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Most common soft tissue sarcoma subtypes are listed below with less common, benign, and low-grade variations included because of the diagnostic overlap and challenges of diagnosing and differentiating specific malignant subtypes and benign from low-grade malignancies.
For additional information see National Comprehensive Cancer Network® NCCN Guidelines for Patients® Soft Tissue Sarcoma.
The most common lipomatous sarcoma is liposarcoma which includes multiple diagnostic subtypes. This sarcoma arises from fat cells or their precursors. Each subtype has its own specific biology and risk of recurrence or spread.
Desmoid tumors form in the connective tissues of the body and can occur anywhere. They are a rare, locally invasive, soft tissue tumor. Though they do not metastasize they can be aggressive and damage surrounding tissues and structures as they grow.
These very rare connective tissue tumors, including pigmented villonodular synovitis (PVNS), arise most commonly near the knee joint, but they can also affect large and small joints alike. They are initially removable with surgery, but some have a high risk of recurrence.
This uncommon group of sarcomas appear to arise from the lining of blood vessels (endothelial cells) or their precursors. They include less aggressive (EHE) and more aggressive forms (angiosarcomas). Some blood-vessel sarcomas can affect the lymphatic system (which carries fluid from soft tissue to lymph nodes). These tumors have a high risk of recurring where they start and can also travel to the liver, bone, lung, and other sites.
This is a tumor of smooth muscle (or its precursors) and can arise anywhere in the body. Leiomyosarcoma is one of the most common types of sarcoma. Common initial sites for this tumor are the uterus, small intestine or stomach, or the wall of a blood vessel in the abdomen, extremity, or skin. These sarcomas have a highly variable behavior, but when they metastasize they characteristically travel to the lungs and less commonly to the liver, bone, and soft tissues.
This rare sarcoma typically affects children. Rhabdmyosarcomas are themselves a separate family of sarcomas, with several recognized subtypes. Treatment for these sarcomas nearly always involves surgery, radiation, and chemotherapy. Cure rates are better for children than for adults, for unclear reasons. This is one form of sarcoma that can travel to lymph nodes, though it can also travel to lungs and other sites.
GIST is one of the most common types of sarcoma. It appears to arise from the interstitial cells of Cajal (or its precursors), which are the pacemaker cells of the intestines. In other words, they are responsible for the “peristaltic wave” that pushes food through the intestines. GIST can occur anywhere in the gastrointestinal tract. The common places that GIST recur are in the abdominal cavity or in the liver.
Extraskeletal osteosarcoma arises in soft tissue, but looks just like its counterpart in bone. It typically arises in older adults, not in children.
MPNST sarcomas arise from the insulating cells that surround nerve endings. They can occur in people who have a familial disposition to forming benign versions of these tumors called neurofibromas or schwannomas. This condition is called neurofibromatosis type I and carries with it the risk of developing MPNSTs and other tumors, such as relatively less aggressive tumors that affect the brain or nerve sheaths of nerve endings as they leave the brain, but within the skull.
Sarcoma types that share no common cellular resemblance to other soft tissue sarcomas.
Source: “WHO Classification of Tumors Editorial Board: Soft Tissue and Bone Tumors,” Lyon (France): International Agency for Research on Cancer; 2020 [internet December 29, 2024]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumorclassification.iarc.who.int/chapters/33.