Superficial paramucosal clear cell sarcoma of the soft parts resembling melanoma in a 13-year-old boy

Michael Sidiropoulos*, Klaus Busam, Joan Guitart, William B. Laskin, Annette M. Wagner, Pedram Gerami

*Corresponding author for this work

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

Clear cell sarcoma (CCS) of tendons and aponeuroses, also known as melanoma of soft parts, represents an aggressive rare malignancy that is characterized by a nested or fascicular pattern of spindled cells and a pathognomonic reciprocal translocation, t(12;22)(q13;q12), that results in the fusion of EWSR1 and ATF1 genes. Numerous recent studies have recognized the importance of a cutaneous CCS variant that can mimic a broad spectrum of entities, including spindle cell melanoma, spindle cell squamous carcinoma, cutaneous leiomyosarcoma and atypical fibroxanthoma. We report a case of a 13-year-old boy with cutaneous CCS who presented with a few months history of an asymptomatic papule on the lower lip that was suggestive of a mucocele. Biopsy of the lesion showed a wedge shaped neoplasm arranged in nests and fascicles of epithelioid- to oval-shaped cells with pale cytoplasm, open chromatin and prominent nucleolus. The superficial component was closely opposed to the basal epithelium resembling the junctional nests of a melanocytic neoplasm. The process extended into and involved the striated muscle of the lip. The cells expressed S-100, CD99 and synaptophysin by immunohistochemistry, and there was focal HMB-45 and microphthalmia transcription factor (MiTF) positivity as well. Fluorescence in situ hybridization confirmed the presence of the t(12;22) (ESWR1-ATF1) translocation.

Original languageEnglish (US)
Pages (from-to)265-268
Number of pages4
JournalJournal of cutaneous pathology
Volume40
Issue number2
DOIs
StatePublished - Feb 1 2013

Keywords

  • FISH
  • clear cell sarcoma
  • melanoma
  • melanoma of soft parts

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

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