Mammary Analog Secretory Carcinoma of the Nasal Cavity: Characterization of 2 Cases and Their Distinction From Other Low-grade Sinonasal Adenocarcinomas

Am J Surg Pathol. 2018 Jun;42(6):735-743. doi: 10.1097/PAS.0000000000001048.

Abstract

Secretory carcinoma, originally described as mammary analog secretory carcinoma (MASC), is a low-grade salivary gland tumor characterized by a t(12;15)(p13;q25) translocation, resulting in an ETV6-NTRK3 gene fusion. Most MASCs are localized to the parotid gland and intraoral minor salivary glands. Moreover, ETV6-rearranged carcinomas with secretory features have been reported recently in the thyroid (with and without a history of radiation exposure), skin, and in very rare instances in the sinonasal tract. Here, we describe 2 cases of primary MASC in the sinonasal tract and provide a detailed clinical and histopathologic characterization of their morphology, immunohistochemical profile, and genetic background and highlight features allowing for its separation from its recently described molecular mimicker, ETV6-rearranged low-grade sinonasal adenocarcinoma.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenocarcinoma / chemistry
  • Adenocarcinoma / genetics
  • Adenocarcinoma / pathology*
  • Aged
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • Biopsy
  • Diagnosis, Differential
  • Female
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Mammary Analogue Secretory Carcinoma / chemistry
  • Mammary Analogue Secretory Carcinoma / genetics
  • Mammary Analogue Secretory Carcinoma / pathology*
  • Mammary Analogue Secretory Carcinoma / surgery
  • Middle Aged
  • Nasal Cavity / chemistry
  • Nasal Cavity / pathology*
  • Nasal Cavity / surgery
  • Neoplasm Grading
  • Nose Neoplasms / chemistry
  • Nose Neoplasms / genetics
  • Nose Neoplasms / pathology*
  • Nose Neoplasms / surgery
  • Oncogene Proteins, Fusion / genetics
  • Predictive Value of Tests
  • Registries
  • Reverse Transcriptase Polymerase Chain Reaction
  • Tomography, X-Ray Computed

Substances

  • Biomarkers, Tumor
  • ETV6-NTRK3 fusion protein, human
  • Oncogene Proteins, Fusion