Soft Tissue Sarcoma

Essential Information

Summarize

 

      Soft tissue sarcoma in children is a disease in which malignant tumor cells (cancer cells) form in the soft tissues of the body. Soft tissue sarcomas can occur in soft tissues anywhere in the body, including muscles, tendons, fat, blood vessels, lymph vessels, nerves, and tissues around joints. In children, it is most common in the arms, legs, or trunk (chest and abdomen). When a soft tissue sarcoma starts and is very small, it is almost undetectable because it usually doesn't cause problems. However, it is as the soft tissue sarcoma grows that it affects the normal functioning of the body.

 

Childhood soft tissue sarcoma is a malignant tumor that forms in soft tissues throughout the body;

Soft tissue sarcomas occur in both children and adults;

Having certain diseases and inherited problems increases the risk of soft tissue sarcoma in children;

The most common symptom of soft tissue sarcoma in children is a painless lump or swelling of the soft tissues throughout the body;

If needed, your doctor will perform a biopsy of the soft tissue sarcoma;

There are many different classifications of soft tissue sarcomas;

Certain factors affect prognosis (chance of recovery) and treatment options.

 

 

Epidemiological

       There are over 50 different types of soft tissue sarcomas, and to be precise, they are a series of related diseases rather than a single disease. Specific types of sarcomas are often named according to the normal tissue cells they most closely resemble. Some sarcomas do not look like any normal tissue and are thought to come from stem cells or other primitive cells. Stem cells are specialized cells that can mature into specific tissues or organs.

 

       Sarcomas can occur in both children and adults, but are rare in adults, accounting for about 1% of all adult cancers; sarcomas, however, account for 15% of childhood cancers. Fortunately, children's soft tissue sarcomas respond differently to treatment, but overall have a better prognosis than adult soft tissue sarcomas.

 

Etiolog & Risk factors

        Scientists have found an association between the risk of soft tissue sarcoma in children and the development of certain genetic problems and diseases that increase the risk of soft tissue sarcoma in children, they are called 'risk factors'.

 

        All of the following diseases or conditions have been shown to be risk factors for soft tissue sarcoma in children, and if your child has any of the following conditions, you should talk to your doctor about them during your visit:

 

● Li Fraumeni syndrome

● Familial adenomatous polyposis (FAP)

● Retinoblastoma 1 gene mutation

● Mutations in the SMARCB1 (IIN1) gene

● Neurofibromatosis type 1 (NF1)

● Werner syndrome

 

         Other risk factors are:

 

● Prior radiation therapy;

● Co-existing with AIDS (Acquired Immune Deficiency Syndrome) and Epstein Barr (EB) virus infection.

 

         It's important to note that having risk factors doesn't mean you'll get cancer, and not having risk factors doesn't mean you won't get cancer.

 

         References:

         https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage&isappinstalled=0

 

Classification & Stage

 

       Soft tissue sarcomas can be divided into many different types. Different types of sarcoma cells look different under the microscope. Soft tissue tumors can be divided into the following categories according to their origin:

 

1. Liposarcoma

 

       This is a rare cancer of fat cells. There are several different types of liposarcoma. Most liposarcomas that occur in children and adolescents are low-grade (tend to grow and spread slowly). The most common type is mucinous liposarcoma, which is usually less malignant and treats well. The other type, pleomorphic liposarcoma, is usually more malignant (tends to grow and spread rapidly) and is difficult to treat well.

 

2. Bone and chondrosarcoma

 

       Bone and cartilage tumors are a mixture of bone cells and cartilage cells. Bone and cartilage tumors include the following types:

 

       2.1 Extraosseous mesenchymal chondrosarcoma

 

       This type of tumor occurs in the head and neck of young people and includes mesenchymal chondrosarcoma, which grows rapidly and often spreads to the lungs.

 

       2.2 Extraosseous Osteosarcoma

 

       This type of bone and chondrosarcoma is very rare in children and adolescents. It may recur after treatment and may spread to the lungs.

 

3. Fibrous (connective) tissue sarcoma

 

       Fibrous (connective) histiomas include the following types:

 

       3.1 Hard fibroids (also known as aggressive fibroids)

 

       This type of tumor is low-grade (tends to be slow-growing) and may recur in adjacent tissues but usually does not spread to distal parts of the body. In some very rare cases, the tumor may disappear after treatment. Sclerofibromas sometimes occur in children with a variant of the adenomatous polyposis coli (APC) gene, which causes familial adenomatous polyposis (FAP), in which many polyps form on the lining of the colorectal wall (FAP). grow on the mucosa.) FAP is a genetic condition, and genetic testing is needed to know this, so it may be necessary to talk to a geneticist.

 

       3.2 Fibrosarcoma

 

       There are two types of fibrosarcoma that occur in children and adolescents:

 

      3.2.1 Infantile fibrosarcoma (also known as congenital fibrosarcoma)

 

       They often occur in children 4 years of age or younger, most commonly in infants, and can be observed on prenatal ultrasound. These tumors are often large and grow rapidly, but rarely spread to the far ends of the body. Their tumor cells usually have a specific genetic variant called translocation (a part of one chromosome is replaced with a part of another chromosome). To diagnose infantile fibrosarcoma, the tumor cells need to be tested for the genetic variant.

 

      3.2.2 Adult-type fibrosarcoma

 

       The cells of these tumors do not have the genetic variants found in infantile fibrosarcoma.

 

:: Bulging dermatofibrosarcoma: this is a rare fibrous tissue tumor found in children and adults. The cells of this type of tumor are ectopic. In order to diagnose rhabdomyosarcoma, the tumor cells are tested for genetic variants.

 

:: Inflammatory myofibroblastoma: This is a fibrous tissue tumor that occurs in children and adolescents. It is likely to recur after treatment but rarely spreads to the distal parts of the body. Nearly half of the tumors show some kind of genetic change.

 

● Low-grade fibromucinous nodular sarcoma: this is a slow-growing tumor seen in young and middle-aged people. Chromosomal translocations are present in the cells of this tumor, so for diagnosis, the tumor cells need to be tested for genetic variants. This tumor may recur and spread to the mucosal layer of the lungs and chest wall years after treatment and therefore requires lifelong follow-up.

 

● Mucinous fibrosarcoma: low grade, this is a rare fibrosarcoma, more uncommon in children as opposed to adults. Tends to grow slowly.

 

:: Sclerosing epithelioid fibrosarcoma: this is a rare fibrous tissue tumor that can recur years after treatment and spread to other parts of the body. Therefore, long-term follow-up is needed.

 

4. Skeletal rhabdomyosarcoma

 

       Skeletal muscles are muscles that attach to bones to help move the body, also known as rhabdomyosarcomas. Rhabdomyosarcoma is the most common soft tissue sarcoma in children. More information is detailed in the  " section." Rhabdomyosarcoma

 

5. Smooth muscle tumors

 

       Smooth muscle is found inside blood vessels and in the hollow parts of internal organs, such as the stomach, intestines, bladder, uterus

 

       Smooth muscle sarcoma is associated with the Epstein-Barr (EB) virus in children who also have AIDS (HIV/AIDS). Smooth muscle sarcoma may occur in survivors of hereditary retinoblastoma, and some patients with retinoblastoma may develop smooth muscle sarcoma years after treatment.

 

6. Fibrous histiocytoma

 

       Fibrous histiocytomas include the following types:

 

       6.1 Plexiform Verrucous Histiocytoma

 

       This is a rare tumor seen in children and young adults. It usually begins as a painless swelling, usually under the skin of the arm, palm, or wrist, and rarely spreads to nearby lymph nodes or the lungs.

 

       6.2 Undifferentiated pleomorphic sarcoma

 

       This type of tumor may be on a part of the body where the patient has previously received radiation therapy or become a secondary tumor in a child with retinoblastoma. This type of tumor is usually found on the arm or leg and may spread to other parts of the body.

 

7. Tumors of the peripheral nervous system

 

       Peripheral nervous system tumors include the following types:

 

      7.1 Mesothelioma

 

       This is a rare, fast-growing tumor of the nerve sheath (nerve sheath: a protective covering for nerves that are not part of the brain or spinal cord) that occurs mainly in children and can form on the head, neck, abdomen, perineum, scrotum, arms, or legs.

 

      7.2 Malignant peripheral nerve sheath tumors

 

       This is a tumor that forms in the nerve sheath. Some children with malignant peripheral nerve sheath tumors also get a rare genetic disease called multiple neurofibromatosis type I (NF1). This tumor has varying degrees of malignancy.

 

      7.3 Malignant salamander tumors

 

       This is a very rare, fast-growing tumor that is common in children with type I multiple neurofibromatosis (NF1).

 

8. Pericyte (perivascular) tumors

 

       Ependymal cell tumors form in the cells that surround blood vessels. The following types are included.

 

       8.1 Pericytoma

 

       For example, infantile angioepithelioma, a pericytoma. The prognosis for this tumor is likely to be better if it is found before the age of 1 year. If found after 1 year of age, the tumor is more likely to spread to other parts of the body, including the lymph nodes and lungs.

 

       8.2 Myofibromatosis in young children

 

       Myofibromatosis in young children is another type of pericytoma. It usually forms before the age of 2 years and may be a single nodule, usually in the head and neck area, or multiple nodules, usually seen in the skin, muscles, and bones. These tumors may disappear naturally.

 

9. Tumors of unknown origin

 

       Tumors with an unknown primary site (not knowing where the tumor first formed) include the following types:

 

       9.1 Adenovascular soft tissue sarcoma

 

       This is a rare tumor of soft tissues that are usually attached to or surround organs and other tissues. It is most commonly found in the extremities, but can also be found in the tissues of the mouth, jaw, and face. This tumor may be slow-growing, but it may also have spread by the time of diagnosis. The prognosis is likely to be better when the tumor is less than 5 centimeters, or when it is possible to remove it completely by surgery. Chromosomal translocations are present in the cells of this tumor, so for diagnosis, testing for genetic alterations in the tumor cells is required.

 

       9.2 Clear cell sarcoma of soft tissue

 

       It is a slow-growing soft-tissue tumor that starts in the tendon (a tough, fibrous, cortical-like tissue that holds muscles and bones or other parts of the body together). Clear cell sarcoma develops in the deep tissues of the foot, heel, and ankle and may spread to nearby lymph nodes. Chromosomal translocations are present in the cells of this tumor, and diagnosis requires testing for genetic changes in the tumor cells.

 

       9.3 Connective tissue-promoting proliferative small round cell tumors

 

       This type of tumor is most often found in the tissues around the abdomen, pelvis, or testicles, but may also form in the kidneys and may spread to the lungs and other parts of the body. Chromosomal translocations are present in the cells of this tumor, and diagnosis requires testing for genetic changes in the tumor cells.

 

       9.4 Epithelioid sarcoma

 

       This is a rare type of sarcoma that usually starts slowly as a hard mass deep in the soft tissues and may spread to the lymph nodes.

 

       9.5 Malignant rhabdomyosarcoma

 

       It is a rare, fast-growing tumor of soft tissues, such as the liver and peritoneum. It usually occurs in young children, including newborns, but can also occur in older children and adults. Rhabdomyosarcoma may be associated with a mutation in a tumor suppressor gene called SMARCB1. The protein encoded by this gene plays an important regulatory role in cell growth.Changes in the SMARCB1 gene may be inherited (passed from parent to next generation). Genetic counseling (a discussion with a trained professional about inherited disorders and the genetic testing that may be needed) may be needed when this occurs.

 

       9.6 Extraosseous mucinous chondrosarcoma

 

       This is a rare soft tissue sarcoma seen in children and adolescents. Over time, it spreads to other parts of the body, including the lymph nodes and lungs. Chromosomal translocations are present in the cells of this tumor, and diagnosis requires testing for genetic changes in the tumor cells. This tumor may recur years after treatment.

 

10. Perivascular epithelioid cell tumors (PEComas)

 

       Benign (non-cancerous) tumors may occur in children with the inherited disease of tuberous sclerosis in the stomach, intestines, lungs, female genitals, and urogenital organs.

 

11. Primitive neuroectodermal tumor (PNET)/peripheral Ewing tumor

 

       Undifferentiated/unclassified sarcomas usually occur in the skeletal muscles, and this type of tumor may affect body movement by affecting the skeletal muscles in turn.

 

12. Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (highly)

 

       This soft tissue tumor may form in a part of the body where a patient has received radiation therapy in the past, or in a child with retinoblastoma. This tumor is usually found in the arm or leg and may spread to other parts of the body.

 

13. Hemangiosarcoma

 

       Hemangiosarcomas include the following types:

 

       13.1 Angiosarcoma of soft tissue

 

       A fast-growing tumor that may form in blood vessels or lymphatic vessels anywhere in the body, most hemangiosarcomas are found under the skin. And those tumors that are deep in the soft tissues can form in the liver, spleen and lungs. Although very rare in children, more than one tumor is sometimes found in the skin or liver. In rare cases, infantile hemangiosarcoma may become a soft tissue hemangiosarcoma.

 

      13.2 Epithelioid hemangioendothelioma

 

       It can be seen in children, but is more common in adults between the ages of 30 and 50. The tumors usually occur in the liver, lungs, or bones. They can grow quickly or slowly. In about 1/3 of cases, the tumor spreads quickly to other parts of the body.

 

      13.3 Vascular Ectodermal Cell Tumor (Infant)

 

       These soft tissue tumors have many blood vessels, and children under 1 year of age have a better prognosis. In children older than 1 year, these tumors are more likely to spread to distal tissues, including the lymph nodes and lungs.

 

       Soft tissue sarcomas may occur in children and adults. Soft tissue sarcomas that occur in children may respond differently to treatment and have a better prognosis than adult soft tissue sarcomas.

 

14. Synovial sarcoma

 

       The mention of synovial sarcoma brings to mind Wei Zexi. Synovial sarcoma is a soft-tissue sarcoma commonly found in children and adolescents. it is usually found in the tissues surrounding the joints of the hands and legs, but it may also form in the trunk, head, and neck. larger tumors have a greater chance of spreading. patients under 10 years of age and those with tumors less than 5 centimeters in diameter have a better prognosis. In patients with this tumor, there is a chromosomal translocation of cells.

 

       References:

       https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage&isappinstalled=0

 

 

       One of the things that must be known early on after a child's soft tissue sarcoma has been diagnosed, in order to determine the most appropriate treatment plan, is whether the cancer has spread to other parts of the body. This process used to find out if the cancer is in the soft tissue or has spread to other parts of the body is called staging.

 

       The following tests can be used to determine if a soft tissue sarcoma has spread:

 

● Sentinel lymph node biopsy

 

       A sentinel lymph node is the first lymph node to which the spread of cancer cells to metastasize occurs first. Sentinel lymph node biopsies are used to determine if cancer cells have spread to the lymph nodes.

 

       During the examination, the doctor will inject a small amount of radioactive material or blue dye near the tumor, which will flow through the lymphatic vessels to the lymph nodes. During surgery, the doctor removes the first lymph node that receives the radioactive substance or dye and hands it over to a pathologist to look for cancer cells under a microscope. If no cancer cells are found, it may not be necessary to remove more lymph nodes. This method is used for epithelioid and clear cell sarcomas.

 

● PET scan

 

       PET scans are used to find malignant tumor cells in the body.

 

       Malignant tumor cells will need a lot of energy because of their active metabolism, and glucose is the best energy substance for cellular metabolism, so scientists came up with the idea of injecting a small amount of radioactive glucose (sugar) into a vein, and as the blood carries this radioactive glucose around the tumor, the tumor cells will grab more of this glucose. At this point, we rotate around the body with a PET scanner and map out where the glucose passes through the body. Malignant tumor cells will appear brighter because they are more active and take up more glucose than normal cells. This method is also known as positron emission tomography (PET).

 

● PET-CT scan

   

       A method of combining PET scan images and CT tomography scans .PET and CT scans are done simultaneously on the same machine. The images from the two scans combined are more detailed than either one.

 

       Metastatic tumors are the same type of cancer as primary tumors. For example, if a soft tissue sarcoma spreads to the lungs, the nature of the cancer cells in the lungs are also soft tissue sarcoma cells. This is still metastatic soft tissue sarcoma, not lung cancer.

       

       There is no standard staging system for soft tissue sarcomas in children. There are two common clinical staging methods, both of which will be based on the following points:

 

1. Staging methods based on the amount of cancer cells remaining after tumor surgery and whether or not the cancer has spread

 

       This is a postoperative staging. Postoperative staging is based on complete resection, regional lymph node metastasis, residual meatus, and the presence of distant metastases, and is divided into 4 groups:

 

        Group 1 - Tumor was surgically removed completely

 

        Group 2 - After tumor removal surgery, remnants of cancer cells can only be seen microscopically at the margins of the removed tissue and/or adjacent lymph nodes

 

        Group 3 - Tumor remnants visible to the naked eye after surgery or biopsy but not spreading

 

        Group 4 - Tumor has metastasized (spread from its starting location to other parts of the body)

 

 

2. Staging methods based on tumor size and whether the tumor has spread to the lymph nodes or other parts of the body

 

       This staging system includes the following information:

 

Tumor size.

Whether the tumor has spread to the lymph nodes.

Whether the tumor has spread to other parts of the body.

       

       The following is a blow-by-blow description:

 

    (1) Stage I is divided into stages 1A and 1B:

 

● At stage 1A, the tumor is low-grade (tends to grow and spread slowly) and does not exceed 5 cm in size;

● At stage 1B, the tumor is low-grade (tends to grow and spread slowly) and is more than 5 cm in size.

 

 

         (2) Phase II is divided into 2 phases A and 2B:

 

                          ●  In stage 2A, the tumor is intermediate grade (slightly tends to grow and spread rapidly) or high grade (tends to grow and spread rapidly) and does not exceed 5 cm in size;

● In stage 2B, the tumor is intermediate grade (with a slight tendency to grow and spread rapidly) and is more than or equal to 5 cm in size.

 

 

         (3) Stage III tumors are one of the following:

 

● High level (tends to grow and spread rapidly) and is greater than 5 centimeters;

● Any grade or size and has spread to neighboring lymph nodes.

 

 

          (4) Stage IV:

 

● The tumor has spread to distal parts of the body, including the lungs. The tumor is of any grade or size and may have spread to adjacent lymph nodes.

 

 

       References:

        1. https://www.bjcancer.org/Mobile/Article/Index/104445

        2. https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage&isappinstalled=0

Clinical manifestations

 

       The most common symptom of soft tissue sarcoma in children is a painless lump or swelling of the soft tissues throughout the body.

 

       Sarcomas can present as painless lumps under the skin, often on the arms, legs or torso. There may be no other signs or symptoms at first, but when the sarcoma becomes large and presses on nearby organs, nerves, muscles or blood vessels, it may present with pain or weakness and weakness.

 

         However, the presence of pain, weakness or lumps is not the same as having a soft tissue sarcoma in children; there may be other reasons for these problems, so it is important to consult your child's doctor when any of these problems occur.

 

        Reference: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage& isappinstalled=0

 

Soft Tissue Function and Anatomy[1] 

       The soft tissues of the body primarily connect, support and surround other parts and organs of the body. Soft tissues include the following parts:

 

50. fat (in the body, in a plant, or in food)
50.  Mixing of bone and cartilage tissue
50. fibrous tissue
50. flesh
50. neuroscience
50. Tendons (bands of tissue that connect muscles to bones)
50. Synovial tissue (periarticular tissue)
50. vasculature
50. lymphatic channel

 

       Reference: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdqfrom=singlemessage&isappinstalled =0

 

 

 

Clinical Department

 

 

       Once you suspect that your child may have a soft tissue sarcoma, your doctor may perform the following tests on your child:

 

1. Physical examination and medical history

 

 

       A checkup for general health signs, including checking for signs of illness, such as lumps or other things that look unusual. The doctor will also ask questions to learn about the child's health habits and history of past illnesses and treatments.

 

2. Imaging

 

 

● X-ray examination: Using the principle that after X-rays penetrate the human body, different internal structures (organs, bones, fats, etc.) develop different images on the fluorescent screen or film, we can directly observe their anatomical and physiological functions as well as pathological changes.

 

● Computed Tomography (also known as CT or CAT scan): A CT scan shows detailed images of any anatomical part of the body, including bones, muscles, fat, and organs.CT scans provide clearer images than regular X-rays. During this test, sometimes the doctor will ask for medications to be drunk or injected through a vein first. These medications will accumulate in areas of active metabolism and, based on this property, will be able to better locate areas that may be tumors (one of the characteristics of tumor cells is that they are particularly metabolically active).

 

● Magnetic Resonance Imaging (MRI): A diagnostic technique that utilizes a combination of large magnets, radiofrequency principles, and computerized imaging for detailed images of organs and structures within the body.

 

● Ultrasound (also known as ultrasound): ultrasound is used to visualize the function of internal organs and to assess hemodynamic changes in blood vessels within a lesion.

 

3. Biopsies

 

 

        If the tests above show that there may be a soft tissue sarcoma, the doctor will next need to do a biopsy, which means that a portion of living tissue is taken from the body, especially from the area suspected of being a tumor, and removed for special pathology tests. There are several ways in which a biopsy can be done:

 

● Needle aspiration biopsy: Tumor tissue is aspirated with a thick needle. This procedure may be guided using ultrasound, CT scan or MRI.

 

● Excisional biopsy: cutting a portion of a tumor or tissue sample.

 

● Excision biopsy: Removing an entire area of mass or tissue that looks abnormal is often called a one-stage enlarged resection. The pathologist looks at the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors near the surface of the skin and can be done with negative margins. If cancer cells are still present, the cancer may come back or spread to other parts of the body.

      

       Doctors typically perform an MRI of the tumor before removing the biopsy. This is done to show where the original tumor is and can be used to guide future surgery or radiation therapy.

 

       The placement of the needle or incision in the biopsy can affect the success of later surgery to remove the tumor. Therefore, if possible, specialists in oncology, pediatrics, and pathology, all work together on the biopsy procedure.

   

       In order to develop the best treatment plan, the tissue sample removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and to perform other laboratory tests. Tissue samples may be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. The pathologist looks at the tissue under a microscope to look for cancer cells and to find the type and grade of the tumor. The abnormality of the cancer cells under the microscope and the rate at which the cells are dividing can help the doctor determine the grade of the tumor; high-grade and intermediate-grade tumors usually grow and spread more quickly than low-grade malignancies.

 

       Because there are so many types of soft tissue sarcomas that it is difficult to make a definitive diagnosis of a specific classification, excised tissue samples can usually be examined by an experienced pathologist with the participation of multiple departments or laboratories with different functions:

 

● Molecular testing: Because doctors need to know whether certain genes, proteins, or other molecules, or changes in certain genes and chromosomes, are present in the cut tissue (or blood, or other body fluids and tissues), molecular testing is needed, and this testing can help doctors identify certain types of soft tissue tumors.

 

● Reverse Transcription-Polymerase Chain Reaction (RT -PCR) assay: The major difference between this assay and the molecular assay is that it is primarily aimed at detecting changes in the expression of certain genes, i.e., instead of detecting the gene molecules themselves, it detects the products (proteins) of the molecules' expression, in order to reverse-engineer whether or not a certain molecule is present. This experiment is also designed to determine the type of tumor.

 

● Cytogenetic analysis: This test focuses on chromosomal abnormalities. The doctor will look at cells in bone marrow, blood, amniotic fluid, tumors, or other tissue samples under a microscope to look for chromosomal changes. Fluorescence in situ hybridization (FISH) is a method of cytogenetic analysis.

 

● Immunocytochemistry: It is known that antigens and specific antibodies pair up. So, this test uses this principle, the doctor who suspects the presence of a particular antigen in the child's body uses antibodies to check for certain antigens (markers) in the cell samples, once such antigens are present they pair up, and the antibodies prepared by the doctor are usually associated with an enzyme or a fluorescent dye, and when they pair up, this fluorescent dye, develops a color under the microscope so that cells with the specific antigens become visible.

 

       Reference: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage& isappinstalled=0

 

 

 

Examination & Diagnosis

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Clinical Management

 

 

1. Some factors that affect prognosis (chances of recovery) and treatment options:

 

 

● The part of the body where the tumor first formed

● Tumor size and grading

● Types of soft tissue sarcoma

● How deep is the tumor under the skin?

● Whether the tumor has spread to other parts of the body

● Number of tumors remaining after surgery

● Whether or not radiation therapy has been administered

● Age and sex of patients

● Cancer is first diagnosed or recurrent

 

2. Treatment options for soft tissue sarcomas:

 

 

       There are eight common and different types of treatment options for soft tissue sarcomas:

 

● surgery

● radiotherapy

● radiotherapy

● Watching and waiting

● hormone therapy

● NSAID therapy

● targeted therapies

● immunotherapy

 

       There are also new treatments that are in clinical trials, such as gene therapy. Eight different types of common treatment options are elaborated on next:

 

       2.1 Surgery

 

       When possible, try to achieve complete surgical removal of soft tissue sarcomas. If the tumor is too large, preoperative radiation therapy or chemotherapy may be given first to make the tumor smaller, which will reduce the number of organs or tissues that need to be removed during surgery. This is called neoadjuvant therapy. Common surgical procedures are:

 

● Wide local excision: removal of the tumor and its surrounding normal tissue.

● Amputation: Surgery to remove all or part of a limb or appendage that has cancer, such as an arm or hand.

● Lymph node dissection: removal of lymph nodes with metastatic cancer cells.

● Mohs (MOHS) Surgery: Mohs surgery, also known as MOHS microsurgical technique, is currently the surgical procedure with the lowest recurrence rate in the treatment of cutaneous malignant tumors. The greatest advantage is that it allows for complete visualization of all tumor boundaries, certainty of a clean tumor excision, and precise control of the excision boundaries. The general approach is that a single focal lesion of cancer is excised and then the margins are quickly examined under the microscope, and while it is cut down, it is sent for examination to ensure that the pathologic results are promptly fed back to the surgical operator, who is then guided to the next step in the operation until all the cancerous tissue has been excised. This type of surgery is used to treat bulging cutaneous fibrosarcoma.

 

       Some patients with soft tissue sarcomas may require a second surgery during the course of treatment, such as those listed below:   

 

● To remove all residual tumor cells from the tissue.

● In Mohs surgery, the margins of the tumor being removed are checked to see if they are negative, and if the margins are positive, more tissue needs to be removed.

● If the tumor occurs in the liver, a hepatectomy or liver transplant (where the liver is removed and replaced with a healthy liver from a donor) may be needed.

 

       After the surgeon removes the tumor tissue that was visible to the naked eye at the time of surgery, it is sometimes necessary to continue postoperative chemotherapy or radiation to ensure that other remaining cancer cells are killed. This treatment given after surgery to reduce the risk of the cancer coming back is called adjuvant therapy.

 

       2.2 Radiotherapy

 

       Radiation therapy, or radiotherapy for short, is an anti-tumor treatment that uses high-energy X-rays or other types of radioactive sources to kill cancer cells or stop them from growing. There are two types of radiation therapy:

 

● External radiation therapy, which involves using a machine outside the body to send radiation particles to tumor tissue. Certain methods are used to help prevent radiation damage to nearby healthy tissue, such as stereotactic body radiation therapy. Stereotactic body radiation therapy uses a special device so that the child is held in the same position for each treatment. Once a day, for several days, the tumor tissue receives a larger than usual dose of radiation. Because the child is immobilized each time he or she is treated, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external beam radiation therapy or stereotactic radiation therapy.

 

● Internal irradiation radiotherapy is a radiotherapy treatment in which radioactive particles are implanted directly into tumor tissue or paraneoplastic tissue by means of needling, embedding, or catheterization.

 

       The choice of the type of radiation therapy depends on the type of tumor, the stage of the tumor at the time of initial treatment, and whether or not the tumor is completely resected. Both external and internal irradiation may be used to treat soft tissue sarcomas in children.

 

       2.3 Chemotherapy

 

       Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by killing the cells or stopping them from dividing. Taken by mouth or injected into a vein or muscle, chemotherapy drugs enter the bloodstream and can reach cancer cells throughout the body (known as systemic chemotherapy). When chemotherapy drugs are injected directly into the cerebrospinal fluid, an organ, or a body cavity, such as the abdomen, the drugs primarily affect tumor cells in a localized area (i.e., local chemotherapy). Combination chemotherapy is chemotherapy that combines more than one chemotherapy drug. The choice of chemotherapy depends on the type of tumor being treated.

 

       However, this is not the best approach for most types of soft tissue sarcomas, which do not respond to chemotherapy.

 

       2.4 Watchful waiting

 

       This refers to closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. These are the following conditions that may need to be observed first:

 

● Inability to remove the tumor completely.

● There is no other treatment available.

● The tumor is unlikely to damage any vital organs.

 

       2.5 Hormone therapy

 

       Hormones are substances produced by the body's endocrine glands, which circulate and act in the blood system. Certain hormones can cause certain cancers to grow. If tests show that the cancer cells have receptors that the hormones can attach to, then these means such as drugs, surgery or radiation therapy can be used to reduce the production of the hormones or stop them from working. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops tumor cells from growing. Anti-estrogen drugs (drugs that block estrogen), such as tamoxifen, might be used to treat sclerofibromatosis.

 

       2.6 Non-steroidal anti-inflammatory drug therapy

 

       Nonsteroidal anti-inflammatory drugs (NSAIDs) are a class of medications (such as aspirin, ibuprofen, and naproxen) commonly used to reduce fever, swelling, pain, and redness. In the treatment of sclerofibromatosis, an NSAID called sulforaphane may be used to help stop the growth of tumor cells.

 

       2.7 Targeted therapy

 

       Targeted therapy is a treatment that uses drugs or other substances to attack cancer cells. Targeted therapy usually causes less damage to normal cells than chemotherapy or radiation.

 

       2.7.1 Kinase inhibitors

 

       Kinase inhibitors are targeted therapies that block a class of enzymes (a type of protein) called kinases. There are different types of kinases in the body and they have different effects.

 

      (1) Mesenchymal lymphoma kinase (ALK) inhibitors stop the growth and spread of tumors such as:

 

● Crizotinib is used to treat inflammatory myofibroblastoma.

 

      (2) Tyrosine kinase inhibitors (TKIs) block signals required for tumor growth, e.g:

 

● Imatinib is used to treat bulging cutaneous fibrosarcoma.

● Pazopanib is used to treat sclerofibromatosis and certain types of recurrent and refractory soft tissue sarcomas. Its effectiveness in many types of newly diagnosed soft tissue sarcomas and some other types of recurrent soft tissue sarcomas is also being studied.

● Sorafenib is used in the treatment of sclerofibromatosis.

 

       Novel tyrosine kinase inhibitors are being investigated that also have a role in some tumors, such as:

 

● LOXO-101 and Entrectinib for Infantile Fibrosarcoma.

● Trametinib is used in the treatment of epithelioid hemangioendothelioma.

 

       2.7.2 Other types of targeted drugs

 

       Other types of targeted therapies are also being studied, such as the following:

 

● mTOR inhibitors, are targeted therapies that block proteins that promote cell division and survival. mTOR inhibitors are currently used for two types of tumors: pro-connective tissue proliferative small round cell tumors, perivascular epithelioid cell tumors (PEComa), and epithelioid angioendotheliomas. Sirolimus is a representative drug for mTOR inhibitor therapy.

 

● Angiogenesis inhibitors, which are targeted therapeutic agents that can block tumor neovascularization. Angiogenesis inhibitors, such as sildenafil, sunitinib and thalidomide, are being investigated for their therapeutic effects on vesicular soft tissue sarcomas and epithelioid cell hemangioendotheliomas; and the therapeutic effect of bevacizumab on vascular tumors is also being studied.

 

● Histone methyltransferase (HMT) inhibitors, a class of targeted therapeutic agents that act within tumor cells and block signals necessary for tumor growth.HMT inhibitors, such as tazemetostat, are being investigated for the treatment of epithelioid sarcomas, malignant peripheral nerve sheath tumors, extra-renal (extracranial) rhabdomyosarcoma-like tumors, extraskeletal mucinous chondrosarcomas, and synovial sarcomas.

 

● Heat shock protein inhibitors that block certain proteins that protect tumor cells and help them grow.Ganetespib, a class of heat shock protein inhibitors, is being studied in combination with the mTOR inhibitor sirolimus for the treatment of unresectable malignant peripheral nerve sheath tumors.

 

● An antibody-drug coupling consists of a monoclonal antibody attached to a drug. A monoclonal antibody (English: monoclonal antibody, abbreviation: mAb), or monoclonal antibody for short, is an antibody made by only one type of immune cell, and is named to distinguish it from a polyclonal antibody (one antibody made by many types of B cells). Antibody-drug couplings that can enter these cells and kill them without harming other cells.Lorvotuzumab mertansine is one such drug that is being studied for the treatment of rhabdomyosarcoma, malignant peripheral nerve sheath tumors, and synovial sarcoma.

 

       2.8 Immunotherapy

 

       The basic principle of immunotherapy is the use of the patient's own immune cells to remove cancer cells, a treatment that, either by stimulating the body to produce, or through the use of medications, promotes the body's restoration of its natural defenses against the disease. Interferon, for example, is an immunotherapy that has been used to treat epithelioid hemangioendothelioma. It interferes with the division of tumor cells and can slow tumor growth.

 

 3. New treatments under research

 

 

● gene therapy

 

       For treating synovial sarcoma in children that has recurred, spread, or cannot be surgically removed, gene therapy may have a silver lining. T-lymphocytes (a type of white blood cell) are taken from a number of children's bodies and their genes are modified (genetically engineered to be modified) in the laboratory and then infused back into the child through an intravenous infusion so that they will attack specific tumor cells.

 

       The treatments mentioned above are not necessary for every child suffering from childhood soft tissue sarcoma, and not all methods will be used on one child; the doctor will work out a suitable program for the child based on their actual condition.

 

4. On clinical trials

 

 

       The vast majority of conventional treatments for cancer now in use are the result of previous clinical trials. Clinical trials are designed to help improve current treatments or to obtain information about new treatments for tumor therapy. When a clinical trial shows that a new treatment is superior to the standard treatment, the new treatment may become the standard treatment.

 

       Since malignant tumors in children are rare, participation in a clinical trial should be considered when a diagnosis of childhood soft tissue sarcoma is made. Depending on the purpose of the trial, clinical trials can be conducted at any point in the course of antitumor therapy, before, during, or after antitumor therapy, although there are some clinical trials that are only open to first-time patients.

 

       Participation in a clinical trial does not necessarily mean that the new method will be applied; depending on the method of the study, participants may be assigned to receive conventional treatment (which has been shown to be safe and effective) or they may be assigned to the new method group. For some people, participation in a clinical trial may be the optimal treatment option.

 

       Clinical trials are being conducted in many areas. Check the Treatment Options section for information on clinical trials that are currently underway. These can be found in the NCI Clinical Trials List.

 

       More details on clinical trial information can be found at: http://www.cancer.gov/clinicaltrials

       

       Regardless of the treatment method chosen, it is essential to follow up with regular checkups as ordered by your doctor during the course of treatment.

 

       Some tests used to diagnose the stage of cancer may need to be repeated; tests used to determine whether treatments are working may also need to be repeated. Some clinical trials require regular follow-up even after treatment has ended, and the results of these tests will tell you if your child's condition has changed or if the cancer has returned. This is called retesting.

 

       Cancer treatments can cause side effects because treating tumor cells also affects healthy tissues or organs. Information about the side effects of cancer treatments can be found in the Children's Palliative and Hospice Care section under 3. Symptom Management.

 

       Don't give up on treatment because you are worried about side effects, as your doctor will have ways to deal with them, versus letting your doctor help you and your child through this time.

 

       It is also important to note that a proportion of cured children will experience side effects months or even years after the end of treatment. These side effects are often caused by a delayed reaction to damage to tissues and organs caused by surgery or radiotherapy, hence the name delayed side effects.

 

       Although most delayed side effects are not life threatening, they can have a very negative impact on a child's health and quality of life. Control of delayed side effects can be achieved through routine post-treatment follow-up and good lifestyle habits, so it is important to promptly communicate with your child's doctor about possible side effects of treatment.

 

       For more information on delayed side effects see Long-term effects of pediatric oncology treatments.

 

       Tip:

 

       The treatment-related information provided by Sunflower Kids is for informational purposes only and should not be used as a substitute for the advice of your doctor and other medical professionals, nor for an in-person doctor's visit. If you have specific questions about a disease, treatment options, diagnosis or clinical symptoms, please seek the help of a health care professional.

 

        References:

        https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq?from=singlemessage&isappinstalled=0

 

 

Prognosis

 

 

       After treatment for soft tissue sarcoma is completed, talk to your doctor about a follow-up care plan, which often includes regular physical exams and/or specialized testing to track recovery from treatment.

 

       Regular follow-up visits with your doctor are an important part of your treatment to detect tumors that may recur early, and to control as well as help mitigate the side effects of treatment. The usual frequency of follow-up visits is every three to four months for three years after treatment, every six months for three to five years, and once a year after five years. Usually at the follow-up visit, the doctor will perform a chest X-ray or CT scan to detect any cancer cells that have metastasized to the lungs. Occasionally, the doctor will also perform imaging tests on the primary site of the patient's tumor, which usually includes an MRI, ultrasound testing, CT scan and/or positron emission tomography scan.

 

       After treatment for soft tissue sarcoma is completed, patients need to report any new symptoms, such as coughing, pain, or new lumps, to their doctor. Some of these symptoms may be signs that the cancer has come back, or they may be after-effects of the treatment, or of course they may be unrelated to the cancer. If a soft tissue sarcoma returns, it usually occurs within two years of treatment, but some soft tissue sarcomas may return much later.

 

       Patients undergoing radiation therapy are at risk of developing swelling of the limbs (called lymphedema), susceptibility of the leg bones to fracture, decreased mobility of the joints, and sclerosis (fibrosis) of the soft tissues in the part of the body that is being treated with radiation therapy. In rare cases, radiation therapy may also cause the patient to develop a soft tissue sarcoma in a different area than the primary tumor. Parents should consult with their doctor to try to manage and avoid these side effects.

 

       Effective treatments such as using compression stockings or other targeted therapies to control lymphedema; avoiding high-intensity exercise to prevent fractures; repairing joint mobility through a rehab program; and taking vitamin E and pentoxifylline (an oral medication) by mouth can produce a significant improvement in fibrosis after a few months. In addition, patients who have undergone radiation therapy should try to minimize direct ultraviolet radiation from the sun through clothing or sunscreen, which will reduce the likelihood of developing skin cancer in the part of the body that has received radiation therapy.

 

       Patients who have been treated for soft tissue sarcomas in the upper and lower extremity areas may be able to regain and maintain motor function in the limbs through rehabilitation programs after surgery. Limited range of motion, strengthening exercises, and programs for lymphedema are often recommended.

 

       Maintaining good limb motor function in patients who have received treatment for soft tissue sarcoma is very important to ensure a good quality of life, therefore, after treatment, a rehabilitation specialist should be considered to help the child recover. On the other hand, for children whose limbs have been amputated in the course of receiving treatment, prosthetic repair should be considered as soon as possible, and in addition, psychologists need to intervene, if necessary, to help the patients adjust to life after amputation.

 

       The American Society of Clinical Oncology (ASCO) offers a Cancer Treatment Summary Sheet to help patients keep track of their treatment time and develop a wellness plan for post-treatment care.

 

       It is recommended that patients with soft tissue sarcoma maintain an appropriate weight, avoid smoking environments, and ensure a healthy diet while following doctor's orders to complete cancer screenings to ensure a healthy life during recovery from treatment. Please work with your child's doctor to develop a recovery plan that works best for you. For example, proper physical activity can help restore your strength and energy. Your doctor can help you develop an appropriate exercise program based on your child's physical condition, health and needs. The website links below give you more information on guidance for life after surgery, including positive lifestyle changes.

 

       References:

        http://www.cancer.net/cancer-types/sarcoma-soft-tissue/after-treatment

 

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

 

 

       Doctors are working to research more information about sarcomas, the best ways to prevent them, the best treatments, and the best care for diagnosed patients. Through clinical trials, the following areas of research may provide new options for patients. Again, communication should be maintained with your physician regarding the diagnosis and the best treatment options.

 

● Proton therapy: Proton therapy is a method of external radiation therapy that uses protons instead of X-rays. High-energy protons destroy cancer cells and are used in Japan and Germany for sarcoma treatment research.

 

● Enhanced drug delivery: Some chemotherapeutic drugs are loaded into fat molecules called liposomes to enhance the absorption and distribution of the drug in the patient's body.

 

● Novel chemotherapeutic agents: New chemotherapeutic applications are being developed and tested for the treatment of cases such as trabectedin (Yondelis), which has been licensed for use in Europe. Trabectedin has shown high activity in the treatment of a specific subtype of liposarcoma, mucinous-round cell sarcoma. However, in the United States, the FDA has only licensed the drug for use in certain circumstances. Meanwhile, eribulin (Halaven), which is licensed for breast cancer treatment, has performed well in early trials in Europe and is now being tested on a larger international scale. Researchers are also working to find versions of standard chemotherapy drugs that treat sarcomas with fewer side effects. For example, two new isocyclophosphamides, glucophage and TH-302, are being used in clinical trials.

 

● Targeted therapies: As explained earlier in the treatment section, a number of targeted therapies have recently been licensed for the treatment of specific sarcoma subtypes, and targeted therapies overall have been the subject of active research. Researchers are now identifying new kinase inhibitors and evaluating the order of dosing in therapy. For example, regorafenib is a novel targeted agent frequently used to treat patients who have been treated with imatinib and sunitinib. The U.S. Food and Drug Administration is currently reviewing phase III clinical trials for this drug.

 

       Other clinical trials are looking at a targeted treatment called antivascular therapy. This approach focuses on stopping the growth of blood vessels, which are needed to transport nutrients as tumors grow and spread. The goal of antivascular therapy is to "starve" cancer cells.

 

● Targeted gene therapy: drugs being studied that block one or more proteins that help tumors grow and spread.

 

● Oncogenetic research: Researchers are studying the specific genetic "fingerprints" that some sarcomas have. A large number of cancer centers and companies are now offering genetic tests for cancer, and by understanding the results of these tests, patients can be helped to decide on better treatment options and possibly better predict the progression of their disease.

 

● Supportive care: Clinical trials are underway to find better options to relieve symptoms and reduce side effects of existing treatment options, with the goal of improving patients' quality of life.

 

       References:

        http://www.cancer.net/cancer-types/sarcoma-soft-tissue/latest-research

 

 

 

 

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