Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature

Shenon Sethi, Brittany Cody, Nada A. Farhat, Mark D. Pool, Nora Katabi

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2 Scopus citations


Biphenotypic sinonasal sarcoma (BSNS) is a rare recently described distinct spindle cell sarcoma which arises exclusively in the sinonasal region and is characterized by concomitant neural and myogenic differentiation. Before this neoplasm was characterized, most were classified as other entities including adult fibrosarcoma, monophasic synovial sarcoma and malignant peripheral nerve sheath tumor. By immunohistochemistry, these tumors characteristically express S100 and smooth muscle actin (SMA) and/or muscle specific actin (MSA). Most cases harbor rearrangements of PAX3 (paired box gene 3), and the most frequent translocation partner is MAML3 (mastermind like transcriptional coactivator 3). Herein, we described three cases of BSNS involving the nasal cavity with or without paranasal sinus involvement. We also did a literature review of the clinical features, histologic and immunophenotypic findings, cytogenetics, pathogenesis and behavior of this rare entity.

Original languageEnglish
Article number200491
JournalHuman Pathology: Case Reports
StatePublished - Jun 2021
Externally publishedYes


  • Head and neck sarcoma
  • MAML3
  • PAX3
  • Sinonasal sarcoma
  • Spindle cell neoplasm


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