Sarcoma

Sarcoma
Classification and external resources
ICD-O:	M8800/3
MeSH	D012509

A sarcoma (from the Greek sarx meaning "flesh") is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm.^[1] Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues. (This is in contrast to carcinomas, which originate from epithelial cells and thus give rise to more common malignancies, such as breast, colon, and lung cancer.)

Classification

Optical Coherence Tomography (OCT) image of a sarcoma

Tissue

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, liposarcoma arises from fat, and leiomyosarcoma arises from smooth muscle.

Grade

In addition to being named based on the tissue of origin, sarcomas are also assigned a grade (low, intermediate, or high) based on the presence and frequency of certain cellular and subcellular characteristics associated with malignant biological behavior. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. Intermediate and high grade sarcomas are more frequently treated with a combination of surgery, chemotherapy and/or radiation therapy.^[2] Since higher grade tumors are more likely to undergo metastasis (invasion and spread to locoregional and distant sites), they are treated more aggressively. The recognition that many 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%.^[3]

Types

(ICD-O codes are provided where available.)

• Askin's Tumor (8803/3)
• Botryodies
• 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 Phyllodes
  • Dermatofibrosarcoma protuberans (DFSP) (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)(Also known as "solitary fibrous tumor". Only a subset of these tumors are classified as malignant.)
  • Hemangiosarcoma (9120/3) (More commonly referred to as "angiosarcoma")
  • Kaposi's sarcoma (9140/3)
  • Leiomyosarcoma (8890/3-8896/3)
  • Liposarcoma (8850/3-8858/3)
  • Lymphangiosarcoma (9170-9175)
  • Lymphosarcoma (Not considered to be sarcomas)
  • Malignant fibrous histiocytoma (8830/3)(This is an obsolete term that is no longer recognized by the World Health Organization. Many of these tumors would currently be classified as "undifferentiated pleomorphic sarcoma".)
  • Malignant peripheral nerve sheath tumor (MPNST)
  • Neurofibrosarcoma (9540/3)
  • Rhabdomyosarcoma (8900-8920)
  • Synovial sarcoma (9040/3-9043/3)
  • Undifferentiated pleomorphic sarcoma (previously referred to as Malignant fibrous histiocytoma)

Treatment

Surgery is important in the treatment of most sarcomas.^[4] Limb sparing surgery, as opposed to amputation, can now be used to save the limbs of patients in at least 90% of extremity tumor cases.^[5] Additional treatments, including chemotherapy and radiation therapy, may be administered before and/or after surgery.^[6] Chemotherapy significantly improves the prognosis for many sarcoma patients, especially those with bone sarcomas.^[7] Treatment can be a long and arduous process, lasting about a year for many patients, but the process is necessary in order to cure the disease.^[8]

• Liposarcoma treatment consists of surgical resection, with chemotherapy not being used outside of the experimental setting. Adjuvant radiotherapy may also be used after surgical excision for liposarcoma.^[9]
• Rhabdomyosarcoma is treated with surgery, radiotherapy, and/or chemotherapy.^[10] The majority of rhabdomyosarcoma patients have between a 50-85% survival rate.^[11]
• Osteosarcoma is treated with surgical resection of as much of the cancer as possible, often along with neoadjuvant chemotherapy.^[12] Radiotherapy is a second alternative although not as successful.

Epidemiology

Sarcomas are quite rare with only 15,000 new cases per year in the United States.^[13] Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year,^[14] and are well below the US's orphan disease threshold of 200,000 cases per year.

Gastrointestinal stromal tumor (GIST) is the most common form of sarcoma, with approximately 3,000-3,500 cases per year in the United States.^[15]

Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35.^[16] Some sarcomas, such as leiomyosarcoma, chondrosarcoma, and gastrointestinal stromal tumor (GIST), are more common in adults than in children. Most high-grade bone sarcomas, including Ewing's sarcoma and osteosarcoma, are much more common in children and young adults.

References

1. ^ John D, Pfeifer, Mark R Wick (2001). "Pathologic Evaluation of Neoplastic Diseases". American Cancer Society's, Clinical Oncology. Blackwell Publishing, Inc. p. 124. 
2. ^ Buecker, P (2005). "Sarcoma: A Diagnosis of Patience". ESUN 2 (5). http://sarcomahelp.org/learning_center/patience.html. Retrieved 2009-04-15. 
3. ^ Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G (Oct 2006). "Primary bone osteosarcoma in the pediatric age: state of the art". Cancer Treat Rev. 32 (6): 423–36. doi:10.1016/j.ctrv.2006.05.005. PMID 16860938. 
4. ^ Morris, C (2005). "Malignant Fibrous Histiocytoma (MFH)". ESUN 2 (2). http://sarcomahelp.org/learning_center/mfh.html#surgery. Retrieved 2011-10-19. 
5. ^ Morris, C (2005). "Malignant Fibrous Histiocytoma (MFH)". ESUN 2 (2). http://sarcomahelp.org/learning_center/mfh.html#surgery. Retrieved 2011-10-19. 
6. ^ Buecker, J (2005). "Sarcoma: A Diagnosis of Patience". ESUN 2 (5). http://sarcomahelp.org/learning_center/patience.html. Retrieved 2011-10-19. 
7. ^ Baker, L (2006). "A Rose is a Rose or a Thorn is a Thorn". ESUN 3 (5). http://sarcomahelp.org/learning_center/chemotherapy_rose.html. Retrieved 2011-10-19. 
8. ^ Buecker, J (2005). "Sarcoma: A Diagnosis of Patience". ESUN 2 (5). http://sarcomahelp.org/learning_center/patience.html. Retrieved 2011-10-19. 
9. ^ http://emedicine.medscape.com/article/1102007-treatment
10. ^ http://www.childrenshospital.org/az/Site1068/mainpageS1068P0.html
11. ^ Wexler, L (2004). "Rhabdomyosarcoma". ESUN 1 (4). http://sarcomahelp.org/learning_center/rhabdomyosarcoma.html. Retrieved 2011-10-19. 
12. ^ http://emedicine.medscape.com/article/1256857-treatment
13. ^ Borden EC, Baker LH, Bell RS, et al. (Jun 2003). "Soft tissue sarcomas of adults: state of the translational science". Clin Cancer Res. 9 (6): 1941–56. PMID 12796356. http://clincancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=12796356. 
14. ^ http://www.cancer.org/docroot/STT/content/STT_1x_Cancer_Facts__Figures_2009.asp?from=fast
15. ^ Tran T, Davila JA, El-Serag HB (Jan 2005). "The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000". Am J Gastroenterol 100 (1): 162–8. doi:10.1111/j.1572-0241.2005.40709.x. PMID 15654796. 
16. ^ Darling, J (2007). "A Different View of Sarcoma Statistics". ESUN 4 (6). http://sarcomahelp.org/sarcoma_statistics.html. Retrieved 2009-04-15. 

External links

• Sarcoma at the Open Directory Project