- Gonadoblastoma
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MeshID = D018238
A gonadoblastoma is a complexneoplasm composed of a mixture ofgonad al elements, such as large primordialgerm cell s, immatureSertoli cell s orgranulosa cell s of thesex cord , and gonadalstromal cell s.Associations
Gonadoblastoma is most often associated with abnormal chromosomal
karyotype , gonadaldysgenesis , and the presence of aY chromosome in over 90% of cases.It has been found in association with
Turner's syndrome .cite journal |author=Maggio MC, Liotta A, De Grazia E, Cimador M, Di Pace R, Corsello G |title=Polycystic ovary and gonadoblastoma in Turner's syndrome |journal=Minerva Pediatr. |volume=59 |issue=4 |pages=397–401 |year=2007 |pmid=17947845 |doi=] cite journal |author=Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi IT |title=Detection of hidden Y mosaicism in Turner's syndrome: importance in the prevention of gonadoblastoma |journal=J. Pediatr. Endocrinol. Metab. |volume=19 |issue=9 |pages=1113–7 |year=2006 |pmid=17128558 |doi=]Classification
Gonadoblastoma is classified as a
germ cell tumor .Fact|date=September 2007Formerly, gonadoblastoma was sometimes regarded as a subset of
dysgerminoma . In modern literature, it is sometimes considered to progress to dysgerminoma.cite journal |author=Cooper C, Cooper M, Carter J, Russell P |title=Gonadoblastoma progressing to dysgerminoma in a 55-year-old woman with normal karyotype |journal=Pathology |volume=39 |issue=2 |pages=284–5 |year=2007 |pmid=17454768 |doi=10.1080/00313020701230708]Treatment
Standard treatment would include surgical exploration via
laparotomy .Laparoscopy may be an option if the surgeon is particularly skilled in removing ovarian neoplasms via laparoscopy intact. If the diagnosis of gonadoblastoma is certain, a bilateral salpingo-oopherectomy (BSO) should be performed to remove both the primary tumor and the dysgenic contralateral ovary. If uninvolved, theuterus should be left intact. Modern reproductive endocrinology technology allows patients post BSO to achieve pregnancy via in-vitro fertilization (IVF ) with a donor egg.References
External links
* [http://www.health.am/cr/testis-non-germ-cell-tumors/ www.health.am: Gonadoblastoma]
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