Osteosarcoma (benign tumor composed of bone-like material)

Summarize

Osteosarcoma is the most common type of primary malignant tumor of bone and accounts for approximately 5% of childhood tumors. Osteosarcoma occurs mainly in adolescents . It often occurs in areas of rapid bone growth, such as the epiphyses of long bones (the epiphysis is the junction of the stem and the epiphysis), and is common above and below the knee, i.e., the distal femur and the proximal tibia, and to a lesser extent, the proximal humerus. However, osteosarcoma can occur in virtually any bone, including the pelvis (hip), shoulder, and jaw.

Epidemiological

Currently, the main treatment for osteosarcoma is surgery combined with chemotherapy-based comprehensive treatment. Osteosarcoma is highly malignant and often metastasizes, with lung metastasis predominating.The 5-year survival rate for patients under 15 years of age is about 76%, and for patients aged 15-19 years, the 5-year survival rate is about 66%.

Etiolog & Risk factors

1. Etiology

For most cases of osteosarcoma, the exact cause is unknown. Mutations in some genes are thought to be associated with the risk of developing the disease.

2. Risk factors

Any factor that increases the risk of developing a disease is called a risk factor. Having a risk factor does not mean that you will definitely get a tumor; not having a risk factor does not mean that you will definitely not get a tumor.

Risk factors for osteosarcoma include.

(1) Age:

The highest risk of osteosarcoma occurs in the age group between 10 and 30 years, especially during the developmental explosion of adolescents, suggesting a link between rapid bone growth and risk of tumorigenesis.

(2) Height:

Children with osteosarcoma are taller than their peers, again suggesting that osteosarcoma may be associated with rapid bone growth.

(3) Gender:

Osteosarcoma is most often seen in males. If it develops in women, it tends to occur at an earlier age, probably because they have an earlier period of the height bulge.

(4) Past treatment with radiation therapy:

Patients who have received radiation therapy to treat other tumors are at a higher risk of developing osteosarcoma near the area of radiation therapy. Receiving high doses of radiation at a young age increases the risk of developing osteosarcoma.

(5) Past treatment with chemotherapeutic agents of anthracycline or alkylating agents.

(6) There are specific changes in the RB1 gene.

(7) Patients with the following hereditary syndromes:

● Bloom's disease (Bloom's syndrome): a rare single-gene autosomal recessive disorder caused by mutations in the BLM gene, with symptoms of short stature and susceptibility to tumors.

:: Diamond-Blackfan anemia (congenital pure red blood cell aplastic anemia): a genetic disorder in which anemia is the predominant manifestation and involves tissues of multiple systems.

● Li-Fraumeni syndrome (LFS): Mostly caused by inherited oncogenic P53 mutations, patients are prone to develop several types of tumors, including breast cancer, brain malignancies, osteosarcoma, and other types of sarcomas.

● Paget's disease (Paget's disease): a rare skin cancer that originates in intradermal or subcutaneous glands and tends to present with dermatitis-like flushing, oozing, and crusting of the skin.

:: Hereditary retinoblastoma: an ocular malignancy originating in the retina, the light-sensitive layer of the eye wall, which can occur in one or both eyes.

● Rothmund-Thomson syndrome (congenital angioatrophic cutaneous heterochromatosis): caused by mutations in the REPL4 gene, this is a multisystemic genetic disorder with onset in the first 3-6 months of life. Typical onset of symptoms is a red, edematous patch on the cheeks, which can spread to other parts of the body, and the patient has scanty hair, eyebrows, and eyelashes.

● Werner syndrome (cataract-scleroderma-premature aging syndrome): a rare genetic disorder, also known as progeria dwarfism, adult progeria syndrome, adult progeria, etc. Typical symptoms are an aged appearance of the skin, gray hair and loss, cataracts in the eyes, short stature, and arrested growth.

As can be seen, the vast majority of known risk factors are innate, including age, height, race, gender, specific bone disorders and inherited syndromes. None of these factors can be altered. Other than radiation therapy, it is not clear what lifestyle habits or environmental factors contribute to the development of osteosarcoma, so there is no way to effectively avoid osteosarcoma.

Classification & Stage

Different osteosarcoma subtypes can be identified by radiographic and pathologic findings. Some of these subtypes have a better prognosis than others. Depending on their pathologic findings, osteosarcomas can be classified as high-grade, intermediate-grade, or low-grade. Tumor grade indicates the ability of the tumor cells to grow and spread.

1. High-grade osteosarcoma

They are a fast-growing type of osteosarcoma. Under the microscope, they differ from normal bone and have many cells that are in the dividing stage. Most osteosarcomas that occur in children and adolescents are high-grade.

There are many types of high-grade osteosarcoma (the first three are the most common):

:: Osteoblastic

● Chondrocytic

● Fibroblastic

:: Hybrid

:: Small cell type

● Capillary Dilatation

:: High-level superficial or high-level paracortical type

Other high-grade osteosarcomas include:

:: Paget's disease-like type: tumors that develop in the bones of patients with Paget's disease

:: Extraosseous: tumors that occur not in bones (but in other parts of the body)

:: Post-radiation type: tumors occurring in bones that have been treated with radiation therapy

2. Intermediate grade osteosarcoma

This portion of the uncommon tumor type is classified between high-grade and low-grade (often treated as low-grade osteosarcoma):

Periosteal or mid-level paracortical type

3. Low-grade osteosarcoma

The slowest growing type of osteosarcoma. Under the microscope tumors resemble normal bone more closely and contain fewer cells in the differentiation cells in the stage of division.

:: Extraperiosteal or low-grade paracortical type

:: Intramedullary or intraosseous highly differentiated or low-grade centralized type

Clinical manifestations

1. Pain in the bone or joint area

Pain in the bones affected by the tumor (usually around the knee or upper arm) is the most common symptom of osteosarcoma. Initially, the pain may not be constant and only gets worse at night (called nocturnal pain). The pain is often worse during exercise.

2. Localized lumps and or soft tissue swelling

Becoming swollen near the affected area is another common occurrence with osteosarcoma, with swelling often occurring weeks after the bone pain begins. Depending on where the tumor arose, a lump or localized bulge may be palpable.

3. Unexplained fractures (pathological fractures)

Although osteosarcoma can weaken the bones it involves, these bones usually do not break. In contrast, although capillary dilatation osteosarcoma occurs very rarely, this type of osteosarcoma affects the bones more than other osteosarcomas, making the bones involved in the tumor more fragile and thus more likely to fracture or break.

Clinical Department

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Examination & Diagnosis

1. Diagnostic strategy

Diagnosis of osteosarcoma follows a combination of clinical, imaging, and pathology. In most cases, MDT (multidisciplinary consultation) is recommended for diagnosis and treatment.

2. Common checks

:: Physical examination and history taking: Checking the general condition of the body and checking for signs of disease, including lumps or other abnormalities. The medical history will include past illnesses, treatment, and family history.

● Complete Blood Count (CBC): Some patients may present with anemia and other manifestations. Blood will need to be drawn to check the number of red blood cells, white blood cells and platelets; the amount of hemoglobin in the red blood cells (hemoglobin is the protein that carries oxygen), and the percentage of red blood cells in the blood sample.

● Blood biochemistry: Biochemical tests for alkaline phosphatase are often elevated in patients with osteosarcoma. Blood is drawn to test the amount of certain substances released into the blood by body organs and tissues. Abnormal levels of a substance (above or below normal) may be a sign of disease. This test is done to check that the liver and kidneys are functioning properly.

● X-rays: The location and size of the tumor growth can be observed, and the tumor may appear uneven rather than solid on the film, or it may appear as an intraosseous cavity. Sometimes the doctor can observe the tumor near the intraosseous defect extending into the surrounding tissue (e.g., muscle or fat tissue). Typical periosteal reactions, including Codman's triangle and sunlight-scattering periosteal reactions, can be seen on X-rays. Chest X-rays can be used to determine if the osteosarcoma has spread to the lungs.

● CT scanning: examine the tumor from various fault lines, and three-dimensional imaging and enhanced scanning are feasible to observe the blood circulation of the tumor and the relationship between the tumor and blood vessels.

● MRI: Magnetic resonance is advantageous for the visualization of tumor boundaries and edema bands.

● PET-CT scan or bone scan: helps to look for tumors throughout the body. This technique combines a positron emission tomography (PET) scan and a computed tomography (CT) scan, both performed at the same time on the same machine, combining the two images to produce a more detailed picture than the two tests themselves. A small amount of radioactive glucose is injected into a vein during the test and the uptake of glucose in the body is photographed. Malignant tumor cells appear brighter in the images relative to normal cells because they are more active and take up more glucose than normal cells.

:: Radionuclide bone scan: used to check for rapidly dividing cells, such as tumor cells, in bones. A very small amount of radioactive material is injected into a vein and transported throughout the body in the bloodstream. The radioactive material collects in bones with tumor cells and shows up as dense, gray to black areas called "hot spots". This can help indicate how much damage the tumor has done to the bone and whether it has spread to other bones.

:: Biopsy: Biopsies are categorized into puncture biopsies and incisional biopsies. The advantage of puncture biopsy is that it is less invasive, but the diagnostic tissue obtained is smaller and more difficult to diagnose. Incisional biopsy has the advantage of obtaining enough tissue for diagnosis, but is more traumatic and carries the risk of tumor contamination. Regardless of the type of biopsy, attention should be paid to the access to the material, and the entire tumor and pathology biopsy area should be removed during radical surgery.

Classification & Stage

The process used to find out if a tumor has spread to other parts of the body is called staging. Staging is also related to the site of origin. For osteosarcoma, most patients are staged based on whether the tumor is in a part of the body (localized) or has spread (metastasized).

There are several staging methods for osteosarcoma, including simple staging, Enneking's surgical staging method, and the AJCC's TNM staging.

Staging usually requires the following tests:

:: X-rays

:: CT scan

● PET-CT scans: more sensitive and accurate than bone scans for detecting bone metastases

● MRI Magnetic Resonance Imaging

:: Bone scans

1. Simple phasing

Depending on the need to develop a treatment plan, osteosarcomas can be simply classified into localized and metastatic tumors.

1.1 Localized osteosarcoma

There is no spread out of the bone where the tumor occurred. There may be one or more areas of tumor in the bone that can be removed during surgery. Approximately half of the tumors occur in the femur, with 80% located in the distal femur. The other major sites in descending order of frequency are the proximal tibia, proximal humerus, pelvis, jawbone, fibula, and ribs.

1.2 Metastatic Osteosarcoma

Tumors spread to other parts of the body from the bone where the disease began. Osteosarcoma most often spreads to the lungs and may also spread to other bones. The spread of a tumor from where it may have started to other parts of the body is called metastasis. Tumor cells leave the place where they started (the primary tumor) and spread through the lymphatic system or bloodstream. Metastatic tumors are the same type of tumor as the primary tumor. For example, if osteosarcoma spreads to the lungs, the tumor cells in the lungs are actually osteosarcoma cells. The disease is metastatic osteosarcoma, not lung cancer.

2. Enneking Surgical Staging (MSTS staging)

Enneking staging was developed by the Musculoskeletal Tumor Society (MSTS) and is determined by three key pieces of information:

(1) Tumor Grading (Grade, G): Tumor grading is an assessment tool that describes the ability of a tumor to grow and spread based on microscopic images. Tumors are classified as low grade (G1) and high grade (G2). Low-grade tumor cells are more similar in appearance to normal cells and are less likely to grow and spread rapidly, while high-grade tumor cells are more heterogeneous, grow rapidly, and spread easily.

(2) Degree of local tumor invasion (Tumor, T): T1, the limited type, indicates that the tumor remains essentially within the bone; T2, the invasive type, indicates that the bone has extended beyond the bone and into other adjacent structures.

(3) Whether the tumor has metastasized to adjacent lymph nodes or other organs (Metastasized, M): Tumors that have not yet spread to lymph nodes or other organs are M0, while those that have spread are M1.

Combining these factors and numbering them with Roman numerals I through III, a composite staging can be determined.Stages I and II are further classified into two categories, A (confined tumors) and B (invasive tumors).

Overall:

● Low-grade, localized tumors are in stage I.

:: High-grade, localized tumors are in stage II.

● Metastatic tumors (regardless of grade) are in stage III.

3. TNM staging

The American Joint Commission on Cancer (AJCC) system stages all osteosarcomas. It combines 4 factors for staging, capitalized as T, N, M and G.

● T stands for the characteristics of the tumor (Tumor) (its size and whether it is present in more than one place in the bone);

● N stands for invasion into the lymph node (Lymph Node);

● M stands for metastasis to distal organs (Metastasis);

● G stands for tumor grade (Grade).

Tumor grading is based on the degree of abnormal cell morphology on microscopic examination, with the higher the grade, the more abnormal the cell morphology. High-grade tumors tend to grow and metastasize more rapidly than low-grade tumors.

The process of integrating information about tumor characteristics, lymph node invasion, tumor metastasis and tumor grading into one is called staging grouping. Each stage can be further divided by Roman numerals into stages I through IV.

3.1 T-staging of bone tumors

TX: Primary tumors can't be measured

T0: no sign of tumor presence

T1: Tumor no larger than 8 cm

T2: Tumor greater than 8 cm

T3: Tumor occurs more than once in the same bone

3.2 N-staging of bone tumors

N0: tumor has not metastasized to adjacent lymph nodes

N1: The tumor has metastasized to adjacent lymph nodes

3.3 M-staging of bone tumors

M0: Tumor has not metastasized to extraosseous or adjacent lymph nodes

M1: Distal tumor metastasis (tumor has spread)

M1a: Tumor metastasized only to the lungs

M1b: Tumor has metastasized to other sites (e.g., brain, liver, etc.)

3.4 Bone tumor grading (G)

GX: Not classifiable

G1-G2: low level

G3-G4: High level

4. Recurrence

Osteosarcoma most often recurs in the lungs, bones, or both. Osteosarcoma recurrence often occurs within 18 months of completing treatment.

Clinical Management

Treatment of osteosarcoma has come a long way in the last few decades. In the 1960s, amputation was the only treatment option, and only a very small number of patients survived more than two years after diagnosis. Since then, doctors have found that chemotherapy before and after surgery has been able to treat many patients with osteosarcoma, which has led to limb-sparing surgeries for many patients who would have otherwise required amputation.

Treatment of osteosarcoma includes standard treatment and clinical trials. Standard treatment refers to the current conventional therapy, which in most cases is surgery combined with chemotherapy as the main treatment. Clinical trials are designed to improve current treatment or to gain information about new treatments. Due to the rarity of malignant tumors in children, participation in a clinical trial may be considered if standard treatment options are not effective. It is important to note that some clinical trials are only open to patients who have not yet begun treatment, so it is important to consult and communicate with your doctor in a timely manner.

1. Standardized treatment programmes

Standard clinical treatment is based on surgery and chemotherapy.

1.1 Surgery

The goal of surgery is to remove the entire tumor if possible. Chemotherapy may be given before surgery to shrink the tumor. This is because if less bone tissue needs to be removed, there are relatively fewer problems after surgery.

Types of surgery include:

1.1.1 Extensive local excision

Surgery to remove the tumor and some healthy tissue around it.

1.1.2 Limb-sparing surgery

Tumors are removed from a patient's limb (arm or leg) without amputation, therefore preserving the appearance and function of the patient's limb. Most patients with osteosarcoma of the extremities can be treated with limb-sparing surgery. It is also possible to replace the removed tissue and bone with a graft from a part of the patient's own body or artificial bone. If a fracture occurs at the time of diagnosis, before surgery, or during chemotherapy, limb-sparing surgery may still be possible in some cases.

1.1.3 Amputation

Surgery to remove part or all of an arm or leg. This may be done when the patient's tumor is large and encroaches on nerves and/or blood vessels, and not all of the tumor can be removed in a limb-sparing surgery. Patients may be fitted with a prosthesis after amputation.

1.1.4 Rotational molding

Surgery to remove the tumor and the knee. The leg below the knee is retained instead of the part of the leg above the knee that was removed, and then the foot faces backward with the ankle acting as the knee. A prosthesis is then attached to the foot (currently rare).

After surgery to remove all visible tumor, patients may receive chemotherapy to kill tumor cells that may remain locally or spread to other parts of the body to reduce the risk of recurrence, called adjuvant therapy.

Postoperative rehabilitation may be an integral part of all treatment. Children and their parents need to discuss treatment options and post-operative arrangements with the rehabilitation physician prior to surgery. In the case of a thigh amputation, the child will need to learn to live with a prosthesis. This is particularly difficult for growing children, as the prosthesis needs to be adjusted from time to time to keep up with their growth. After limb-sparing surgery, the situation is sometimes more complicated. Growing children may need more follow-up surgeries to replace the endoprosthesis to match their growing size.

1.2 Chemotherapy

Chemotherapy is a commonly used treatment for malignant tumors, in which the growth of tumor cells is controlled by drugs that kill the cells or prevent them from reproducing. Chemotherapy is a very important treatment for most patients with osteosarcoma.

Systemic chemotherapy is when drugs are given orally or intramuscularly or intravenously, where they enter the bloodstream and reach the entire body. Localized chemotherapy is when the drug is administered directly into the cerebrospinal fluid\an organ or a body cavity (e.g., the abdominal cavity), where it works locally.

Patients with osteosarcoma tend to have about 3 cycles of chemotherapy, called neoadjuvant chemotherapy, before surgery to remove the primary tumor. About 8 cycles of chemotherapy are given after surgery.

The most commonly used drugs include: methotrexate (along with folinic acid to prevent side effects when used in high doses), adriamycin, cisplatin or carboplatin, epirubicin, isocyclophosphamide, cyclophosphamide, etoposide, gemcitabine, or topotecan.

Combination chemotherapy refers to the use of more than one anticancer drug at the same time. Common combinations include: high-dose methotrexate, adriamycin, and cisplatin (sometimes isocyclophosphamide); adriamycin and cisplatin; isocyclophosphamide and etoposide; and isocyclophosphamide, cisplatin (or carboplatin), and epirubicin.

2. Other therapies

2.1 Radiotherapy

Patients with osteosarcoma may be treated with external radiation therapy, but because this type of tumor cell is not easily killed by radiation therapy, it is usually used only to kill a small amount of leftover tumor after surgery or in combination with other treatments.

Several new radiotherapy techniques have made it possible to deliver radiation therapy more accurately, including three-dimensional conformal radiation therapy (3D-CRT), intensity modulated radiation therapy (IMRT), or conformal proton beam radiation therapy (CPBRT), which allows the radiologist to more accurately target the tumor while minimizing the dose to normal tissue surrounding the tumor. Such techniques allow the radiotherapist to more accurately target the tumor while minimizing the dose to the normal tissue surrounding the tumor. As a result, the success rate of the treatment is increased and side effects are reduced.

Samarium is a radioactive element that is enriched in areas where bone cells grow. It helps relieve pain caused by tumors in the bones and kills blood cells in the bone marrow. It is also used to treat osteosarcoma that recurs on other bones. A stem cell transplant may be needed after treatment with samarium. Before samarium treatment, healthy hematopoietic stem cells (immature blood cells) are removed from the patient's blood or bone marrow and stored frozen. After samarium treatment is completed, the stored stem cells are thawed and infused back into the patient. These reinfused stem cells will restore the body's hematopoietic function.

2.2 Targeted therapy

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific tumor cells without harming normal cells.

There are different types of targeted therapies used to treat osteosarcoma or to study osteosarcoma treatment in clinical trials:

2.2.1 Kinase inhibitors:

It blocks a protein needed for tumor cells to divide. Sorafenib is a kinase inhibitor used to treat recurrent osteosarcoma.

2.2.2 mTOR inhibitors:

It stops the growth of tumor cells and prevents the growth of new blood vessels needed for tumor growth. Everolimus is an mTOR inhibitor used to treat recurrent osteosarcoma.

2.2.3 Monoclonal antibodies:

Antibodies come from specific immune cells made in a laboratory. These antibodies recognize substances on tumor cells, or those normal substances that help tumor cells grow. The antibody attaches to the target substance and kills the tumor cell, stops it from growing or prevents it from spreading. Monoclonal antibodies are given by infusion. They can be used alone or carry other drugs, toxins, or radioactive substances to the tumor cells.Denosumab and Dinutuximab are monoclonal antibodies being studied for the treatment of recurrent osteosarcoma.

2.3 Clinical trials

For some children, participating in a clinical trial may be the best treatment option. Clinical trials are part of the oncology research process to find out if new tumor treatments are safe and effective or better than standard treatments. Many of today's standard treatments for tumors are based on early clinical trials. Children enrolled in a clinical trial may receive the standard treatment or may go straight to the new treatment. Even when a clinical trial does not result in an effective new treatment, participants can contribute to the research process. Children can enter a clinical trial before, during, or after treatment.

It is important to note that different clinical trials have different purposes and target different groups of patients: some trials only accept patients who have not yet had any treatment; some trials are aimed at patients who have received standard treatment and have not improved; and some trials are aimed at testing to stop relapses or to reduce the side effects of treatment.

Participation in a clinical trial may require several tests to observe the effects of treatment, which are used to decide whether to continue, change, or stop treatment. Follow-up testing exams occur after treatment is completed and focus on the patient's status and whether the tumor has returned.

For more information, please refer to the website https://clinicaltrials.gov

3. Determination of treatment programs

The selection of corresponding treatment options for osteosarcoma depends on the different subtypes of osteosarcoma:

(1) The location of the tumor in the body;

(2) Size of the tumor;

(3) Staging and grading of tumors;

(4) Whether the bones are still growing;

(5) Age and overall health of the patient;

(6) The patient's and family's wishes regarding the patient's ability to participate in activities such as sports, or requirements regarding appearance;

(7) Whether the tumor was first diagnosed or recurred after treatment.

3.1 Localized osteosarcoma

Treatment may include the following:

:: Surgical removal of the primary tumor

● Chemotherapy, which may be given before and/or after surgery to remove the primary tumor

● Radiation therapy may be considered for patients who cannot undergo surgery or whose tumors have not been completely removed by surgery.

3.2 Metastatic Osteosarcoma

After metastasis from osteosarcoma occurs, surgery is possible in a few cases, such as single and very rarely multiple. In cases where surgery is possible, the following modalities are considered:

3.2.1 Lung metastases

When osteosarcoma spreads, it usually spreads to the lungs and treatment includes:

● Chemotherapy after surgery to remove the primary tumor or tumor cells that have spread to the lungs.

3.2.2 Bone metastases or bone combined with lung metastases

Osteosarcoma can spread to distant bone and/or lungs, and treatment may include the following options:

● Chemotherapy before surgery, then surgery to remove the primary tumor and tumors that have spread to other parts of the body, and more chemotherapy given after surgery.

● Surgical removal of the primary tumor followed by chemotherapy and surgical removal of tumor cells that have spread to other parts of the body.

3.3 Recurrent osteosarcoma

Treatment of recurrent osteosarcoma includes...

:: Surgery to remove tumors that have spread to other parts of the body

:: Chemotherapy

:: Samarium alone, or in combination with the patient's own stem cell transplant, for tumors that recur only in the bone, as a way to relieve pain and improve quality of life

:: Targeted therapy: try sorafenib or everolimus

:: Some new clinical trials: examining patient tumor samples for certain genetic changes. The type of targeted therapy available depends on the type of genetic change

:: Clinical trials for patients whose tumors cannot be surgically removed: including targeted therapies such as monoclonal antibody therapy.

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.

4. Side effects of treatment

4.1 Possible side effects specific to osteosarcoma

:: Skeletal swelling that extends beyond the bones or bony parts of the body

:: Pain in bones or joints

:: Redness or warmth of bones or joints

:: Stiffness or difficulty moving joints

:: Short stature (shorter than normal height)

:: Curvature of the spine or kyphosis

:: Osteoporosis (loss of bone mass due to reduced activity)

:: Radiation osteoradionecrosis (especially of the hip or knee due to insufficient blood flow)

:: Problems with activities of daily living

:: Can't be as active as usual

:: Chronic pain or infection

:: Problems with the fitting or use of prostheses

:: Fractures

:: Bones may not heal after surgery

● The affected limb is shorter than the other healthy limb.

4.2 Common Side Effects of Oncology Treatments

Oncology treatments can cause side effects because killing tumor cells can also affect healthy tissues or organs, and the body may react as follows after starting treatment:

:: Anemia

:: Loss of appetite

:: Bleeding and bruising (thrombocytopenia)

:: Constipation

:: Delirium

:: Diarrhea

:: Oedema (swelling)

:: Fatigue

:: Male fertility issues

:: Female fertility

:: Hair loss

:: Infection and neutropenia

:: Lymphoedema

:: Changes in memory and concentration

:: Oral and throat problems

:: Nausea and vomiting

:: Neurological problems (peripheral neuropathy)

:: Pain

:: Men's sexual health issues

:: Women's sexual health issues

:: Skin and nail changes

:: Sleep changes

:: Urinary changes

Information about the side effects of cancer treatment can be found in the Children's Palliative and Hospice Care section of 3. Symptom Management The side effects listed above vary from person to person, and may be different even if they receive the exact same treatment. It is important for your child to talk to his or her healthcare provider if he or she experiences any side effects.

Prognosis

The 5-year survival rate for osteosarcoma is approximately 76% for patients under 15 years of age and 66% for patients 15-19 years of age.

1. Factors affecting prognosis

(1) The location of the tumor in the body and whether the tumor forms in more than one bone: it is generally better to occur in the extremities than in the axial skeleton, and the prognosis of extremity tumors is better when they occur distally.

(2) Size of the tumor: smaller tumors have a better prognosis.

(3) Whether the tumor has spread to other parts of the body and where it has spread: patients without metastases have a better prognosis. The prognosis for patients with metastases depends on the location and number of metastases and whether the metastases can be surgically removed.

(4) The type of tumor (based on the morphology of the tumor cells under the microscope).

(5) Age and weight of the patient at the time of diagnosis: patients younger than 18 years of age at the time of onset have a better prognosis than those who develop the disease in adulthood.

(6) Whether the patient has been treated for other tumors.

(7) Whether the tumor caused a fracture. Patients in whom no pathologic fractures occurred had an overall better prognosis.

(8) Whether the patient has certain genetic disorders.

(9) Tumor response to chemotherapy: how many tumor cells are killed by chemotherapy. The higher the percentage of tumor necrosis induced by preoperative adjuvant chemotherapy, the better the prognosis.

(10) How many tumors are surgically removed: tumors that can be completely removed during surgery have a better prognosis.

(11) Whether the tumor recurred within 2 years of diagnosis.

2. Observation of recurrence

When treatment is over, the doctor will want to continue to follow the patient closely. It is important to be on time for each office visit. During each follow-up visit, the doctor will ask the patient about any problems he or she may be experiencing and look for signs of tumors or side effects of treatment through physical exams, lab tests, or x-rays and other imaging. Almost all treatments have side effects. Some may last from a few weeks to a few months, while others may last a lifetime.

If major orthopedic surgery is performed, adherence to rehabilitation and physical therapy is important for patients to regain as much movement and independence as possible.

3. Managing long-term and long-term side effects

3.1 Social, Emotional and Other Problems in Osteosarcoma Patients

Many osteosarcomas occur during adolescence or early adulthood, which is a very sensitive time in a person's life. Osteosarcoma and its treatment can have a profound effect on a person's appearance and perception of their body, as well as how they go about their daily lives, such as school, work, and recreational activities. These effects are often greatest in the first year of treatment, but can also last a long time in some people.

Some children and adolescents have emotional and psychological problems during and after treatment. Depending on age, they may have problems with normal life and academic tasks. These can often be overcome with support and encouragement.

The oncology care team recommends that school-age children and adolescents go to school as often as possible, which can help them maintain their daily lives and keep their friends informed. Friends can be an important source of support, but children and parents should be aware that some people have misconceptions and fears about tumors.

Some hospitals may refer new patients to programs for patients who have completed treatment. This can give the new patient some idea of what to expect during and after treatment. Also seeing another osteosarcoma patient doing well often increases confidence in treatment. Encourage participation in athletics and activities that make good use of the extremities. Many patients with amputations or prostheses are eventually able to participate in athletics.

Parents and other family members may also be affected emotionally or in other ways. Common family problems include financial stress, living near a hospital, the possibility of losing a job, and the need for home schooling.

During treatment, patients and families tend to focus on all aspects of getting through and beating the tumor, but once treatment is over, a plethora of emotional issues can add up, many of which will continue for a long time and may include:

:: Facing physical changes after treatment

:: Fear that the tumor will return or that new health problems will develop

:: Resentment for having a tumor and having to face treatment

:: Fear of being treated differently or discriminated against (by friends, classmates, coworkers, employers, etc.)

:: Concerns about dating, marriage and subsequent family formation

No one actively chooses to have osteosarcoma, but for many children and adolescents, the experience may turn out to be a positive one, helping to build a strong sense of self-worth. Others may experience a more difficult time recovering and adjusting to life afterward so they can move on. It is normal to experience anxiety, depression, or other emotional reactions after treatment, but excessive anxiety, depression, or anger can affect many aspects of a young person's growth, which may involve relationships, schooling, career, and other aspects of life.

3.2 Distant and long-term effects of osteosarcoma treatment

Many young people who develop osteosarcoma are now able to survive. But treatment can affect them later in life, so keeping a close eye on health issues as they grow up has been important in recent years.

Just as the treatment of young oncology patients is a very specific process, so is the process of their rehabilitation and care. The earlier the problem is detected, the more effective the treatment is likely to be.

The long-term effects of treatment depend on many factors, such as the type of tumor, the type of treatment received, the intensity and dosage of the treatment, and the age at the time of treatment. The late effects of surgery may be as small as a scar or as large as the loss of an amputated limb, thus requiring physical rehabilitation and emotional adjustment. Other long-term effects may include:

(1) Problems with the heart and lungs: caused by specific chemotherapeutic drugs or radiotherapy that target the heart and lungs;

(2) Loss of hearing: caused by specific chemotherapy drugs;

(3) Slow growth: skeletal or overall;

(4) Learning problems of young children;

(5) Changes in Sexual Development and Fertility: Problems with fertility after treatment for osteosarcoma are uncommon, but may still occur. Older girls or adult women may experience changes in their menstrual cycles during chemotherapy, but normal cycles return after treatment. Boys and men may temporarily lose the ability to produce sperm, which usually returns, but sperm counts may still be low. Radiation therapy to the pelvis may damage the reproductive organs and affect fertility. The risk of infertility can be discussed with you or your child's doctor prior to treatment and to inquire if there are ways to preserve fertility, such as a sperm bank;

(6) Development of a second tumor: In a few cases, some chemotherapy treatments have resulted in the development of a second tumor (e.g., leukemia) many years after cure of osteosarcoma. Radiotherapy may also increase the risk of developing new tumors at the site of action. But overall the importance of treating osteosarcoma outweighs this small risk.

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

Ask a question at the clinic

It is important to have a frank and open discussion with your oncology treatment team. Please note the following questions:

Question 1: What type of osteosarcoma do I (or my child) have? Will this affect treatment?

Question 2: Has it spread to other parts of the bone?

Question 3: What is the staging of osteosarcoma and what does this mean?

Question 4: Are there any other tests needed before we decide on a treatment plan?

Question 5: How much experience do you have with this tumor?

Question 6: Will we need to see another doctor?

Question 7: What treatment options do we have?

Question 8: What treatment program do you recommend and why?

Question 9: How quickly do we need to start implementing treatment?

Question 10: How long does the treatment take? What is the approximate condition of the treatment and where is it implemented?

Question 11: How will treatment affect our daily lives?

Question 12: What do I (we) need to do to prepare for treatment?

Question 13: What are the possible risks and side effects of your proposed treatment plan?

Question 14: Which side effects will appear soon after treatment and which will appear later?

Question 15: Will the treatment affect my child's growth and development?

Question 16: Will it affect my (child's future) fertility?

Question 17: What is the probability of tumor recurrence based on these treatment options? What do we need to do if it recurs?

Question 18: What is the rehabilitation program after treatment?

Question 19: Are there volunteer groups or other families who have gone through similar situations that I can consult and ask for help?

Follow-up & Review

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Routine

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Cutting-edge Therapeutic & Clinical Research

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References

References

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2. Goldsby R, Burke C, Nagarajan R, et al. Second solid malignancies among children, adolescents, and young adults diagnosed with malignant bone tumors after 1976: follow-up of a Children's Study. after 1976: follow-up of a Children's Oncology Group cohort. Cancer 113 (9): 2597-604, 2008.
3. Bhatia S, Krailo MD, Chen Z, et al. Therapy-related myelodysplasia and acute myeloid leukemia after Ewing sarcoma and primitive neuroectodermal tumor of bone: a report from the bone tumor of Ewing sarcoma. tumor of bone: a report from the Children's Oncology Group. Blood 109 (1): 46-51, 2007
4. Kushner BH, Heller G, Cheung NK, et al. High risk of leukemia after short-term dose-intensive chemotherapy in young patients with solid tumors. J Clin J Clin Oncol 16 (9): 3016-20, 1998.
5. Ginsberg JP, Goodman P, Leisenring W, et al. Long-term survivors of childhood Ewing sarcoma: report from the childhood cancer survivor study. J Natl Cancer Inst 102 (16): 16. Cancer Inst 102 (16): 1272-83, 2010.
6. Kuttesch JF Jr, Wexler LH, Marcus RB, et al. Second malignancies after Ewing's sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol 14 (10): 2818-25, 1996.
7. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 348 (8): 694-701, 2003.
8. Leavey PJ, Mascarenhas L, Marina N, et al. Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: a report from the Children's Hospital for Children with EWS. therapy: a report from the Children's Oncology Group. Pediatr Blood Cancer 51 (3): 334-8, 2008.
9. Stahl M, Ranft A, Paulussen M, et al. Risk of recurrence and survival after relapse in patients with Ewing sarcoma. Pediatr Blood Cancer 57 (4): 549-53, 2011. 2011.
10. Barker LM, Pendergrass TW, Sanders JE, et al. Survival after recurrence of Ewing's sarcoma family of tumors. J Clin Oncol 23 (19): 4354-62, 2005.
11. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 348 (8): 694-701, 2003.
12. Juergens C, Weston C, Lewis I, et al. Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E.W.I.N.G. 99 clinical trial. Pediatr Blood Cancer 47 (1): 22-9, 2006.