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.

Bioptic Laboratory Ltd

Department of Molecular Pathology Bioptic Laboratory Ltd Plzen Czech Republic

Department of Otolaryngology Head and Neck Surgery Rigshospitalet Denmark

Department of Otorhinolaryngology and Maxillofacial Surgery Køge University Hospital

Departments of Pathology

Institute of Pathology Friedrich Alexander University Erlangen Germany

Oncology and Radiotherapy Oncological Clinic Faculty of Medicine in Plzen Charles University

Otorhinolaryngology

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

(Mammary Analogue) Secretory Carcinoma of the Nasal Cavity: Report of a Rare Case with p63 and DOG1 Expression and Uncommon Exon 4-Exon 14 ETV6-NTRK3 Fusion Diagnosed with Next Generation Sequencing