Clear cell sarcoma of soft tissue with eccrine differentiation: A case report and review of the literature. (14th March 2021)

• Record Type: Journal Article
• Authors: Sezer, Engin; Çeliker, Pelin; Yalçın, Özben; Erdem, Zeynep Betül
• Is Part Of: Journal of cutaneous pathology. Volume 48:Number 8(2021)
• Page Start: 1034
• Page End: 1037
• Publication Date: 2021-03-14
• Subjects: clear cell sarcoma of soft tissue -- dermatopathology -- ductal differentiation; Skin -- Diseases -- Periodicals; Dermatology -- Periodicals; 616
• DOI: 10.1111/cup.13989
• Languages: English
• ISSNs: 0303-6987

Abstract

Abstract: Clear cell sarcoma of soft tissue (CCSST) is a deep soft tissue tumor presenting in the extremities of young adults. Histopathologically, nests and sheets of polygonal cells with clear to eosinophilic cytoplasm separated by fibrous septa as well as occasional "wreath‐like" giant cells are visualized. However, CCSST has been noted to have atypical histopathological features, such as epidermotropism or myxoid differentiation, or occurrence at unusual sites. Here, we present a case of eccrine ductal differentiation in CCSST. The patient, a 21‐year‐old woman, presented with a lump of 10‐year duration sized 3 × 5 cm on the plantar surface of the fourth and fifth interdigital spaces. There had been an increase in size as well as pain and redness over 6 years. Besides the characteristic findings, there were ductal structures in continuity with the upper dermis indicative of ductal differentiation. The tumor stained positively for S100, HMB45, and succinic dehydrogenase; ducts stained positively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). CCSST was confirmed with cytogenetic analysis showing the translocation associated with EWSR1‐ATF1 fusion gene. Therefore, ductal differentiation is a unique finding that should be considered when evaluating for CCSST.