Soft Tissue Sarcoma Subtype Information


Liposarcoma is the most commonly diagnosed soft tissue tumor. They are also among the largest category of sarcomas reported. These tumors usually develop in the deep fatty tissue. They most commonly occur in the thigh, behind the knee, the groin, the gluteal area or behind the abdominal cavity (retroperitoneum). Liposarcomas are usually malignant (cancerous) and rarely are from a pre-existing lipoma, which is a non-cancerous tumor. They are most commonly found in adults between 30 and 60 years old and are slightly more common in men than women.

Tumors are usually firm and bumpy and invade surrounding tissue aggressively. Metastases (spreading) to the lymph nodes occur in approximately 10% of patients with liposarcoma. They are very different than lipomas, which are superficial and typically soft and smooth and are mobile.

Fibrosarcoma (Fibrous Tissue)
Fibrosarcomas usually occur in the arms or legs or on the trunk but can occur in any of the soft tissues. They can occur around scars, muscles, nerves, tendons, and around the lining of the bone (periosteum). Local recurrence after surgery is common. Fibrosarcomas typically invade local tissues and can metastasize through the bloodstream to the lungs. Less than 5% of patients will experience metastases to the lymph nodes.

Dermatofibrosarcoma Protuberans
This lesion develops as a tumor sticking out of the skin usually found on the back or abdomen. In the early stages the tumor remains under the skin and the skin surface appears dark red-violet. If untreated the tumor can break through the skin forming an open wound. It has a tendency to recur but rarely metastasizes. Treatment consists of wide surgical excision.

Malignant Fibrous Histiocytoma (MFH)
Malignant fibrous histiocytomas are the most commonly diagnosed soft tissue sarcoma in patients between the ages of 50 and 70. It is twice as common in men than women.

There are four variations of MFH: storiform pleomorphic, myxoid malignant fibrous histiocytoma, malignant giant cell tumor of soft parts, and inflammatory malignant fibrous histiocytoma. Storiform and Myxoid MFH types are typically high grade (fast growing and invasive) tumors, whereas, malignant giant cell MFH and inflammatory MFH tend to be lower grade (slower growing and less invasive) tumors. While the Myxoid type is lower grade they tend to have local recurrence rate and therefore treatment usually consists of radical resection or amputation. Storiform pleomorphic MFH is typically a deep-seated large tumor of the thigh. The deeper and larger tumors found in MFH have a higher rate of metastases than the more superficial smaller tumors.

Synovial Cell Sarcoma
Synovial Cell sarcoma usually occurs in young adults. They are most commonly found in the arms or legs next to a joint. They are usually found around the joint capsule but rarely invade the joint itself. The most common site is adjacent to the knee. They are also commonly found near the foot, ankle and hand.

Unlike other soft tissue sarcomas, synovial cell sarcomas are often painful. Treatment usually consists of radical excision with radiation and chemotherapy or amputation combined with chemotherapy.

Epithelioid Sarcomas
Epithelioid sarcomas typically occur in the hand or foot of young adults. They appear like small nodules, which sometimes merge together. Spreading to lymph nodes occurs in approximately 20% of patients. Because of their proximity to the joints, surgical excision can be difficult and often amputation is the surgery of choice for long-term survival.

Rhabdomyosarcomas (Striated muscle tumor)
Cancerous tumors in the striated or skeletal muscle are one of the most common types of soft tissue sarcoma, and account for about half the soft tissue sarcomas diagnosed in children. There are several different types, including pleomorphic, alveolar, embryonal and botryoid.

  • Embryonal Rhabdomyosarcoma
    This is the most common type of rhabdomyosarcoma, and is diagnosed most frequently in children under the age of 10. It can be found anywhere in the body, butoften occurs in the head and neck especially around the eye. Chemotherapy is very effective in these tumors. There is a high cure rate in these tumors when combination therapy is used (i.e. surgery and/or radiation combined with chemotherapy).
  • Alveolar Rhabdomyosarcoma
    This tumor is extremely aggressive and typically occurs in adolescents and young adults. Tumors metastasize widely at an early stage.  Alveolar rhabdomyosarcoma almost always is associated with a translocation between two genes (PAX3 or PAX7 and FOX01).  Treatment includes radiation and chemotherapy as well as surgery for some cases.  Localized alveolar rhabdo responds fairly well to treatment.
  • Botryoid Rhabdomyosarcoma
    These lesions usually occur in children at the average age of 7. They generally occur in the genital region and urinary tract. They appear as a swollen lumpy mass.
  • Pleomorphic Rhabdomyosarcoma
    This tumor occurs most commonly in people over 30 years of age. It commonly affects males on their extremities but not exclusively. Because it grows in the skeletal muscle, it usually is deep in the limb. Tumors can arise at many sites within the same muscle group and grow very rapidly. The tumor spreads through the bloodstream although in approximately 15% of cases the tumor spreads through the lymphatic system. Rhabdomyosarcoma is often widely spread at the time of diagnosis. Treatment generally consists of surgery and combination chemotherapy (multiple drugs used).


Leiomyosarcoma (Smooth Muscle Tumor) and Uterine SarcomaLeiomyosarcomas are cancerous tumors of the smooth muscle. They most commonly occur in the organs (e.g., gastrointestinal tract and the uterus). The average age of patients is 60 years. Of the tumors occurring in the GI tract 61% occur in the stomach, 29% in the small bowel, and 10% in the colon. Symptoms of GI or uterine leiomyosarcomas are significant bleeding and pain. Metastases occur in more than half of patients.

Leiomyosarcomas of the retroperitoneum and the vena cava are found mostly in women. The growth of the tumors can accelerate during pregnancy. Metastases usually occur in the lungs except in GI tumors, which often metastasize in the liver. Treatment for uterine leiomyosarcoma is total abdominal hysterectomy.

Gastrointestinal Sarcoma, otherwise known as Gastrointestinal Stromal Tumor (GIST).

GIST develops in the stroma, the supporting connective tissue, of the stomach and intestines.  It is now often treated with the biological agent Gleevec.  Gleevec is a biologic agent that works by shutting down the activities of gtenes that apparently are needed for the tumor’s growth.  In particular, it targets a gene called c-kit that produces inappropriate amounts of an enzyme that promotes the tumor growth.  Extraosseous Tumors
There are three types of Extraosseous tumors. They are giant cell tumors, osteosarcomas, and Ewing’s sarcoma. These are bone sarcomas, which are found in the soft tissue. They have very similar characteristics as their bone sarcoma variation, Extraosseous Ewing’s sarcomas respond well to radiation therapy.

Myxomas occur in men and women averaging the age of 50. They are most often found in the arms and legs. Tumors can be small nodules or very large tumors. They do not metastasize; however, they do spread locally. Treatment is usually wide local excision.

Mesenchymomas can be distributed throughout the body and approximately 75% are malignant. The tumors are invasive especially when they are located in the skeletal muscle. Treatment is wide excision sometimes combined with radiation and or chemotherapy.

Vascular Sarcomas (containing many blood vessels)

  • Hemangioendothelioma
    Hemangioendotheliomas are a very rare vascular (containing many blood vessels) tumor, which can be malignant. They occur in men and women but rarely in children. One variety of this tumor occurs in younger males and typically arises on the hand and tends to recur locally.
  • Angiosarcomas
    Angiosarcomas represent 1% of all sarcomas. One-third of the cases occur on the skin, one-fourth of the cases occur in the soft tissue or organs such as breast, liver, heart and lungs. Tumors arising in the liver are seen in adults exposed to angiography, insecticides or plastics. Angiosarcomas of the breast typically occur in young or middle aged women. Additionally some angiosarcomas occur at the site of previous radiation therapy. Tumors metastasize widely. The most common form of treatment is radical amputation.
  • Hemangiopericytoma
    Hemangiopericytomas are typically found in the thigh, retroperitoneum or near the kidneys. Intracranial hemangiopericytoma grow along the sinuses, tend to locally recur and can metastasize.

Malignant Neurilemoma (Malignant Schwannoma & Neurosarcoma)
Malignant Neurilemomas usually develop in young to middle aged adults, most commonly in males. They occur in the peripheral nerves (not brain or spinal cord nerves). Half of these tumors occur in people with Von Recklinghausen’s disease or multiple neurofibromatosis. The spreading of these tumors occurs in the surrounding soft tissue forming a nodular tumor. Metastases can occur through the bloodstream. Tumors can be painful, tender and sometimes nerve function is affected. Treatment is generally wide resection of the nerve in either direction. Radiation therapy and chemotherapy can be used as well.

Alveolar Soft-Parts Sarcoma
This is a very rare tumor that typically occurs in female adolescents and young adults. It is usually a slow growing tumor found in the extremities and commonly metastasizes to the lung. The typical form of treatment is wide resection of the lesion.

Kaposi’s Sarcoma
Kaposi’s Sarcoma is a disease in which cancerous cells are found in the tissues under the skin or mucous membranes that line the mouth, nose and anus. There are three groups of patients for Kaposi’s sarcoma. The first group typically includes older men of Jewish, Italian or Mediterranean heritage. This type of Kaposi’s usually progresses slowly over 10-15 years. Patients commonly develop a bluish lesion on the front of the lower leg, which typically spreads to multiple lesions. After some time the disease can spread to other organs.

The second group of Kaposi’s sarcoma occurs in patients who have received organ transplant. Due to the Immunosuppressive treatment following a transplant, patients immune systems are weakened thus are more susceptible to infection.

The third group of Kaposi’s sarcoma is found in AIDS patients. This group is referred to as epidemic Kaposi’s sarcoma. Due to the weakened immune system cause by the HIV virus, Infections and other diseases such as Kaposi’s can invade the body. Kaposi’s sarcoma in people with AIDS usually spreads more quickly and can be found in many parts of the body.

Radiation therapy is usually the treatment for Kaposi’s; however, when lesions have spread to the organs, chemotherapy is often used as well.

For Additional Information


Gastrointestinal Stromal Tumor (GIST)
GIST Support International

Gastrointestinal Stromal Tumor (GIST)
Life Raft Group
Kaposis Sarcoma

Kaposi’s Sarcoma

Kaposi’s Sarcoma Treatment



LMSarcoma Direct Research

National Leiomyosarcoma Foundation

Leiomyosarcoma News – LMSeAlerts

LMS Lifeline, with new Articles added regularly


Osteosarcoma and bone tumors


Bone Tumor Information

Osteosarcoma/Malignant Fibrous Histiocytoma of Bone Treatment

Ewing Sarcoma and Pediatric Cancers




Childhood Rhabdomyosarcoma

The Alliance Against Alveolar Soft Part Sarcoma (ASPS)


Synovial Sarcoma

Synovial Survivors Network


Look Up Specific Soft Tissue Sarcoma Diagnosis Here

The National Cancer Institute’s Soft Tissue Sarcoma Home Page

The National Cancer Institute’s Bone Cancer Home Page

The National Cancer Institute’s Uterine Sarcoma Home Page

General Sarcoma Information

National Cancer Institute Adult Soft Tissue Treatment PDQ (Physician Data Query)

University of Pennsylvania OncoLink Sarcoma Page

British Columbia Cancer Agency – Bone and Connective Tissue Sarcomas

Dr. Steve Fadem’s Sarcoma Page – Doctor with Sarcoma

Surviving Cancer – Sarcoma Survivor’s Website

Management of Soft Tissue Sarcomas: An Overview and Update
Excellent article about soft tissue sarcoma in the journal The Lancet Oncology. There is a fee for the complete article.

MDAnderson Cancer Center Sarcoma Practice Guidelines

Sarcoma Specific Organizations

The Sarcoma Foundation of America

Connective Tissue Oncology Society

Desmoplastic Small Round Cell Tumor – Gift to the Cure will help DSRCT patients with information and support:

Kristen Ann Carr Fund Sarcoma Forum

Amschwand Sarcoma Cancer Foundation

The Liddy Shriver Sarcoma Initiative

CureSearch (Children’s Oncology Group / National Childhood Cancer Foundation

Jennifer Hunter Yates Sarcoma Foundation

Karen Wyckoff Rein Sarcoma Fund

Northwest Sarcoma Foundation

Organizations outside the United States

SOS Desmoide Association