Key Terms: Alveolar rhabdomyosarcoma, Brachytherapy, Cytogenetics, Dermatofibrosarcoma protuberans, Embryonal rhabdomyosarcoma, Epithelioid hemangioendothelioma, Extraosseous Ewing's tumor, Hemangioendothelioma, Fine needle aspiration, Lymph nodes, Mesoderm, Malignant fibrous histiocytoma, Primitive neuroectodermal tumor, Non-RMS, Retroperitoneum, Rhabdomyosarcoma.

Definition

Soft tissue sarcomas are cancerous (malignant) tumors that develop in mesodermal tissues that surround, support, and connect the structures and organs of the body.

Description

Soft tissues include muscles, fibrous (connective) tissues, fat, blood and lymph vessels, synovial tissues surrounding the joints, peripheral nerve tissues, and deep skin tissues. As soft tissue sarcomas grow, they may invade surrounding tissue, or spread (metastasize) to distant sites in the body. Together they account for less than 1% of all newly diagnosed cancers.

About one-half of all soft tissue sarcomas develop in the arms, legs, hands, or feet. About 40% occur in the trunk, internal organs, or the retroperitoneum—the back of the abdominal cavity. The remaining 10% occur in the head and neck.

Muscle Tissue Sarcomas

Rhabdomyosarcoma (RMS)—a skeletal muscle tumor—is the most common soft tissue sarcoma in children. Embryonal rhabdomyosarcoma (ERMS) is more common than alveolar rhabdomyosarcoma (ARMS). ERMS commonly develops in the head, neck, or reproductive or urinary tract organs. ARMS develops in the large muscles of the arms, legs, or trunk. All other soft tissue sarcomas in children are classified as non-rhabdomyosarcoma or non-RMS.

Leiomyosarcomas are smooth muscle tumors that occur most often in the retroperitoneum or internal organs but also may occur in the deep soft tissues of the arms or legs.

Fibrous Tissue Sarcomas

Soft tissue sarcomas often occur in connective tissue:

• Fibrosarcoma is a cancer of the tendons and ligaments.
• Malignant fibrous histiocytoma (MFH) is the most common soft tissue tumor of the limbs, although it also occurs in the retroperitoneum. MFH accounts for 40% of all soft tissue sarcomas.
• Dermatofibrosarcoma protuberans (DFSP) is a lowgrade cancer of fibrous tissue under the skin, usually in the limbs or trunk.
• Desmoid tumors may be low-grade fibrosarcomas or a unique type of fibrous tissue tumor.

Fat Tissue Sarcomas

Liposarcomas develop in fat tissue anywhere in the body but occur most often in the thigh or the retroperitoneum. They range from very slow to very fast growing and account for 25% of all soft tissue sarcomas.

Blood and Lymph Vessel Sarcomas

• Angiosarcoma is called hemangiosarcoma if it occurs in a blood vessel and lymphangiosarcoma if it occurs in a lymph vessel.
• Hemangioendothelioma—usually called epithelioid hemangioendothelioma (EHE) in adults—is a lowgrade cancer in the blood vessels of soft tissue or internal organs such as the lungs or liver.
• Hemangiopericytoma is a sarcoma of the perivascular tissue around blood vessels that help control blood flow. It most often develops in the legs, pelvis, or retroperitoneum.
• Kaposi's sarcoma is a tumor formed by cells similar to those that line blood and lymph vessels.

Synovial Sarcoma

Synovial sarcomas are tumors of the synovium—the tough tissue that surrounds the joints. They occur most often in leg and arm joints, especially the knee. They are the most common non-RMS in children. Approximately 30% of synovial sarcomas occur in those under age 20.

Peripheral Nerve Sarcomas

Malignant peripheral nerve sheath tumors—also called malignant schwannomas, neurofibrosarcomas, or neurogenic sarcomas—are tumors in cells surrounding the peripheral nerves that run throughout the body. Ewing's tumors are a group of related cancers that share characteristics with nerve tissue in a developing embryo. Ewing's tumors that occur in soft tissue are extraosseous (outside of the bone) Ewing's (EOE) and primitive neuroectodermal tumors (PNET).

Other Soft Tissue Sarcomas

Some soft tissue sarcomas are of uncertain origin:

• Mesenchymonia is a combination of tissue types that resemble fibrosarcoma and others.
• Alveolar soft-part sarcoma most commonly develops in the legs.
• Epithelioid sarcoma usually develops under the skin of the hands, forearms, lower legs, or feet.
• Clear cell sarcoma—also called malignant melanoma of the soft parts (MMSP), clear cell sarcoma of tendons, aponeuroses—is a rare sarcoma of the tendons and related tissues. It has some characteristics of malignant melanoma or skin cancer.
• Desmoplastic small cell tumors usually occur in the abdomen, pelvis, or tissues around the testes, primarily in males.

Demographics

It is estimated that 9,420 new cases of soft tissue sarcomas will be diagnosed in the United States during 2005—5,530 in males and 3,890 in females. Every year in the United States 850–900 children under age 20 are diagnosed with soft tissue sarcoma, accounting for 7.4% of cancers in that age group. During 2005 an estimated 1,910 American males and 1,580 females will die of soft tissue sarcoma.

Childhood soft tissue sarcomas occur most frequently during infancy or after age 10. Male children have a slightly higher incidence than females and black children have a slightly higher incidence than white children, particularly among 15–19-year-olds.

More than 85% of RMS occur in infants, children, and teenagers. Almost 60% of soft tissue sarcomas in children up to age four are RMS. The prevalence of RMS declines steadily with increasing age, accounting for only 23% of soft tissue sarcomas in 16–19-year-olds. ERMS accounts for 75% of RMS in children aged 1–14. ARMS can affect children in all age groups but is more prevalent among older children.

Other soft tissue sarcomas also affect different age groups:

• Adolescents are more likely to develop leiomyosarcoma in the trunk, whereas in adults it is more common in the uterus or digestive tract.
• Infantile fibrosarcoma affects children up to age four.
• Adolescents are more likely to develop fibrosarcoma in the arms or legs and MFH in the legs.
• Adults are more likely to develop fibrosarcoma in the arms, legs, or trunk, or DFSP in the trunk; MFH is most common in older adults.
• Liposcarcoma can occur in the arms and legs of older teenagers and in the arms, legs, or trunk of adults; however it is most common in people aged 60–65.
• Adults are more likely to develop hemangiosarcoma in the arms, legs, or trunk, lymphangiosarcoma in the arms, or Kaposi's sarcoma in the legs or trunk.
• Hemangiopericytoma is more common in adults, although infantile hemangiopericytoma occurs in children up to age four.
• Synovial sarcomas usually occur in young adults.
• Teenagers and adults can develop malignant peripheral nerve sheath tumors in the arms, legs, or trunk.
• Soft tissue Ewing's tumors are relatively common in children and very rare in adults.
• Mesenchymonia is a rare sarcoma of children.
• Aveolar soft-part sarcoma is rare, usually affecting young adults.
• Alveolar soft-part sarcoma of the muscular nerves of the arms or legs can affect children in all age groups but is more prevalent in older children.
• Epithelioid sarcoma usually affects adolescents and young adults.
• Desmoplastic small cell tumors are rare and affect primarily male teenagers and young adults.

Causes & Symptoms

Causes

Although most soft tissue sarcomas have no known cause, those in children generally are associated with chromosomal changes. Other soft tissue sarcomas are caused by changes in the DNA carried on the chromosomes. Some of these changes or mutations are inherited but most are acquired during a person's lifetime, possibly from exposure to radiation or cancer-causing chemicals.

In addition:

• Leiomyosarcoma and some other soft tissue sarcomas have been linked to the Epstein-Barr virus in people with AIDS.
• Some hormones, particularly estrogen, cause desmoid tumors to grow.
• Angiosarcomas sometimes develop in an area that has been exposed to radiation.
• Kaposi's sarcoma appears to be related to infection by human herpesvirus-8.

Symptoms

During their early stages most soft tissue sarcomas do not cause symptoms. However as they grow larger the tumors begin to press against normal tissue causing soreness or pain. Synovial sarcoma causes tenderness, pain, or swelling in a joint.

Symptoms of soft tissue sarcoma can include:

• a new or growing lump anywhere in the body
• a usually painless swelling or lump in an arm or leg that grows over weeks or months.

About one-third of abdominal sarcomas cause increasing pain. Although symptoms may be nonspecific, abdominal tumors can grow large enough to be felt or to cause blockage or bleeding in the stomach or bowels. This leads to blood in vomit or stools and may cause stools to be very black and tarry.

RMS often develops in easily detectable regions such as a lump just under the skin or around the testes:

• RMS in an eye muscle can cause bulging eye
• RMS in the nasal cavity may cause nosebleeds
• RMS often occurs in the bladder or genitourinary tract causing difficult urination or blood in the urine
• RMS in the abdomen or pelvis may cause vomiting, abdominal pain, or constipation

Diagnosis

Only about 50% of soft tissue sarcomas are diagnosed at early stages before the cancer has spread. Soft tissue sarcomas in children may be particularly difficult to diagnose.

Diagnosis may include:

• a medical history to uncover any risk factors
• a physical examination
• ultrasound imaging for visualizing internal organs and masses
• a computed tomography (CT) scan—sometimes in conjunction with a radiocontrast dye—to help determine whether a sarcoma has spread to the liver or other organs
• magnetic resonance imaging (MRI)—sometimes with radiocontrast dyes—to obtain detailed images of organs or masses
• chest x rays to determine whether a sarcoma has spread to the lungs
• positron emission tomography (PET) to scan the entire body for metastasized cancer

Biopsies

Unlike most cancers, the size of a soft tissue sarcoma may be less important than the appearance of the cancer cell. Cells that appear similar to normal cells of the same tissue are called well-differentiated or moderately differentiated. Sarcoma cells that appear very different from normal tissue are referred to as poorly differentiated or undifferentiated. For example, ERMS cells resemble developing skeletal muscle cells in a 6–8-week-old fetus and ARMS cells resemble the normal muscle cells of a 10-week-old fetus. Therefore microscopic examination of sarcoma cells obtained by a biopsy—the removal of sarcoma tissue—is very important for determining the clinical stage, the probable growth rate of the cancer, the likelihood of metastasis, and the prognosis.

A fine needle aspiration (FNA) biopsy uses a very thin needle and syringe to remove small fragments of a superficial (near the surface), easily accessed sarcoma. The needle may be guided by feeling a mass near the surface or using a CT scanner. Although much less invasive than other types of biopsies, FNA may not provide enough tissue to identify a sarcoma, determine its type, and grade it. However it is useful for determining whether a suspected sarcoma is a benign tumor, another type of cancer, an infection, or some other disease. FNA also is used to determine whether tumors in other organs are metastases of the sarcoma.

If FNA indicates a sarcoma, another type of biopsy is used to confirm the diagnosis:

• A core needle biopsy removes a cylindrical piece of tissue of about one-sixteenth in. (0.15 cm) diameter and 0.5 in. (1.3 cm) long. A CT scanner may be used to guide the needle into tumors located in internal organs. Although a core biopsy avoids an incision and may not require general anesthesia, the small sample size may cause a cancer to be missed or misdiagnosed.
• If the sarcoma is small, near the surface, and away from vital tissues, an excisional biopsy may be used to remove the entire mass and surrounding normal tissue. This combines a diagnostic biopsy with surgical treatment.
• An incisional biopsy removes a small portion of a large sarcoma.
• An open surgical biopsy under general anesthesia is used to diagnose RMS in children. In addition to the tumor sample, nearby lymph nodes may be removed for testing.

Testing

In addition to histological examination under a microscope, biopsy samples may require special testing to identify a sarcoma and its type and grade:

• An immunohistochemical test treats the sample with antibodies that recognize cell proteins that are typical of some types of sarcomas. When an antibody binds such a cell protein, a color change is detected microscopically.
• For cytogenetic techniques biopsied cells are grown in the laboratory for about a week and examined microscopically to determine whether chromosomal changes have occurred.
• Fluorescent in situ hybridization (FISH) may be used to detect chromosome abnormalities without first growing the cells.

Treatment Team

Soft tissue sarcomas are treated by a multidisciplinary team of cancer specialists including:

• pathologists
• hematologists
• oncologists
• surgeons
• radiation oncologists

Children and adolescents with soft tissue sarcomas are treated at medical centers specializing in childhood cancers with treatment teams that include:

• a primary care physician
• pediatric hematologists/oncologists
• pediatric surgeons
• radiation oncologists
• pediatric oncology nurses
• nurse practitioners
• rehabilitation and physical therapists
• psychologists
• child-life specialists
• nutritionists
• social workers
• educators

Clinical Staging, Treatments, and Prognosis

Staging Systems

Tnm

Soft tissue sarcomas often are staged according to the TNM system of the American Joint Committee on Cancer, in which T is the primary tumor size and location:

• TX—cannot be assessed
• T0—no evidence of a primary tumor
• T1—the sarcoma is 2 in. (5 cm) or less
• T2—the sarcoma is more than 2 in. (5 cm)
• a—the tumor is superficial
• b—the tumor is deep in a limb or the abdomen.

N represents lymph node involvement in the region of the sarcoma:

• NX—cannot be assessed
• NO—lymph nodes free of sarcoma cells
• N1—regional lymph nodes have sarcoma cells.

Although RMS and synovial and epithelioid sarcomas commonly spread to lymph nodes, overall lymph node involvement occurs with less than 3% of adult soft tissue sarcomas.

M represents metastasis to distant organs:

• MX—cannot be assessed
• MO—sarcoma has not spread
• M1—distant metastases

Grading

In addition to a standard staging system, soft tissue sarcomas are graded according to the microscopic appearance of the cells, where G is the histological grade:

• GX—cannot be assessed
• G1 or low grade—cells appear normal, well-differentiated, slow-growing; these rarely metastasize
• G2 or intermediate—cells are moderately differentiated and fast growing
• G3 or high grade—cells are poorly differentiated and faster growing
• G4—cells are abnormal, poorly or undifferentiated, and very fast growing.

Clinical Staging

Stage I sarcomas are low-grade cancers:

• Stage IA—G1–2, T1a or b, N0, M0
• Stage IB—G1–2, T2a, N0, M0
• Stage II, III, and IV sarcomas are high-grade cancers:
• Stage IIA—G1–2, T2b, N0, M0
• Stage IIB—G3–4, T1a–b, N0, M0
• Stage IIC—G3—4, T2a, N0, M0
• Stage III—G3–4, T2b, N0, M0
• Stage IVA—any G, any T, N1, M0
• Stage IVB—any G, any T, any N, M1.

Treatments

In addition to the stage, treatment depends on other factors including the location of the sarcoma. Treatment of children with non-RMS usually follows the standard treatment for adults. However potential long-term effects of treatment are a greater concern in children, who are much more susceptible to radiation and are expected to live much longer than adults.

Surgery

Most stage I, II, and III soft tissue sarcomas are surgically removed, with the goal of completely removing (resectioning) the tumor, as well as at least 0.8–1.2 in. (2–3 cm) of surrounding tissue. Many soft tissue sarcomas in infants and young children can be treated successfully by surgery alone. Only about 5% of arm or leg sarcomas require amputation of the limb. Most patients have limb-sparing surgery followed by radiation therapy, although these procedures are more difficult in children than in adults. Amputation may be necessary when invading sarcoma cells surround essential nerves, arteries, or muscles, or when limb-sparing surgery would result in a dysfunctional limb or chronic pain. Amputation is not recommended if the sarcoma has metastasized to the lungs or other organs. Abdominal sarcomas are difficult to remove because they can be quite large and adjacent to vital organs.

Stage IVA sarcomas and nearby lymph nodes are surgically removed. Sometimes the removal of stage IVB sarcomas and all of their metastases is attempted. Surgery may be preceded by high-dose radiation and/or chemotherapy to shrink the tumor or for high-grade sarcomas that are at risk of metastasizing. If the only metastasis is in the lungs, sometimes the lung tumor can be removed.

RADIATION THERAPY Radiation therapy uses high-energy rays such as x rays to kill cancer cells:

• External beam radiation—delivered from outside the body—is aimed directly into the sarcoma and is the most common radiation treatment for soft tissue sarcomas.
• Internal radiation therapy (brachytherapy) delivers small pellets of radioactive material directly into the sarcoma through thin plastic tubes. It may be used alone or in combination with external beam radiation.

Radiation may be used:

• before and/or after surgery for all sarcoma stages
• for inoperable stage I and II sarcomas
• as the primary treatment for patients with health conditions that preclude surgery
• to kill small clusters of cancer cells
• to relieve symptoms of stage IVB sarcoma
• as an adjunct treatment 6–9 weeks after chemotherapy
• for recurrent sarcomas that were not treated previously with radiation
• to treat pain accompanying recurrences

Tumors of the retroperitoneum, trunk, head, or neck may be treated with fast neutron therapy.

Short-term side effects of radiation therapy may include:

• fatigue
• mild skin conditions
• infections
• nausea, vomiting, and diarrhea after irradiation of the abdomen
• mouth sores and loss of appetite after head or neck irradiation
• swelling, weakness, or pain following irradiation of large portions of a limb

Longer-term radiation effects can include:

• worsening of chemotherapy side effects
• breathing difficulties and lung damage from chest irradiation
• bone fractures, sometimes occurring years later
• headaches and mental problems one to two years after radiation therapy for metastatic sarcoma in the brain

CHEMOTHERAPY Chemotherapy may be used:

• as primary therapy for some sarcomas
• to shrink a stage II tumor prior to surgery
• as postoperative treatment for stage II sarcomas
• before or after surgery for stage III sarcomas to reduce the risk of recurrence
• to treat metastasized sarcomas
• to reduce pain with stage IV sarcomas
• for recurrence at a distant site

Synovial sarcomas respond more readily to chemotherapy than other soft tissue sarcomas. Chemotherapy usually does not prevent metastasis and the benefits of postoperative chemotherapy in children have been questioned.

Ifosfamide and doxorubicin (Adriamycin) are the most common drugs for treating soft tissue sarcoma. They may be used alone, together, or in combination with other drugs including:

• dacarbazine
• methotrexate
• vincristine
• cisplatin
• paclitaxel
• mesna for protecting the bladder from severe irritation caused by ifosfamide

When used alone only doxorubicin and ifosfamide have response rates above 20%. Doxorubicin alone or in combination with dacarbazine is the most frequently used chemotherapy for advanced sarcomas. High-dose ifosfamide is used to relieve symptoms of inoperable sarcomas.

Postoperative chemotherapy for ERMS is usually vincristine and dactinomycin (actinomycin-D). For group II and III RMS, cyclophosphamide is added for a three-drug combination called VAC. Topotecan also may be included.

Temporary side effects of chemotherapy may include:

• nausea and vomiting
• loss of appetite
• hair loss
• mouth sores

Chemotherpy can damage blood-producing bone marrow cells, increasing the risk of:

• fatigue
• bruising or bleeding
• infection

Most side effects disappear when chemotherapy ends, although it sometimes causes infertility. Doxorubicin can weaken the heart and ifosfamide and cyclophosphamide can cause permanent kidney or bladder damage.

Recurrences

Treatment of recurrent soft tissue sarcomas depends on the initial type and treatment. If the initial treatment was minimal, a local recurrence may be treated with surgery and radiation. If the original treatment was aggressive, limb amputation may be necessary. The lungs are the most common distant site of sarcoma recurrences, usually within two to three years after the initial diagnosis. These are treated as stage IV disease. In older patients symptoms of recurrence may be treated by the sequential use of single chemotherapy drugs. Synovial sarcomas tend to recur locally and involve regional lymph nodes; however distant metastasis occurs in about 50% of cases, sometimes many years later.

Prognosis

Stage I and II soft tissue sarcomas rarely metastasize although they may recur locally if inadequately treated:

• Stage I sarcomas have a five-year-survival rate of 99% and only a 20% chance of recurrence within five years.
• Stage II sarcomas have an 82%-five-year-survival rate and a five-year-recurrence risk of 35%.
• Stage III sarcomas have a five-year-survival rate of 50% and a five-year-recurrence risk of about 65%.
• Stage IV sarcomas are usually incurable with a five-year-survival rate of 10–15%.
• Surgery to remove metastatic lung sarcomas has a five-year-survival rate of 20–30% and occasionally a complete cure.
• Patients over age 60 have a poorer prognosis than younger adults.

In children:

• stage I: 90% never have a recurrence
• stage II: about 89% survive long term and about 50% of recurrences are cured in the second round of treatment
• stage III: about 70% survive long term
• stage IV: a five-year-survival rate of less than 30%, although children under age 10 with metastatic embryonal tumors have a 50% chance of survival.

Younger children with RMS have higher survival rates than older children and adolescents and ERMS has a more favorable prognosis than ARMS. More than 70% of children survive ERMS and second malignancies arise in less than 25% of survivors, usually in children with more advanced disease.

Children with non-RMS generally have a better prognosis than adults, although if the sarcoma is not removed completely, metastasizes, or recurs, the prognosis is poor:

• Leiomyosarcoma has a good prognosis unless it is within the gastrointestinal tract.
• Infantile fibrosarcoma—which occurs in children under five—has an excellent prognosis when treated with surgery alone.
• Adult-type fibrosarcomas have a survival rate of about 60% in both children and adults.
• MFH has a survival rate of about 50%.
• Desmoid tumors rarely metastasize and have an excellent prognosis.
• Liposarcomas have a good prognosis if completely removed.
• The prognosis for angiosarcomas and hemangioendotheliomas depends on their removal, the extent of the disease, and the grade of the malignancy.
• Hemangiopericytoma has an excellent prognosis in young children and an overall survival rate of 30–70%.
• Synovial sarcoma has a survival rate of 80%.
• Neurofibrosarcoma has a very good prognosis with complete removal; otherwise the prognosis is poor.
• Alveolar and clear cell soft-part sarcomas have a 50% survival rate and late relapses are common.

High-grade retroperitoneal sarcoma has a less favorable prognosis because of the difficulty of completely removing the tumor and the limitations on high-dose radiation therapy. Local recurrence is the most common cause of death in these patients.

Clinical Trials

As of 2005 numerous clinical trials for treating soft tissue sarcomas in children and adults were underway, including trials to evaluate:

• chemotherapy prior to surgery
• regional chemotherapy in which drugs are injected directly into the artery that supplies an affected limb
• new drugs for reducing heart damage from doxorubicin, so that higher doses can be used
• the use of radiation therapy during surgery for abdominal and retroperitoneal sarcomas
• drugs such as interleukin-2 to boost the immune system
• vaccines that may cause the immune system to recognize abnormal chemicals in sarcomas and destroy the cells
• stem-cell transplantations that allow higher levels of chemotherapy for treating RMS.

Prevention

Most soft tissue sarcomas develop in people with no known risk factors. Since early detection is very important, a healthcare professional should be consulted about unexplained lumps, growths, or other symptoms. Less than 5% of soft tissue sarcomas are caused by radiation exposure. Lymphangiosarcomas can develop where lymph nodes have been damaged by radiation or surgically removed.

A high percentage of patients with angiosarcoma of the liver have been exposed to vinyl chloride. Although exposures to other chemicals—including dioxin, herbicides containing phenoxyacetic acid, and chlorophenols in wood preservations—have been suggested as risk factors for soft tissue sarcoma, there are no proven connections.

The only known risk factors in children are congenital (present at birth) abnormalities and genetic (inherited) conditions:

• Li-Fraumeni syndrome increases the risk of soft tissue sarcomas as well as other types of cancer and there is a high risk of developing soft tissue sarcoma in an area that was irradiated to treat another cancer.
• Children with inherited retinoblastoma—an eye cancer—are at increased risk for soft tissue sarcoma.
• Children with Beckwith-Wiedemann syndrome are at risk for developing RMS.
• Gardner's syndrome increases the risk of desmoid tumors in the abdomen.
• Neurofibromatosis or von Recklinghausen's disease is characterized by benign neurofibromas; in about 5% of cases these develop into malignant peripheral nerve sheath tumors.

Those with a family history of sarcomas or other cancers occurring at a young age may have genetic testing to assess their risk.

Questions to Ask Your Doctor

• What type of sarcoma do I have?
• Has the cancer spread?
• What stage is the cancer and what does that mean for me?
• What are the treatment options?
• What treatment do you recommend and why?
• What are the risks and side effects of each treatment?
• What are the risks of recurrence after each treatment?
• How should I prepare for the treatment?
• How much work or school will be missed?
• What is the recovery time after treatment?
• What is my estimated survival time?

Special Concerns

Since advanced soft tissue sarcoma has a high risk of metastasis and recurrence, following treatment a patient may have:

• frequent physical examinations
• chest x rays, ultrasound, or CT or MRI scans

Resources

Books

Meyer, W. H. 'Soft Tissue Sarcomas.' In Cancer Medicine. 6th ed., edited by D. W. Kufe, et al. Hamilton, Ontario: BC Decker, 2003: 2377–82.

Pizzo, P. A., and D. G. Poplack, editors. Prinicples and Practice of Pediatric Oncology. 4th ed. Philadelphia: Lippincott, Williams and Wilkins, 2002.

Pollack, Raphael E. Soft Tissue Sarcomas. Atlanta: American Cancer Society, 2002.

Weiss, S. W., and J. R. Goldblum. Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis: Mosby, 2001.

Yasko, A., et al. 'Sarcomas of Soft Tissue and Bone.' In Clinical Oncology, edited by R. E. Lenhard Jr, et al. Atlanta: American Cancer Society, 2001: 611-32.

Periodicals

O'Sullivan, B., et al. 'Preoperative Versus Postoperative Radiotherapy in Soft-Tissue Sarcoma of the Limbs: A Randomized Trial.' The Lancet 359, no. 9325 (2002): 2235–41.

Organizations

American Cancer Society. PO Box 102454, Atlanta, GA 30368-2454. 800-ACS-2345. . Information, research, and patient support.

CureSearch. 4600 East West Highway, #600, Bethesda, MD 20814-3457. 800-458-6223. 240-235-2200. . Children's Oncology Group and the National Childhood Cancer Foundation. Information, research, and advocacy.

National Cancer Institute. Public Inquiries Office, Suite 30361, 6116 Executive Blvd., MSC-8322, Bethesda, MD 20892-8322. 800-4-CANCER (800-422-6237). . Information, research, and clinical trials.

National Children's Cancer Society. 1015 Locust, Suite 600, St. Louis, MO 63101. 800-5-FAMILY (800-532-6459). 314-241-1600. . Financial assistance, support services, advocacy, and education.

Other

Adult Soft Tissue Sarcoma (PDQ): Treatment, Health Professional Version. February 1, 2005. National Cancer Institute. March 30, 2005. .

Adult Soft Tissue Sarcoma (PDQ): Treatment, Patient Version. April 22, 2004. National Cancer Institute. March 30, 2005. .

Childhood Soft Tissue Sarcoma (PDQ): Treatment, Health Professional Version. February 17, 2005. National Cancer Institute. March 30, 2005. .

Childhood Soft Tissue Sarcoma (PDQ): Treatment, Patient Version. February 16, 2005. National Cancer Institute. March 30, 2005. .

Gurney, James G., et al. 'Soft Tissue Sarcomas.' SEER Pediatric Monograph. National Cancer Institute. March 30, 2005. .

Rhabdomyosarcoma. September 11, 2003. American Cancer Society. March 30, 2005. .

Sarcoma—Adult Soft Tissue Cancer. January 5, 2005. American Cancer Society. March 30, 2005. .

'Soft Tissue Sarcomas: Questions and Answers.' Cancer Facts. May 6, 2002. National Cancer Institute. March 30, 2005. .

—Margaret Alic, Ph.D.

Gale Encyclopedia of Cancer. Copyright © 2006 by The Gale Group, Inc. All rights reserved.