A novel STRN3::PRKD1 fusion in a cribriform adenocarcinoma of salivary gland with high-grade transformation

Genes Chromosomes Cancer. 2023 Oct;62(10):624-628. doi: 10.1002/gcc.23181. Epub 2023 Jun 6.

Abstract

Cribriform adenocarcinoma of salivary gland (CASG) is a rare form of salivary gland neoplasm that mostly arises from minor salivary glands. We report a case of CASG with high-grade transformation harboring a novel STRN3::PRKD1 fusion. A 59-year-old male presented with a palatal mass. Morphologically, the tumor consisted of two components: solid high-grade and glandular low-grade areas. The solid high-grade area comprised solid nests of high-grade carcinoma with central necrosis arranged in lobules delineated with prominent stromal septa. The glandular low-grade area comprised of cribriform and microcystic architecture in a hyalinized and hypocellular stroma. Immunophenotypically, the tumor was positive for S100 but negative for p40 and actin. However, due to the high-grade component, tissue was sent for salivary gland NGS fusion panel analysis to confirm the diagnosis. The current case illustrates high-grade transformation in CASG. Furthermore, identification of a STRN3::PRKD1 fusion expands the genetic spectrum of CASG.

Keywords: PRKD1 rearrangement; STRN3 rearrangement; high-grade transformation; polymorphous adenocarcinoma; salivary gland tumor.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma* / pathology
  • Autoantigens
  • Biomarkers, Tumor / genetics
  • Calmodulin-Binding Proteins
  • Humans
  • Male
  • Middle Aged
  • Salivary Gland Neoplasms* / genetics
  • Salivary Gland Neoplasms* / pathology
  • Salivary Glands

Substances

  • Biomarkers, Tumor
  • STRN3 protein, human
  • Autoantigens
  • Calmodulin-Binding Proteins