Soft-tissue sarcoma

Sarcoma is a group of rare solid tumors of connective tissue. More than 50 different histologic subtypes are known.

Most commonly it presents as a soft-tissue swelling, which may or may not be painful. Differential diagnoses that should be considered are lymphoma, metastatic carcinoma, and benign lesions such as lipoma and neuroma.

Some sarcomas are associated with specific gene mutations, in particular chromosomal translocations.

Diagnosis is made by biopsy, which should have a sufficient amount of tissue for accurate pathologic diagnosis.

Pathology should be reviewed by an expert pathologist who has experience in sarcoma histology. Diagnostic laboratory methods may include immunohistochemistry, cytogenetics, and molecular genetic testing.

Treatment is based on stage and histological subtype, with only some subtypes being chemotherapy-sensitive.

Sarcoma is a group of rare solid tumors originating in mesenchymal (connective tissue) cells and accounting for 1% of all adult malignancies. They can be divided into 2 broad categories: sarcoma of soft tissues (including fat, muscle, nerve and nerve sheath, blood vessels, and other connective tissue) and sarcoma of bone.[1]Pisters PW, Casper ES, Mann GN. Soft-tissue sarcomas. In: Pazdur R, Coia LR, Hoskins WJ, et al, eds. Cancer management: a multidisciplinary approach. 9th ed. Melville, NY: PRR Inc.; 2005:585-614.[2]Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004 Mar-Apr;54(2):94-109. http://onlinelibrary.wiley.com/doi/10.3322/canjclin.54.2.94/full http://www.ncbi.nlm.nih.gov/pubmed/15061599?tool=bestpractice.com [3]Jemal A, Siegel R, Xu J, et al. Cancer statistics, 2010. CA Cancer J Clin. 2010 Sep-Oct;60(5):277-300. http://onlinelibrary.wiley.com/doi/10.3322/caac.20073/full http://www.ncbi.nlm.nih.gov/pubmed/20610543?tool=bestpractice.com [4]Pisters P. Staging and prognosis. In: Pollock RE, ed. American Cancer Society atlas of clinical oncology: soft tissue sarcomas. Hamilton, Ontario: BC Decker; 2002:80-8.[5]Clark MA, Fisher C, Judson I, et al. Soft tissue sarcomas in adults. N Engl J Med. 2005 Aug 18;353(7):701-11. http://www.ncbi.nlm.nih.gov/pubmed/16107623?tool=bestpractice.com [6]Coindre JM, Terrier P, Guillou L, et al. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group. Cancer. 2001 May 15;91(10):1914-26. http://www.ncbi.nlm.nih.gov/pubmed/11346874?tool=bestpractice.com [7]Dileo P, Demetri GD. Update on new diagnostic and therapeutic approaches for sarcomas. Clin Adv Hematol Oncol. 2005 Oct;3(10):781-91. http://www.ncbi.nlm.nih.gov/pubmed/16258489?tool=bestpractice.com [8]Borden EC, Baker LH, Bell RS, et al. Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res. 2003 Jun;9(6):1941-56. http://clincancerres.aacrjournals.org/content/9/6/1941.long http://www.ncbi.nlm.nih.gov/pubmed/12796356?tool=bestpractice.com More than 50 different histologic subtypes of sarcoma of soft tissues are known.[9]Fletcher CD, Gustafson P, Rydholm A, et al. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol. 2001 Jun 15;19(12):3045-50. http://www.ncbi.nlm.nih.gov/pubmed/11408500?tool=bestpractice.com [10]Fletcher CD, Bridge JA, Hogendoorn PCW, et al., eds. WHO/IARC classification of tumours: World Health Organization classification of tumours of soft tissue and bone. 4th ed (Vol 5). Lyon, France: IARC Press; 2013.