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<p><i>SS18::POU5F1</i>-fused sarcomas are rare tumors that show an undifferentiated round cell morphology. We report for the first time a case of <i>SS18::POU5F1-</i>fused sarcoma occurring in the parotid gland, with a highly unusual biphasic morphology comprising of epithelioid/undifferentiated round cell component and a ganglioneuromatous component consistent with divergent differentiation. We illustrate the cytology, histomorphological, immunohistochemical profile of the tumor, and show that both components of the tumor share a common origin. We discuss the differential diagnoses of this tumor, discuss its possible histogenesis, and perform a broad review of the literature covering this uncommon tumor.</p>

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SS18::POU5F1-fused sarcoma of the parotid with divergent ganglioneuromatous differentiation—a novel manifestation of a rare tumor

  • 期刊论文

  • 发表日期：

    2025年5月8日

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  • Ming Liang Oon,
  • Bingcheng Wu,
  • Jaslyn Jie Lin Lee,
  • Charmaine Tan,
  • Ju Ee Seet,
  • Michael Michal,
  • Fredrik Petersson

摘要

SS18::POU5F1-fused sarcomas are rare tumors that show an undifferentiated round cell morphology. We report for the first time a case of SS18::POU5F1-fused sarcoma occurring in the parotid gland, with a highly unusual biphasic morphology comprising of epithelioid/undifferentiated round cell component and a ganglioneuromatous component consistent with divergent differentiation. We illustrate the cytology, histomorphological, immunohistochemical profile of the tumor, and show that both components of the tumor share a common origin. We discuss the differential diagnoses of this tumor, discuss its possible histogenesis, and perform a broad review of the literature covering this uncommon tumor.