Predictive Patterns of Glutamine Synthetase Immunohistochemical Staining in CTNNB1-mutated Hepatocellular Adenomas

Am J Surg Pathol. 2021 Apr 1;45(4):477-487. doi: 10.1097/PAS.0000000000001675.

Abstract

Some hepatocellular adenoma (HCA) subtypes are characterized by different CTNNB1 mutations, leading to different beta-catenin activation levels, hence variable immunostaining patterns of glutamine synthetase (GS) expression, and different risks of malignant transformation. In a retrospective multicentric study of 63 resected inflammatory (n=33) and noninflammatory (n=30) molecularly confirmed CTNNB1-mutated b-(I)HCA, we investigated the predictive potential of 3 known GS patterns as markers for CTNNB1 exon 3, 7/8 mutations. Pattern 1 (diffuse homogenous) allowed recognition of 17/21 exon 3 non-S45 mutated b-(I)HCA. Pattern 2 (diffuse heterogenous) identified all b-(I)HCA harboring exon 3 S45 mutation (20/20). Pattern 3 (focal patchy) distinguished 12/22 b-(I)HCA with exon 7/8 mutations. In exon 3 S45 and 7/8 mutations, both b-HCA and b-IHCA showed a GS+/CD34- rim with diffuse CD34 positivity in the center of the lesion. Interobserver reproducibility was excellent for exon 3 mutations. Comparative analysis of GS patterns with molecular data showed 83% and 80% sensitivity (b-HCA/b-IHCA) and 100% specificity for exon 3 non-S45. For exon 3 S45, sensitivity was 100% for b-(I)HCA, and specificity 93% and 92% (b-HCA/b-IHCA). For exon 7/8, sensitivity was 55% for both subtypes and specificity 100% and 96% (b-HCA/b-IHCA). Preliminary data from 16 preoperative needle biopsies from the same patients suggest that this panel may also be applicable to small samples. In surgically resected HCA, 2 distinct GS patterns can reliably predict CTNNB1 exon 3 mutations, which are relevant because of the higher risk for malignant transformation. The third pattern, although specific, was less sensitive for the identification of exon 7/8 mutation, but the GS+/CD34- rim is a valuable aid to indicate either an exon 3 S45 or exon 7/8 mutation.

Publication types

  • Multicenter Study

MeSH terms

  • Adenoma, Liver Cell* / enzymology
  • Adenoma, Liver Cell* / genetics
  • Adenoma, Liver Cell* / pathology
  • Adolescent
  • Adult
  • Aged
  • Biomarkers, Tumor* / analysis
  • Biomarkers, Tumor* / genetics
  • Biopsy, Needle
  • DNA Mutational Analysis
  • Europe
  • Exons
  • Female
  • Glutamate-Ammonia Ligase / analysis*
  • Humans
  • Immunohistochemistry*
  • Liver Neoplasms* / enzymology
  • Liver Neoplasms* / genetics
  • Liver Neoplasms* / pathology
  • Male
  • Middle Aged
  • Mutation*
  • Predictive Value of Tests
  • Reproducibility of Results
  • Retrospective Studies
  • Young Adult
  • beta Catenin / genetics*

Substances

  • Biomarkers, Tumor
  • CTNNB1 protein, human
  • beta Catenin
  • GLUL protein, human
  • Glutamate-Ammonia Ligase