NAB2::STAT6 Rearranged Carcinoma of the Parotid Gland with Sebaceous Differentiation: A Case Report

Head Neck Pathol. 2025 Jan 13;19(1):9. doi: 10.1007/s12105-024-01733-3.

Abstract

Purpose: The NAB2::STAT6 fusion is predominantly associated with solitary fibrous tumors (SFTs) and is utilized in diagnosing SFTs through nuclear STAT6 protein overexpression. Recent studies expanded the phenotypic spectrum of NAB2::STAT6 rearranged neoplasms, including adamantinoma-like and teratocarcinosarcoma-like phenotypes. We report a case of a NAB2::STAT6 rearranged epithelial tumor exhibiting sebaceous differentiation in the parotid gland.

Methods: Fine-needle aspiration (FNA), histopathology, immunohistochemistry, and molecular studies of this case were described.

Results: Fine-needle aspiration (FNA) revealed atypical basaloid cells, suggesting primary salivary gland carcinoma or metastasis. Histological examination showed basaloid-squamous cells with a monomorphic appearance containing foci of sebaceous differentiation, expressing pancytokeratin, p40, and androgen receptor, while CD34 staining was negative. Molecular studies identified a NAB2::STAT6 fusion, along with an AKT1 mutation.

Conclusion: This case further expands the phenotypic spectrum of NAB2::STAT6 rearranged neoplasms and emphasizes comprehensive histopathological and molecular analysis in challenging head and neck tumors. It suggests STAT6 immunohistochemistry as a potential screening tool for head and neck tumors resembling sebaceous carcinoma, myoepithelial tumors, or GLI1-altered neoplasms.

Keywords: Molecular diagnostics; NAB2::STAT6; STAT6; Salivary gland; Solitary fibrous tumor.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • Carcinoma / genetics
  • Carcinoma / pathology
  • Female
  • Gene Rearrangement
  • Humans
  • Male
  • Middle Aged
  • Parotid Neoplasms* / genetics
  • Parotid Neoplasms* / pathology
  • Repressor Proteins* / genetics
  • STAT6 Transcription Factor* / genetics

Substances

  • STAT6 Transcription Factor
  • STAT6 protein, human
  • Repressor Proteins
  • NAB2 protein, human
  • Biomarkers, Tumor