Malignant peripheral nerve sheath tumor with a t(X;18): A synovial sarcoma variant?

Russell Vang, David A. Biddle, Wilbur R. Harrison, Kent Heck, Linda D. Cooley

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Abstract

We describe an ankle tumor arising in a 16-year-old girl. The tumor demonstrated histology typical of a malignant peripheral nerve sheath tumor (MPNST), but exhibited a variant form of the (X;18) translocation associated with synovial sarcoma. Immunohistochemical stains were positive for vimentin, CD57, collagen type IV, and Bcl-2. Routine and molecular cytogenetic studies showed an unbalanced 3-way chromosomal translocation that involved chromosomes X, 18, and 1. Electron microscopic findings were noncontributory. This unusual tumor raises the following questions and possibilities: (1) As the t(X;18) suggests, could this tumor be a monophasic synovial sarcoma with the histologic features of an MPNST? (2) Or, as the histology suggests, is this tumor an MPNST that has a t(X;18)? (3) Finally, could MPNST histology, a t(X;18), and no defining immunohistochemical or electron microscopic features represent an as yet unrecognized part of a spectrum that spans from synovial sarcoma to MPNST or other spindle cell tumors?

Original languageEnglish (US)
Pages (from-to)864-867
Number of pages4
JournalArchives of Pathology and Laboratory Medicine
Volume124
Issue number6
StatePublished - Jul 3 2000

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ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

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