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clear cell sarcoma of tendons & aponeuroses (malignant melanoma of soft parts)
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:
- synovial sarcoma
- fibrosarcoma
- epithelioid MPNST
- spindle cell melanoma
Management:
- surgical resection
- multiple recurrences
- prognosis
- late metastasis
- death likely
Interactions
disease interactions
General
clear cell sarcoma
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
- 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: 20087159