A current perspective on the pathological assessment of FOXL2 in adult-type granulosa cell tumours of the ovary

Histopathology. 2014 Feb;64(3):380-8. doi: 10.1111/his.12253. Epub 2013 Nov 6.

Abstract

Aims: The diagnosis of adult-type granulosa cell tumours of the ovary (aGCT) is based on histomorphology aided by immunohistochemical staining for sex cord markers. Recently a single, recurrent somatic point mutation (402C→G) in FOXL2 was described in aGCT. We have investigated the impact of FOXL2 mutation testing in a large cohort of aGCT diagnosed previously by conventional histology and immunohistochemistry.

Methods and results: Formalin-fixed, paraffin-embedded tissue cores from a cohort of 52 aGCT diagnosed previously by expert gynaecopathologists were analysed immunohistologically. FOXL2 mutation status was determined by Sanger sequencing and high-sensitivity TaqMan allelic discrimination assay. Histomorphology was reassessed by two expert gynaecopathologists. FOXL2 mutation analyses could be performed successfully in 46 cases, 40 of which were positive for the c.402C>G mutation, confirming a diagnosis of aGCT. In the six cases negative for the c.402C>G mutation, one case was confirmed on review as FOXL2 wild-type aGCT, whereas in the remaining five cases diagnoses other than aGCT were made.

Conclusion: In cases where a diagnosis of aGCT is a consideration and unequivocal diagnosis is not possible based on morphology and routine immunostains, FOXL2 mutation testing can help to confirm the diagnosis. It is particularly relevant for accurate subclassification within the group of sex cord-stromal tumours.

Keywords: FOXL2 mutation; adult-type granulosa cell tumour; immunohistochemistry; molecular diagnostics; ovary.

MeSH terms

  • Adult
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Cohort Studies
  • DNA Mutational Analysis
  • DNA, Neoplasm / genetics
  • Female
  • Forkhead Box Protein L2
  • Forkhead Transcription Factors / genetics*
  • Forkhead Transcription Factors / metabolism
  • Granulosa Cell Tumor / genetics*
  • Granulosa Cell Tumor / metabolism
  • Granulosa Cell Tumor / pathology*
  • Humans
  • Immunohistochemistry
  • Mutant Proteins / genetics*
  • Mutant Proteins / metabolism
  • Ovarian Neoplasms / genetics*
  • Ovarian Neoplasms / metabolism
  • Ovarian Neoplasms / pathology*
  • Point Mutation
  • Retrospective Studies

Substances

  • Biomarkers, Tumor
  • DNA, Neoplasm
  • FOXL2 protein, human
  • Forkhead Box Protein L2
  • Forkhead Transcription Factors
  • Mutant Proteins