A rare case of vulvar extraskeletal myxoid chondrosarcoma: Mimics and diagnostic clues

Sofia S. Liou, Sanaz Memarzadeh, Sarah M. Dry, Rondell P. Graham, Neda Aliyari Moatamed

Research output: Contribution to journalArticlepeer-review

Abstract

Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.

Original languageEnglish (US)
Article numbere2021322
JournalAutopsy and Case Reports
Volume11
DOIs
StatePublished - 2021

Keywords

  • Chondrosarcoma, Extraskeletal Myxoid
  • EWSR1
  • FISH
  • NR4A3
  • Vulva

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Internal Medicine

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