Sarcoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
A sarcoma (from the Greek 'sarx' meaning "flesh") is a cancer of the connective or supportive tissue (bone, cartilage, fat, muscle, blood vessels) and soft tissue. This is in contrast to carcinomas, which are of epithelial origin (breast, colon, pancreas, and others).
Classification
Sarcomas are given a number of different names, based on the type of tissue from which they arise. For example, osteosarcoma arises from bone, chondrosarcoma arises from cartilage, and leiomyosarcoma arises from smooth muscle. Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of cancer.[1] Soft tissue sarcomas, such as leiomyosarcoma, chondrosarcoma, and gastrointestinal stromal tumor (GIST), are more common in adults than in children. GIST is the most common form of sarcoma, with approximately 3000-3500 cases per year in the United States.[2] This should be compared with breast cancer, with approximately 200,000 cases per year in North America.[3] Bone sarcomas, such as osteosarcoma and Ewing's sarcoma, are more common in children than in adults. These tumors most commonly strike adolescents and young adults between the ages of 12 and 25. In addition to being named based on the tissue of origin, sarcomas are also assigned a grade, such as low grade or high grade. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. High grade sarcomas are more frequently treated with chemotherapy. Since these tumors are more likely to undergo metastasis (spreading to distant sites), these tumors are treated more aggressively. Childhood sarcomas are almost always treated with a combination of surgery and chemotherapy, and radiation is frequently used as well. The recognition that childhood sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60-70%.[4]
Tables
| Tissue of Origin | Type of Cancer | Usual Location in the Body |
|---|---|---|
| Fibrous tissue | Fibrosarcoma | Arms, legs, trunk |
| Malignant fibrous hystiocytoma |
Legs | |
| Dermatofibrosarcoma | Trunk | |
| Fat | Liposarcoma | Arms, legs, trunk |
| Muscle |
Rhabdomyosarcoma Leiomyosarcoma |
Arms, legs Uterus, digestive tract |
| Blood vessels | Hemangiosarcoma | Arms, legs, trunk |
| Kaposi's sarcoma | Legs, trunk | |
| Lymph vessels | Lymphangiosarcoma | Arms |
| Synovial tissue (linings of joint cavities, tendon sheaths) |
Synovial sarcoma | Legs |
| Peripheral nerves | Malignant peripheral nerve sheath tumour/Neurofibrosarcoma | Arms, legs, trunk |
| Cartilage and bone-forming tissue | Extraskeletal chondrosarcoma | Legs |
| Extraskeletal osteosarcoma | Legs, trunk (not involving the bone) |
| Tissue of Origin | Type of Cancer | Usual Location in the Body | Most common ages |
|---|---|---|---|
| Muscle | |||
| Rhabdomyosarcoma | |||
| Head and neck, genitourinary tract | Infant–4 | ||
| Arms, legs, head, and neck | Infant–19 | ||
| Leiomyosarcoma | Trunk | 15–19 | |
| Fibrous tissue | Fibrosarcoma | Arms and legs | 15–19 |
| Malignant fibrous histiocytoma |
Legs | 15–19 | |
| Dermatofibrosarcoma | Trunk | 15–19 | |
| Fat | Liposarcoma | Arms and Legs | 15–19 |
| Blood vessels | Infantile hemangio- |
Arms, legs, trunk, head, and neck | Infant–4 |
| Synovial tissue (linings of joint cavities, tendon sheaths) |
Synovial sarcoma | Legs, arms, and trunk | 15–19 |
| Peripheral nerves | Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas) | Arms, legs, and trunk | 15–19 |
| Muscular nerves | Alveolar soft part sarcoma | Arms and legs | Infant–19 |
| Cartilage and bone-forming tissue | Extraskeletal myxoid chondrosarcoma | Legs | 10–14 |
| Extraskeletal mesenchymal | Legs | 10–14 |
Types of sarcoma
(ICD-O codes are provided where available.)
- Askin's Tumor (8803/3)
- Chondrosarcoma (9220/3-9240/3)
- Ewing's (9260/3) - PNET (9473/3)
- Malignant Hemangioendothelioma (9130/3)
- Malignant Schwannoma (9560/3-9561/3)
- Osteosarcoma (9180/3-9190/3)
- Soft tissue sarcomas, including:
- Alveolar soft part sarcoma (9581/3)
- Angiosarcoma (9120/3)
- Cystosarcoma Phylloides[2]
- Dermatofibrosarcoma (8832/3-8833/3)
- Desmoid Tumor (8821/1-8822/1)
- Desmoplastic small round cell tumor (8806/3)
- Epithelioid Sarcoma (8804/3)
- Extraskeletal chondrosarcoma (9220/3)
- Extraskeletal osteosarcoma (9180/3)
- Fibrosarcoma (8810/3)
- Hemangiopericytoma (9150)
- Hemangiosarcoma (9120/3)
- Kaposi's sarcoma (9140/3)
- Leiomyosarcoma (8890/3-8896/3)
- Liposarcoma (8850/3-8858/3)
- Lymphangiosarcoma (9170-9175)
- Lymphosarcoma
- Malignant fibrous histiocytoma (8830/3)
- Neurofibrosarcoma (9540/3)
- Rhabdomyosarcoma (8900-8920)
- Synovial sarcoma (9040/3-9043/3)
Causes
Differential Diagnosis
Soft tissue sarcoma needs to differentiate from soft tissue benige tumors such as:
Drug Side Effect
References
- ↑ Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, Fletcher CD, Fletcher JA, Ladanyi M, Meltzer P, O'Sullivan B, Parkinson DR, Pisters PW, Saxman S, Singer S, Sundaram M, van Oosterom AT, Verweij J, Waalen J, Weiss SW, Brennan MF. Soft tissue sarcomas of adults: state of the translational science.Clin Cancer Res. 2003 Jun;9(6):1941-56. Review. PMID 12796356
- ↑ Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005;100:162-8. PMID 15654796
- ↑ Smigal C, Jemal A, Ward E, Cokkinides V, Smith R, Howe HL, Thun M. Trends in breast cancer by race and ethnicity: update 2006. CA Cancer J Clin 2006;56:168-83. PMID 16737949
- ↑ Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: state of the art. Cancer Treat Rev. 2006;32:423-36. PMID 16860938
External Links
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