HMGA2 is a specific immunohistochemical marker for pleomorphic adenoma and carcinoma ex-pleomorphic adenoma

Histopathology. 2017 Oct;71(4):511-521. doi: 10.1111/his.13246. Epub 2017 Jul 5.

Abstract

Aims: Accurate classification of salivary gland neoplasms may be challenging, owing to morphological overlap, particularly in small biopsies. Recurrent translocations involving the high-mobility group AT-hook 2 (HMGA2) gene are present in a subset of pleomorphic adenomas (PAs) and carcinoma ex-pleomorphic adenomas (CA ex-PAs). The aim of this study was to evaluate immunohistochemical HMGA2 expression in 225 salivary gland tumours, including 56 PAs, 37 CA ex-PAs, and 132 potential histological mimics, to determine its diagnostic utility.

Methods and results: HMGA2 expression was identified in 19 PAs (33.9%) and nine CA ex-PAs (24.3%). Expression was strong and diffuse throughout all PAs, and in four of nine positive CA ex-PAs. In five CA ex-PAs, HMGA2 showed weak-to-strong multifocal staining within the carcinomatous component, and strong diffuse HMGA2 expression in the residual PA. Among histological mimics, six de-novo salivary duct carcinomas (28.5%), three epithelial-myoepithelial carcinomas (33.3%) and one case each of myoepithelioma and basal cell adenoma expressed HMGA2. Fluorescence in-situ hybridization for HMGA2 rearrangement performed on a subset of tumours that showed diffuse HMGA2 expression in PAs and CA ex-PAs was frequently associated with rearrangement of the HMGA2 locus, whereas cases of de-novo salivary duct carcinoma, or CA ex-PA with limited or no HMGA2 expression, had an intact HMGA2 locus.

Conclusions: HMGA2 expression is a highly specific (96.2%), but low-sensitivity (29.8%), marker for PA and CA ex-PA when compared with histological mimics, and is frequently associated with rearrangement of the HMGA2 locus.

Keywords: HMGA2; carcinoma ex-pleomorphic adenoma; immunohistochemistry; pleomorphic adenoma; salivary gland.

MeSH terms

  • Adenoma, Pleomorphic / metabolism*
  • Adenoma, Pleomorphic / pathology
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism*
  • HMGA2 Protein / genetics
  • HMGA2 Protein / metabolism*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Salivary Gland Neoplasms / metabolism*
  • Salivary Gland Neoplasms / pathology
  • Salivary Glands / metabolism
  • Salivary Glands / pathology
  • Translocation, Genetic

Substances

  • Biomarkers, Tumor
  • HMGA2 Protein