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ovarian gonadoblastoma

Friday 18 February 2011

Definition: Gonadoblastomas are neoplasms of dysgenetic gonads which may undergo regression or become overgrown by malignant germ cell tumors (mGCTs).

It is the most frequent tumor found in gonadal dysgenesis , usually a phenotypic female with an XY karyotype and streak gonads.

Microscopy

Gonadoblastoma has ovarian stroma surrounding nests of germ cells and sex cord stromal cells, with frequent hyalinization and calcification. It is the most frequent tumor found in gonadal dysgenesis, usually a phenotypic female with an XY karyotype and streak gonads.

Morphologically, microfollicular pattern of gonadoblastomas shows a fetal germ cell organization reminiscent of oocytic clusters of fetal ovaries. (15968543)

Gonadoblastoma has ovarian stroma surrounding nests of germ cells and sex cord stromal cells, with frequent hyalinization and calcification.

IHC

They contained both cell types, similar to oocytes (M2A-, GCAP-, c-kit+/-, sACE-) and oogonia (M2A+, GCAP+, c-kit+, sACE+). (15968543)

The percentage of germ cells immunoreactive for oncofetal markers and the proliferation index increases from microfollicular over coronary patterns to adjacent dysgerminomas. (15968543)

Supportive cells of gonadoblastomas show a uniform phenotype (CK18+, vimentin+, sACE+, alpha-inhibin+, M2A-) but in contrast to fetal germ cells lacked a clear equivalence to fetal tissues. (15968543)

Gonadoblastomas mimic female fetal ovary and exhibit a stepwise progression from follicular pattern to coronary pattern and finally to dysgerminomas. (15968543)

Associations

- pure yolk sac tumor (pure endodermal sinus tumor) (18179152)

See also

- gonadoblastoma

  • testicular gonadoblastoma

Open access references

- Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Ulbright TM. Mod Pathol. 2005 Feb;18 Suppl 2:S61-79. PMID: 15761467

References

- Ovarian gonodoblastoma with yolk sac tumor in a young 46, XX female: case report. Erdemoglu E, Ozen S. Eur J Gynaecol Oncol. 2007;28(6):516-8. PMID: 18179152

- Gonadoblastoma: evidence for a stepwise progression to dysgerminoma in a dysgenetic ovary. Pauls K, Franke FE, Büttner R, Zhou H. Virchows Arch. 2005 Sep;447(3):603-9. PMID: 15968543