21st Century Pediatric Cancer Sourcebook: Childhood Soft Tissue Sarcoma (including Schwannoma, Chondrosarcoma, PEComas, Mesenchymoma, Angiosarcoma, Dermatofibrosarcoma, Others)
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
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This is a privately authored news service and educational publication of Progressive Management. Our publications synthesize official government information with original material - they are not produced by the federal government. They are designed to provide a convenient user-friendly reference work to uniformly present authoritative knowledge that can be rapidly read, reviewed or searched. Vast archives of important data that might otherwise remain inaccessible are available for instant review no matter where you are. This e-book format makes a great reference work and educational tool. There is no other reference book that is as convenient, comprehensive, thoroughly researched, and portable - everything you need to know, from renowned experts you trust. For over a quarter of a century, our news, educational, technical, scientific, and medical publications have made unique and valuable references accessible to all people. Our e-books put knowledge at your fingertips, and an expert in your pocket!
PART ONE
Chapter 1A: Childhood Soft Tissue Sarcoma
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
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Chapter 1A: Childhood Soft Tissue Sarcoma
Last Modified: 12/14/2010
Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen). There are many different types of soft tissue sarcomas. The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles that surround bone. Rhabdomyosarcoma is not discussed in this summary. This summary is about the other types of soft tissue sarcoma:
Fibrous (connective) tissue tumors
Desmoid tumor.
Dermatofibrosarcoma.
Fibrosarcoma.
Fibrohistiocytic tumors
Malignant fibrous histiocytoma (also called MFH, undifferentiated pleomorphic sarcoma, or spindle cell sarcoma).
Plexiform histiocytic tumor.
Fat tissue tumors
Liposarcoma.
Smooth muscle tumors
Leiomyosarcoma.
Blood and lymph vessel tumors
Angiosarcoma.
Lymphangiosarcoma.
Hemangiopericytoma.
Hemangioendothelioma.
Peripheral nervous system tumors
Malignant schwannoma (malignant peripheral nerve sheath tumor).
Bone and cartilage tumors
Extraosseous osteosarcoma.
Extraosseous myxoid chondrosarcoma.
Extraosseus mesenchymal chondrosarcoma.
PEComas
Angiomyolipoma.
Lymphangioleiomyomatosis.
Clear cell "sugar" tumor.
Tumors with more than one type of tissue
Malignant mesenchymoma.
Malignant Triton tumor.
Malignant ectomesenchymoma.
Tumors of unknown origin (the place where the tumor first formed is not known)
Alveolar soft part sarcoma.
Epithelioid sarcoma.
Clear cell sarcoma (malignant melanoma of soft parts).
Synovial sarcoma.
Desmoplastic small round cell tumor.
Undifferentiated soft tissue sarcoma.
Besides rhabdomyosarcomas, the most common soft tissue sarcomas in children are in joint tissue, connective tissue, and nerve tissue.
Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better outcome than soft tissue sarcoma in adults.
Having certain diseases and inherited disorders can increase the risk of developing childhood soft tissue sarcoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:
Li-Fraumeni syndrome.
Neurofibromatosis type 1 (NF1).
Familial adenomatous polyposis (FAP).
Other risk factors include the following:
Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection.
Having retinoblastoma in both eyes.
Past treatment with radiation therapy.
The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body.
A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other symptoms at first. As the sarcoma grows larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may occur, including pain or weakness.
Other conditions may cause the same symptoms that soft tissue sarcomas do. A doctor should be consulted if any of these problems occur.
Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood soft tissue sarcoma.
The following tests and procedures may be used:
Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
X-rays: An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. A series of x-rays may be done to check the lump or painful area.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Magnetic resonance imaging (MRI) of the abdomen. The patient lies on a table that slides into the MRI machine, which takes pictures of the inside of the body. The pad on the patient’s abdomen helps make the pictures clearer.
If these tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies may be used:
Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle. A pathologist views the tissue or fluid under a microscope to look for cancer cells.
Core biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI. A pathologist views the tissue under a microscope to look for cancer cells.
Incisional biopsy: The removal of part of a lump or a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells.
Excisional biopsy: The removal of an entire lump or area of tissue that doesn’t look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin.
In order to plan the best treatment, a large sample of tissue may be removed during the biopsy to find out the type of soft tissue sarcoma and do laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have a tumor. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, patients should ask to have the tissue sample checked by a pathologist who has experience in diagnosing soft tissue sarcoma.
One or more of the following laboratory tests may be done to study the tissue samples:
Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
Immunohistochemistry study: A laboratory test in which dyes or enzymes are added to a blood or bone marrow sample to test for certain antigens (proteins that stimulate the body's immune response).
Immunocytochemistry study: A laboratory test that uses different substances to stain (color) cells in a sample of tissue. This is used to tell the difference between the different types of soft tissue sarcoma.
Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
The type of soft tissue sarcoma.
The stage of the cancer (the amount of tumor remaining after surgery to remove it or whether the tumor has spread to other places in the body).
The location, grade, and size of the tumor and how deep under the skin the tumor is.
Whether the patient also has a condition called neurofibromatosis type 1 (NF1).
The age of the patient.
Whether the cancer has just been diagnosed or has recurred (come back).
Stages of Childhood Soft Tissue Sarcoma
Key Points for This Section
After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
There are three ways that cancer spreads in the body.
One method used to stage childhood soft tissue sarcoma is based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread:
Nonmetastatic childhood soft tissue sarcoma
Metastatic childhood soft tissue sarcoma
Another method used to stage childhood soft tissue sarcoma is based on the size of the tumor and whether cancer has spread to lymph nodes or other parts of the body.
Stage I
Stage II
Stage III
Stage IV
After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. Two methods that are commonly used for staging are based on the amount of tumor remaining after surgery to remove the tumor and/or the grade and size of the tumor and whether it has spread to the lymph nodes or other parts of the body. It is important to know the stage in order to plan treatment.
The following tests and procedures may be used in the staging process:
Sentinel lymph node biopsy may be used to stage childhood soft tissue sarcoma. This is the removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
The results of the sentinel lymph node biopsy and CT scan are viewed together with the results of the diagnostic tests and initial surgery to determine the stage of the soft tissue sarcoma.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
Through tissue. Cancer invades the surrounding normal tissue.
Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
One method used to stage childhood soft tissue sarcoma is based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread:
Nonmetastatic childhood soft tissue sarcoma
In nonmetastatic childhood soft tissue sarcoma, the cancer has been partly or completely removed by surgery and has not spread to other parts of the body.
Group I: The tumor has been completely removed by surgery.
Group II: After surgery to remove the tumor, there are remaining cancer cells that can be seen only with a microscope.
Group III: After surgery, there is tumor remaining that can be seen with the eye.
Metastatic childhood soft tissue sarcoma
Group IV: The cancer has spread from where it started to other parts of the body (metastasis).
Another method used to stage childhood soft tissue sarcoma is based on the size of the tumor and whether cancer has spread to lymph nodes or other parts of the body.
This staging system is based on the following:
The size of the tumor.
Whether the tumor has spread to the lymph nodes.
Whether the tumor has spread to other parts of the body.
Stage I
Stage I is divided into stages IA and IB:
In stage IA, the tumor is low-grade (likely to grow and spread slowly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
In stage IB, the tumor is low-grade (likely to grow and spread slowly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
Stage II
Stage II is divided into stage IIA and stage IIB:
In stage IIA, the tumor is mid-grade (somewhat likely to grow and spread quickly) or high-grade (likely to grow and spread quickly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
In stage IIB, the tumor is mid-grade (somewhat likely to grow and spread quickly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
Stage III
In stage III, the tumor is either:
high-grade (likely to grow and spread quickly), larger than 5 centimeters, and either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue); or
any grade, any size, and has spread to nearby lymph nodes.
Stage IV
In stage IV, the tumor is any grade, any size, and may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body such as the lungs.
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body. Progressive childhood soft tissue sarcoma is cancer that did not respond to treatment.
Treatment Option Overview
Key Points for This Section
There are different types of treatment for patients with childhood soft tissue sarcoma.
Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Some cancer treatments cause side effects months or years after treatment has ended.
Seven types of standard treatment are used:
Surgery
Radiation therapy
Chemotherapy
Hormone therapy
Watchful waiting
Nonsteroidal anti-inflammatory drugs
Liver transplant
New types of treatment are being tested in clinical trials.
Targeted therapy
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for patients with childhood soft tissue sarcoma.
Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included:
Radiation oncologist. * Pediatric hematologist. * Pediatric nurse specialist. * Rehabilitation specialist. * Psychologist. * Social worker.
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Second cancers (new types of cancer).
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child.
Seven types of standard treatment are used:
Surgery
Surgery to completely remove the soft tissue sarcoma is done whenever possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. The following types of surgery may be used:
Wide local excision: Removal of the tumor along with some normal tissue around it.
Amputation: Surgery to remove part or all of a limb or appendage, such as the arm or hand.
Limb-sparing surgery: Removal of the tumor in an arm or leg without amputation, so the use and appearance of the limb is saved. Radiation therapy or chemotherapy may be given first to shrink the tumor. The tumor is then removed in a wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone.
Lymphadenectomy: Removal of the lymph nodes that contain cancer.
A second surgery may be needed to: remove any remaining cancer cells; check the area around where the tumor was removed for cancer cells and then remove them.
Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy.
External radiation therapy uses a machine outside the body to send radiation toward the cancer. Stereotactic radiation therapy aims radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Hormone therapy
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen) may be used to treat childhood soft tissue sarcoma.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting may be done when complete removal of the tumor is not possible, no other treatments are available, and the tumor does not place any vital organs in danger.
Nonsteroidal anti-inflammatory drugs
Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of soft tissue sarcomas, an NSAID called sulindac may be used to help block the growth of cancer cells.
Liver transplant
The liver is removed and replaced with a healthy one from a donor.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Imatinib (Gleevec) is a type of targeted therapy called a tyrosine kinase inhibitor. It finds and blocks an abnormal protein on cancer cells that causes them to divide and grow.
Other targeted therapies being studied in clinical trials include angiogenesis inhibitors. In cancer treatment, angiogenesis inhibitors prevent the growth of new blood vessels needed for tumors to grow.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Soft Tissue Sarcoma
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Nonmetastatic Childhood Soft Tissue Sarcoma
For treatment of hemangiopericytoma (in infants and young children) and infantile fibrosarcoma
If an infant or young child has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include chemotherapy to reduce the size of the tumor, followed by surgery.
If an older child or adolescent has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include the following:
Chemotherapy followed by radiation therapy, surgery, or both.
Radiation therapy or chemotherapy after surgery.
A clinical trial of internal radiation therapy.
A clinical trial of radiation therapy given during surgery.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
For treatment of desmoid tumor
Treatment of desmoid tumor will be surgery whenever possible. Other treatments may include watchful waiting, chemotherapy, or surgery followed by internal radiation therapy.
If surgery to completely remove the tumor is not possible, treatment to shrink the tumor before surgery may include the following:
External radiation therapy
A nonsteroidal anti-inflammatory drug (NSAID).
Antiestrogen therapy.
Chemotherapy.
For treatment of alveolar soft part sarcoma
Treatment of alveolar soft part sarcoma will be surgery whenever possible. Other treatments may include the following:
Radiation therapy.
A clinical trial of surgery followed by watchful waiting, chemotherapy and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
A clinical trial of targeted drug therapy.
For treatment of clear cell sarcoma of soft parts
Treatment of clear cell sarcoma of soft parts will be surgery whenever possible. Other treatments may include the following:
Radiation therapy after surgery.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
For treatment of desmoplastic small round cell tumor
Treatment of desmoplastic small round cell tumor may include surgery, chemotherapy (which may be given before surgery), and radiation therapy.
For treatment of extraosseous osteosarcoma
For treatment of hemangioendothelioma
Treatment of hemangioendothelioma in children younger than one year may include the following:
Watchful waiting as some tumors will disappear without treatment.
Treatment for blood clotting disorders.
Surgery to remove the tumor if there are symptoms.
Liver transplant (if the tumor is in the liver).
Treatment of hemangioendothelioma in children aged one year and older may include the following:
Surgery to remove the tumor.
Treatment for blood clotting disorders.
Liver transplant (if the tumor is in the liver).
For treatment of malignant peripheral nerve sheath tumor
Treatment of malignant peripheral nerve sheath tumor will be surgery whenever possible. Other treatments may include the following:
Radiation therapy after surgery.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
For treatment of plexiform histiocytic tumor
Treatment of plexiform histiocytic tumor is surgery to completely remove the tumor.
For treatment of synovial sarcoma
Treatment of synovial sarcoma may include the following:
Surgery with or without chemotherapy.
Chemotherapy.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
For treatment of undifferentiated soft tissue sarcoma
Treatment of undifferentiated soft tissue sarcoma may be within a clinical trial for patients with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). This study includes surgery and/or radiation therapy with or without chemotherapy.
For treatment of angiosarcoma and lymphangiosarcoma
Treatment of angiosarcoma and lymphangiosarcoma may include the following:
Surgery to completely remove the tumor.
Radiation therapy or chemotherapy after surgery.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
For treatment of aggressive fibromatosis, dermatofibrosarcoma, and angiomatoid malignant fibrous histiocytoma
Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:
Targeted drug therapy with imatinib (Gleevec).
Radiation therapy after surgery.
For treatment of epithelioid sarcoma, leiomyosarcoma, liposarcoma, and mesenchymal chondrosarcoma
Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:
Chemotherapy followed by radiation therapy, surgery, or both.
Radiation therapy or chemotherapy after surgery.
A clinical trial of internal radiation therapy.
A clinical trial of radiation therapy given during surgery.
A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.
Metastatic Childhood Soft Tissue Sarcoma
Treatment of metastatic childhood soft tissue sarcoma may include the following:
Combination chemotherapy, radiation therapy, and surgery to remove cancer that has spread to the lungs.
Stereotactic radiation therapy or repeat surgery if several tumors appear or come back in the lungs.
A clinical trial of combination chemotherapy and granulocyte colony-stimulating factor (substance that helps the body increase the number of white blood cells).
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following:
Watchful waiting for some desmoid tumors.
Surgery followed by radiation therapy, if not already given.
Surgery to remove the arm or leg with cancer, if radiation therapy was already given.
Surgery to remove cancer that has spread to the lungs.
A clinical trial of new combinations of chemotherapy.
A clinical trial of targeted drug therapy with imatinib (Gleevec).
Glossary Terms
abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
abnormal
Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).
AIDS
A disease caused by the human immunodeficiency virus (HIV). People with AIDS are at an increased risk for developing certain cancers and for infections that usually occur only in individuals with a weak immune system. Also called acquired immunodeficiency syndrome.
alveolar soft part sarcoma (al-VEE-oh-lur … sar-KOH-muh)
A soft tissue tumor that is most common in older children and teenagers. It begins in the soft supporting tissue that connects and surrounds the organs and other tissues. Alveolar soft part sarcoma usually occurs in the legs, but can also occur in the arms, hands, head, or neck. It can cause the growth of new blood vessels that help the tumor grow and spread. Also called ASPS.
angiomyolipoma (AN-jee-oh-MY-oh-lih-POH-muh)
A benign (noncancer) tumor of fat and muscle tissue that usually is found in the kidney. Angiomyolipomas rarely cause symptoms, but may bleed or grow large enough to be painful or cause kidney failure. They are common in patients with tuberous sclerosis (a genetic disorder in which benign tumors grow in the kidneys, brain, eyes, heart, lungs, and skin, causing seizures, mental problems, and skin lesions).
angiosarcoma (AN-jee-oh-sar-KOH-muh)
A type of cancer that begins in the cells that line blood vessels or lymph vessels. Cancer that begins in blood vessels is called hemangiosarcoma. Cancer that begins in lymph vessels is called lymphangiosarcoma.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.