High-grade non-intestinal type sinonasal adenocarcinoma with ETV6::NTRK3 fusion, distinct from secretory carcinoma by immunoprofile and morphology

Virchows Arch. 2023 Aug;483(2):187-195. doi: 10.1007/s00428-023-03587-6. Epub 2023 Jul 6.

Abstract

We report 2 cases of high-grade sinonasal adenocarcinoma with a distinct morphological and immunohistochemical phenotype. Albeit histologically different from secretory carcinoma of the salivary glands, both tumors presented here share an ETV6::NTRK3 fusion. The highly cellular tumors were composed of solid and dense cribriform nests, often with comedo-like necroses in the center, and minor areas with papillary, microcystic, and trabecular formations without secretions, mostly located at the periphery of the lesion. The cells displayed high-grade features, with enlarged, crowded, and often vesicular nuclei with conspicuous nucleoli and brisk mitotic activity. The tumor cells were immunonegative for mammaglobin while showing immunopositivity for p40/p63, S100, SOX10, and GATA3, as well as for cytokeratins 7, 18, and 19. For the first time, we describe 2 cases of primary high-grade non-intestinal type adenocarcinomas of the nasal cavity, distinct from secretory carcinoma by morphology and immunoprofile, harboring the ETV6::NTRK3 fusion.

Keywords: ETV6::NTRK3; Nasal cavity; Salivary duct carcinoma; Secretory carcinoma; Sinonasal; non-intestinal-type adenocarcinoma.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma* / genetics
  • Adenocarcinoma* / pathology
  • Biomarkers, Tumor / genetics
  • Carcinoma* / genetics
  • Carcinoma* / pathology
  • ETS Translocation Variant 6 Protein
  • Humans
  • Immunohistochemistry
  • Oncogene Proteins, Fusion / genetics
  • Salivary Gland Neoplasms* / genetics
  • Salivary Gland Neoplasms* / pathology

Substances

  • Biomarkers, Tumor
  • Oncogene Proteins, Fusion
  • NTRK3 protein, human

Supplementary concepts

  • Secretory breast carcinoma