Salivary secretory carcinoma with a novel ETV6-MET fusion

Lisa M. Rooper, Theodoros Karantanos, Yi Ning, Justin A. Bishop, Sarah W. Gordon, Hyunseok Kang

Research output: Contribution to journalArticle

17 Scopus citations

Abstract

Secretory carcinoma of the salivary glands, also known as mammary analogue secretory carcinoma, is a recently described tumor characterized by generally indolent clinical behavior and recurrent ETV6-NTRK3 fusions. However, a small subset of recent cases with high-grade histology, aggressive behavior, or alternate molecular findings are expanding the spectrum of this entity. In this case, a 59-year-old female presented with an infiltrative submandibular gland tumor that was originally classified as a high-grade acinic cell carcinoma, papillary-cystic variant. She developed persistent local disease and, 11 years after initial presentation, was found to have widespread metastases. Rereview of her primary tumor highlighted microcystic, papillary, and solid architecture, eosinophilic cytoplasm, vesicular nuclei with prominent nucleoli, abundant mitotic figures, and necrosis. Immunostains showed the tumor cells to be positive for S100 and mammaglobin and negative for DOG-1, and fluorescence in situ hybridization highlighted an ETV6 rearrangement, supporting a diagnosis of high-grade secretory carcinoma. Finally, next-generation sequencing demonstrated a novel ETV6-MET fusion. To our knowledge, this is the first ETV6-MET fusion reported in secretory carcinoma. This finding further expands the definition of secretory carcinoma while carrying implications for selecting appropriate targeted therapy.

Original languageEnglish (US)
Pages (from-to)1121-1126
Number of pages6
JournalAmerican Journal of Surgical Pathology
Volume42
Issue number8
DOIs
StatePublished - Aug 1 2018

Keywords

  • cabozantinib
  • ETV6
  • mammary analogue secretory carcinoma
  • MET
  • salivary gland carcinoma
  • secretory carcinoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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