Gonadoblastoma: Case report of two young patients with isochromosome 12p found in the dysgerminoma overgrowth component in one case

Pathol Res Pract. 2012 Oct 15;208(10):628-32. doi: 10.1016/j.prp.2012.07.006. Epub 2012 Aug 18.

Abstract

Gonadoblastomas are unusual neoplasias that frequently appear in the dysgenetic gonads of women with chromosome Y anomaly. We present two cases of gonadoblastoma associated with complete gonadal dysgenesis and Turner syndrome, respectively, with dysgerminoma overgrowth found in one case. We were interested in the DNA ploidy, the presence of Y chromosome DNA sequence and the status of chromosome 12p arm among the tumor cells. We performed cytophotometry to analyze the DNA content and fluorescence in situ hybridization (FISH) to identify the Y chromosome and the isochromosome 12p within the tumor cells. The cytophotometric result showed diploid DNA content in gonadoblastoma, whereas dysgerminoma revealed aneuploid DNA. The FISH result revealed Y chromosome DNA sequence within gonadoblastoma and dysgerminoma. Isochromosome 12p was identified in dysgerminoma, but not in gonadoblastoma. We conclude that gonadoblastoma and dysgerminoma have a strong association with the Y chromosome, and dysgerminoma overgrowth is due to further chromosomal aberrations, such as isochromosome 12p. Histological, immunohistocheimcal and molecular studies should render the correct diagnosis. Identifying dysgerminoma overgrowth is crucial since it is associated with adverse prognosis and requires additional therapy.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Chromosome Disorders / genetics*
  • Chromosome Disorders / pathology
  • Chromosome Disorders / therapy
  • Chromosomes, Human, Pair 12 / genetics
  • Chromosomes, Human, Y
  • Cytophotometry
  • Dysgerminoma / genetics*
  • Dysgerminoma / pathology
  • Dysgerminoma / therapy
  • Female
  • Genetic Predisposition to Disease
  • Genetic Testing
  • Gonadal Dysgenesis, 46,XY / genetics*
  • Gonadal Dysgenesis, 46,XY / pathology
  • Gonadal Dysgenesis, 46,XY / therapy
  • Gonadoblastoma / genetics*
  • Gonadoblastoma / pathology
  • Gonadoblastoma / therapy
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Ovarian Neoplasms / genetics*
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / therapy
  • Phenotype
  • Ploidies
  • Predictive Value of Tests
  • Turner Syndrome / genetics*
  • Turner Syndrome / pathology
  • Turner Syndrome / therapy

Supplementary concepts

  • Pallister Killian syndrome