Recurrent MDM2 Amplification in the Spectrum of HMGA2-Altered Pleomorphic Adenoma, Atypical Pleomorphic Adenoma and Carcinoma Ex Pleomorphic Adenoma

Head Neck Pathol. 2025 May 8;19(1):56. doi: 10.1007/s12105-025-01794-y.

Abstract

Introduction: Pleomorphic adenoma is the most common neoplasm of the salivary glands. While the overall risk of malignancy is relatively low, a distinct molecular sub-group harboring HMGA2 alterations seems to show an increased risk of malignant progression to carcinoma ex pleomorphic adenoma.

Purpose: This study investigates MDM2 amplification in HMGA2-altered pleomorphic adenoma, atypical pleomorphic adenoma, and carcinoma ex pleomorphic adenoma.

Methods: In this multicenter, retrospective case series analysis, we examined 37 cases of HMGA2-altered pleomorphic adenoma, carcinoma ex pleomorphic adenoma, and pleomorphic adenoma with atypical features. A total of 18 cases were included from our institutional archives, with 19 additional cases derived from published literature. The cases from our institutes were analyzed for MDM2 amplification using a stepped approach by immunohistochemistry and FISH.

Results: Collectively, an MDM2 amplification was present in 27% of pleomorphic adenoma (4 of 15), compared to 78% of carcinoma ex pleomorphic adenoma (14 of 18) (p-value = 0.003). In the group of pleomorphic adenomas with atypical features, an MDM2 amplification was present in 50% of cases (2 of 4). These findings indicate an association between MDM2 amplification and malignancy. Strikingly, a mixed control group of 12 benign and malignant PLAG1-altered neoplasms showed no immunohistochemical staining for MDM2.

Conclusion: Immunohistochemical MDM2 expression, including MDM2 amplification, is enriched in the group of HMGA2-altered pleomorphic adenoma, and potentially plays role in malignant progression. This study highlights the importance of recognizing the molecular sub-group of HMGA2-altered pleomorphic adenomas and integrate MDM2 analysis into routine diagnostics to corroborate the cytonuclear atypia in these challenging cases.

Keywords: Malignant transformation; Molecular analysis; Pleomorphic adenoma; Salivary gland neoplasm.

Publication types

  • Multicenter Study

MeSH terms

  • Adenoma, Pleomorphic* / genetics
  • Adenoma, Pleomorphic* / pathology
  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • Female
  • Gene Amplification
  • HMGA2 Protein* / genetics
  • Humans
  • Male
  • Middle Aged
  • Proto-Oncogene Proteins c-mdm2* / genetics
  • Retrospective Studies
  • Salivary Gland Neoplasms* / genetics
  • Salivary Gland Neoplasms* / pathology

Substances

  • Proto-Oncogene Proteins c-mdm2
  • HMGA2 Protein
  • HMGA2 protein, human
  • MDM2 protein, human
  • Biomarkers, Tumor