Ovary tumor
Sex cord stromal tumors
Gonadoblastoma

Authors: Shahrzad Ehdaivand, M.D., Nalini Gupta, M.D. (see Authors page)

Revised: 7 June 2016, last major update December 2012

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PubMed search: Ovarian gonadoblastoma
Cite this page: Gonadoblastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorgonadoblastoma.html. Accessed December 6th, 2016.
Definition / General
  • First described by Scully in 1953 (Cancer 1953;6:455)
  • Also called dysgenetic gonadoma
  • Mixture of germ cell tumor and sex-cord stromal tumor
  • Usually occurs in individuals with abnormal sexual development and indeterminate gonads; usually gonadal dysgenesis with Y chromosome (i.e. XY gonadal dysgenesis, XO-XY mosaicism, not XX gonadal dysgenesis); 25% risk of neoplasia in these gonads
  • Also present in phenotypically normal women, even during pregnancy, although ovary is never normal
  • Y chromosome material appears to participate in gonadoblastoma tumorigenesis (Am J Clin Pathol 1997;108:197, Birth Defects Res C Embryo Today 2009;87:114)
  • Associated with ataxia-telangiectasia
  • 80% are phenotypic women, 20% are phenotypic men with undescended testicles and female internal secondary organs
  • 50% have coexisting dysgerminoma
  • Excellent prognosis if completely excised; almost never malignant
  • Chromosomal analysis useful to diagnose androgen insensitivity / male pseudohermaphroditism (46,XY) and Turner syndrome (45,XO)

  • Imaging:
    • Imaging studies useful to diagnose intersexuality and identify patients at risk of developing gonadoblastoma
    • Flat abdominal radiograph may reveal gonadal calcification, a classic pathologic finding in gonadoblastoma
Pathophysiology
  • Gonadal differentiation starts after 5 weeks of gestation and depends on sex chromosome of fetus
  • Errors in this complex multistep process of sexual differentiation may cause dysgenetic gonads
Case Reports
Treatment
  • Pure Gonadoblastoma have excellent prognosis
  • Local excision is adequate treatment if no frankly malignant are present
Gross Description
  • 36% bilateral, tumors usually small and may be microscopic
Gross Images

Images hosted on PathOut server:

Bilateral tumor, with germinoma

With germinoma



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Gonadal dysgenesis

Micro Description
  • Primitive germ cells and sex cord stromal cells surrounded by ovarian-type stroma
  • Nests of dysgerminoma-like germ cells and sex cord derivatives resemble immature Sertoli and granulosa cells
  • Arranged in nests surrounded by ovarian stroma containing Leydig or lutein-type cells
  • Hyalinization and calcification are common
  • May be dysgerminoma if overgrowth of this component (Am J Clin Pathol 1997;108:197)
Micro Images

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Nests of tumor

With germinoma

Small sex cord-type cells



Images hosted on other servers:

Hyalinized basement membrane

Heavily calcified area

Dual cell populations of germ cells

Abundant clear cytoplasm

Admixture of germ cells

Isolated nuclei under Feulgen stain

Morphology of case

Positive Stains
Differential Diagnosis
  • Incidental finding in ovaries of normal infants / children: associated with follicular cysts, microscopic foci resembling gonadoblastoma or sex cord tumor with annular tubules