A Pediatric Case of High-Grade Secretory Carcinoma of the Maxillary Sinus With ETV6::NTRK3 Gene Fusion, Therapeutic Implications, and Review of the Literature

Pediatr Dev Pathol. 2023 Jan-Feb;26(1):59-64. doi: 10.1177/10935266221138706. Epub 2022 Nov 30.


Secretory carcinoma (SC) is a salivary gland tumor with a generally low grade microscopic appearance, a characteristic immunophenotype, and a recurrent translocation leading to ETV6::NTRK3 fusion gene. Rare cases are reported in children. The maxillary sinus is an unusual localization. SC have an overall favorable prognosis, but cases with high grade morphology have been described in adult population and are related to a more aggressive clinical course. We present a pediatric case of secretory carcinoma involving the maxillary sinus with high grade morphology, with a review of the literature of secretory carcinomas with high grade component.

Keywords: pediatric secretory carcinoma high grade.

Publication types

  • Review

MeSH terms

  • Adult
  • Biomarkers, Tumor / genetics
  • Carcinoma* / pathology
  • Child
  • Gene Fusion
  • Humans
  • Maxillary Sinus* / pathology
  • Oncogene Proteins, Fusion / genetics


  • Oncogene Proteins, Fusion
  • Biomarkers, Tumor

Supplementary concepts

  • Secretory breast carcinoma