clear cell sarcoma of tendons & aponeuroses (malignant melanoma of soft parts)
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Introduction
Neuroectodermal tumor, usually deep seated, bound to tendons or aponeuroses, with no connection to overlying skin.
Epidemiology
- rare
- mostly young adults, ages 20 - 40 years (age range 7-83)
- women more than men
Pathology
anatomic distribution:
- lower extremity 75.1%
- upper extremity 22%
- trunk 2.1%
- head & neck 0.8%
size most important prognostic factor
necrosis also portends poor prognosis
Microscopic pathology
- nests or fascicles of rounded or fusiform cells
- clear cytoplasm
- framework of fibrocollagenous tissue
- often contain intracellular melanin Immunohistochemistry
- S100: +
- vimentin: +
- tyrosinase: +
- HMB-45: +
- Melan-A: +
- MITF-1: +
- cytokeratin -
- EMA: -
Genetics
- associated with chromosomal translocation t(12;22)(q13;q12) involving EWS & ATF-1 genes
Clinical manifestations
- slowly enlarging mass
- tenderness or pain ~1/2
- may present as a cutaneous disorder[4]
Differential diagnosis
Management
- surgical resection
- multiple recurrences
- prognosis
- late metastasis
- death likely
More general terms
References
- ↑ Enzinger & Weiss. Soft Tissue Tumors. Mosby 3rd ed. 1995
- ↑ WHO Classification Tumours of Soft Tissue and Bone Fletcher, Unni & Mertens Eds. IARC Press 2002
- ↑ Dabbs. Diagnostic Immunohistochemistry. Churchill-Livingstone, 2002. page 91-2
- ↑ 4.0 4.1 Hantschke M et al Cutaneous clear cell sarcoma: A clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. Am J Surg Pathol 2010 Feb; 34:216 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20087159