedicine.medscape.com/article/378644-overview
2. Lee, J. S., Sanchez, T. R., & Wootton-Gorges, S. (2015). Malignant renal tumors in children. Journal of Kidney Cancer and VHL, 2(3), 84-89. http://doi.org/10.15586/jkcvhl.2015.29
3. Neeraj B. Chepuri, Peter J. Strouse, and Gregory A. Yanik. CT of Renal Lymphoma in Children. American Journal of Roentgenology 2003 180:2, 429-431
4. Coca, P., Linga, V. G., Gundeti, S., & Tandon, A. (2017). Renal Lymphoma: Primary or First Manifestation of Aggressive Pediatric B-cell Lymphoma. Indian Journal of Medical and Paediatric Oncology : Official Journal of Indian Society of Medical & Paediatric Oncology, 38(4), 538-541. http://doi.org/10.4103/ijmpo.ijmpo_48_16

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18. Paraglomerular cell tumors

Summarize

Juxtaglomerular Cell Tumor (JGCT), also known as nephrolithiasis, is a very rare glomerulonephritic tumor cell with less than 100 cases reported in the literature.

This tumor usually secretes renin, hence the name nephrolithiasis was once given to it. It often causes severe and uncontrollable high blood pressure and occurs in both adults and children.

It is most common in adolescents and young adults in their 20s and 30s, and is more common in women, but may be recognized and diagnosed many years after its onset. The disease is generally considered benign, but its malignant potential is uncertain.

Epidemiological

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Etiolog & Risk factors

The etiology is unknown.

Classification & Stage

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Clinical manifestations

Patients with JGCT often present with a variety of symptoms, including headache, retinopathy, double vision, dizziness, nausea, vomiting, polyuria, and urinary protein. Most of these symptoms may be attributed to hypertension or hypokalemia.

Clinically, JGCT is divided into three categories:

● Typical JGCT: Most JGCTs are characterized by high renin concentrations, aldosteronism, hypokalemia, and hypertension. By overproducing renin, JGCT usually causes severe and persistent hypertension, with occasional paroxysmal hypertension in some cases.JGCT causes secondary aldosteronism and induces hypokalemia, but hypokalemia can be mild despite high renin production. In both cases the tumor can be surgically removed;

● Atypical JGCT: The most common presentation shows hypertension with normal potassium levels;

● Non-functional JGCT: very rare, characterized by normotension and normal potassium levels.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

JGCT may cause malignant hypertension. Clinicians should strongly suspect JGCT if unexplained aldosteronism, or associated severe or even moderate hypertension, is found in adolescents or young adults.The diagnosis of JGCT can be made by a combination of tests such as CT or postoperative pathology, and evidence of renal tumors.

However, other renal tumors can also cause hypertension by secreting renin and need to be carefully differentiated. Some JGCT have also been associated with membranous glomerulonephritis.

The morphology of JGCT is characterized by multifocal malignant mesenchymal epithelioid cells, often with mixed necrosis and perivascular growth patterns.

The immunophenotype was characteristic, with tumor cells expressing renin, CD34, smooth muscle actin, CD138, waveform protein, collagen IV, and being negative for cytokeratin as well as S100, c-kit, and junctional proteins.

The presence of renin granules in pathological specimens and immunohistochemical analysis in postoperative pathology can help to distinguish this disease from other primary renal tumors such as angioepithelial cell tumors, hemangioglobular tumors, renal mesenchymal adenomas, epithelioid angiomyolipomatous smooth muscle lipomas, nephroblastomas, isolated fibrous tumors, and some epithelial tumors.

Karyotype analysis of a few of these tumors showed common losses of chromosomes 9 and 11.

2. Other routine inspections

Clinical Management

Most JGCT tumors have an intact envelope and can be radically or locally nephrectomized by surgery or radiation therapy. Antihypertensive medications may be used to assist in the treatment of hypertension if necessary. In most cases, blood pressure and plasma renin levels usually return to normal after nephrectomy for JGCT.

However, about 10% of patients do not get rid of hypertension even after complete removal of the tumor. The reason for this is that long-term hypertension makes the blood vessels lesioned, and even after removing the cause of the kidney disease, the blood vessels still have not recovered, so this hypertensive vascular lesion will make the hypertension persist.

● Surgery
Two types of surgery are available depending on the progress of the disease: total nephrectomy and partial nephrectomy.
Some patients may receive chemotherapy or radiation therapy after surgery to kill all the cancer cells. Treatment given after surgery, called adjuvant therapy, is used to reduce the risk of the cancer coming back. Sometimes, a second surgery is needed with the goal of seeing if the cancer cells remain after chemotherapy or radiation.

:: Radiotherapy

Prognosis

The majority of JGCT patients present with benign manifestations, and no local recurrence or metastasis has occurred with radical or partial nephrectomy.

However, one case of JGCT metastasis has been reported to date, so its malignant tendency is unknown. In addition, death due to massive cerebral hemorrhage secondary to severe hypertension and fetal death due to hypertension secondary to JGCT have been reported.

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References

References

1. https://en.wikipedia.org/wiki/Juxtaglomerular_cell_tumor
2. Naoto Kuroda.(2011). Review of juxtaglomerular cell tumor with focus on pathobiological aspect. Diagnostic Pathology, 6, 80. http://doi.org/10.1186/1746-1596-6-80
3. Akio Hasegawa. (1997) Juxtaglomerular Cell Tumor of the Kidney: A Case Report with Electron Microscopic and Flow Cytometric Investigation. Ultrastructural Pathology 21:2, pages 201-208. 4.
4. David Cucchiari. (2013) Juxtaglomerular Cell Tumor: Multicentric Synchronous Disease Associated With Paraneoplastic Syndrome. Journal of Clinical Oncology 2013 31:14, e240-e242

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19. Medullary carcinoma of the kidney

Summarize

Renal Medullary Carcinoma (RMC) is a rare cancer of the kidneys that is almost exclusively found in children or young adults (11-39 years old) of African descent, with a male predominance.

Medullary carcinoma of the kidney often presents with hematuria, flank and back pain, significant weight loss, palpable kidney mass or lymph and enlargement. The disease tends to be aggressive and difficult to treat, and metastasis has usually occurred by the time of diagnosis. Treatment is primarily through chemotherapy and radiation and has a very poor prognosis.

Epidemiological

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Etiolog & Risk factors

The etiology of medullary carcinoma of the kidney is not fully understood.

The vast majority of individuals with this type of cancer have malformed red blood cells, which were originally disc-shaped with a concave center, and grow sickle-shaped in the patient.

This means that the patient may also have sickle cell disease, which is an inherited disease, and children with this disease may also have this tumor, which is why medullary carcinoma of the kidney is also known as the "seventh sickle cell nephropathy".

Medullary carcinoma of the kidney is very rare and it is not possible to predict the probability that those individuals with sickle cell trait will eventually develop this cancer. Early detection has a better prognosis, but screening is still not feasible.

Genetically, relevant mutated genes have been reported including topoisomerase II alpha guo expression, INI1 expression deletion, mesenchymal lymphoma kinase (ALK) receptor TYROSINE kinase rearrangement, and T(2;10)(p23;q22) chromosome translocation, and the specific mechanisms need to be determined by further studies.
Other genetic or environmental factors contributing to the risk of renal medullary carcinoma have not been identified.

Classification & Stage

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Clinical manifestations

Medullary carcinoma of the kidney is often associated with gross hematuria (60%), back/flank pain (50%), palpable renal mass (75% appear on the right side) or enlarged lymph nodes, and significant weight loss (25%). Medullary renal carcinoma is highly aggressive and the most common sites of metastasis are localized lymph nodes, adrenal glands, lungs, liver, inferior vena cava and peritoneum.

Clinical Department

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Examination & Diagnosis

When a patient of African ancestry presents with a right-sided abdominal mass and hematuria, the diagnosis of medullary carcinoma of the kidney should be highly suspected and confirmed by a combination of imaging and, ultimately, surgical biopsy and pathology of the tumor.

Histopathological studies show a distinctive pattern of this disease that differs from other renal tumors. Medullary carcinoma of the kidney is a malignant epithelial tumor that originates from the collecting duct epithelium. Severe tumors tend to appear as isolated grayish-white masses with marked necrosis and hemorrhage.

Microscopically, renal medullary carcinoma is often infiltrative, including solid sheets of poorly differentiated carcinoma. Poorly formed vacuoles are seen, and a variety of microscopic morphologies are seen in individual specimens, including solid, reticular, tubular, trabecular, cristate, and micropapillary structures, such as sarcomatoid morphology. Tumor tissue is usually accompanied by infiltration of inflammatory cells, especially neutrophils and lymphocytes.

Immunohistochemically, renal medullary carcinomas show positivity for CAM5.5 and epithelial membrane antigen and usually express cytokeratin AE1/AE3, low molecular weight cytokeratins, waveform proteins, hypoxia-inducible factor (HIF), and vascular endothelial growth factor (VEGF); cytokeratins 34EE12 are negative; and the expression of cytokeratins 7,20, carcinoembryonic antigen, and high molecular weight cytokeratins of variability were common.

Clinical Management

Renal medullary carcinoma is highly aggressive, often with distant cancer metastases already present at the stage of detection, and is less responsive to chemotherapeutic agents, often becoming chemoresistant. As current chemotherapy options are very limited, early detection may provide an opportunity for surgical resection and a greater time frame for initiating a new chemotherapy regimen in patients who have failed their current chemotherapy regimen.

Chemotherapy and radiotherapy have not been found to be particularly effective in this disease. Chemotherapy regimens that have been tried include cyclophosphamide, doxorubicin, cisplatin, topotecan, methotrexate, and vincristine. However, none of the current regimens have significantly improved outcomes. The role of radiation therapy is unclear. Because of the apparent tendency of tumors to spread to the central nervous system, it has been suggested that prophylactic cranial irradiation should be considered. Immunotherapy is still under investigation, and current studies suggest that topoisomerase IIα may be a new therapeutic target.

Prognosis

As the disease is mostly detected at an advanced stage, the prognosis is generally extremely poor, with a median survival time of 3 months (1 to 7 months). In the case report, one patient reported a survival of more than one year; another patient whose tumor was well controlled and non-metastatic was followed up for more than 8 years. This suggests that early detection can significantly improve survival.

Follow-up & Review

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Cutting-edge Therapeutic & Clinical Research

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References

References

1. https://en.wikipedia.org/wiki/Renal_medullary_carcinoma
2. Narendrakumar Alappan, Creticus P. Marak, Amit Chopra, Parijat S. Joy, Olena Dorokhova, and Achuta K. Guddati, "Renal Medullary Cancer in a Patient with Sickle Cell Trait," Case Reports in Oncological Medicine, vol. 2013, Article ID 129813, 4 pages, 2013.
3. Shetty, A., & Matrana, M. R. (2014). Renal Medullary Carcinoma: A Case Report and Brief Review of the Literature. the Ochsner Journal, 14(2), 270-275.
4. Lee, J. S., Sanchez, T. R., & Wootton-Gorges, S. (2015). Malignant renal tumors in children. Journal of Kidney Cancer and VHL, 2(3), 84-89.

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