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Purpose <p>The <i>NAB2::STAT6</i> 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 <i>NAB2::STAT6</i> rearranged epithelial tumor exhibiting sebaceous differentiation in the parotid gland.</p> Methods <p>Fine-needle aspiration (FNA), histopathology, immunohistochemistry, and molecular studies of this case were described.</p> Results <p>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 <i>NAB2::STAT6</i> fusion, along with an AKT1 mutation.</p> Conclusion <p>This case further expands the phenotypic spectrum of <i>NAB2::STAT6</i> 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.</p>

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

  • 期刊论文

  • 发表日期：

    2025年1月13日

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  • Emma J. de Ruiter,
  • Johannes A. Rijken,
  • Thom Doeleman,
  • Lennart A. Kester,
  • Frank A. Pameijer,
  • Mihaela G. Raicu,
  • Gerben E. Breimer

摘要

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.