gonadoblastoma


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go·nad·o·blas·to·ma

(gō-nad'ō-blas-tō'ma), [MIM*424500]
Benign neoplasm composed of germ cells, sex cord derivatives, stromal cells; appears in cases of mixed or pure gonadal dysgenesis; usually small (1-3 cm) and partially calcified, but may give rise to malignant germ-cell tumors, most often seminoma/dysgerminoma or embryonal.

gonadoblastoma

[gō′nədōblastō′mə]
Etymology: Gk, gone, seed + blastos, germ + -oma, tumor
a rare benign type of germ cell tumor, usually occurring in patients with gonadal dysgenesis, and often bilateral. It contains all gonadal elements and is frequently associated with an abnormal chromosomal karyotype. It may give rise to a dysgerminoma or other more malignant germ cell tumor. See also dysgerminoma, gonadal dysgenesis.

gonadoblastoma

Gynecology Dysgenetic gonadoma A mixed germ cell-sex cord-stromal tumor which is most commonly seen in sexually abnormal persons, especially with gonadal dysgenesis–GD with a Y chromosome–ie, XY GD and XO-XY mosaicism; gonadoblastomas carry a 25% risk of neoplasia. See Sex-cord tumor Urology A mixed germ cell-sex cord-stromal tumor which almost invariably arises in a background of a gonadal disorder, either pure or mixed gonadal dysgenesis, male pseudohermaphroditism or, less commonly, in a phenotypically or karyotypically normal male. See Sex cord-stromal tumor.

go·nad·o·blas·to·ma

(gō-nad'ō-blas-tō'ma)
Rare, benign neoplasm composed of germ cells, sex cord derivatives, and stromal cells; appears in cases of mixed or pure gonadal dysgenesis; usually small (1-3 cm) and partially calcified but may give rise to malignant germ-cell tumors, most often seminoma and dysgerminoma or embryonal; usually bilateral; found in patients with gonadal dysgenesis.

gonadoblastoma

a rare, primary tumor of testicle or ovary containing, besides interstitial cells, germ cells, epithelial cells and granulosa cells.
References in periodicals archive ?
Sindrome de Frasier: caracterizado por neuropatia progresiva, infantilismo sexual, falla renal y gonadas rudimentarias en las cuales se puede desarrollar gonadoblastomas.
In these cases, the precursor lesion would be a gonadoblastoma.
Gonadoblastoma progressing to dysgerminoma in a 55-year-old woman with normal karyotype.
The chromosome Y-linked testis-specific protein locus TSPY1 is characteristically present in gonadoblastoma.
106) For patients with Y mosaicism, a 43% risk of developing gonadoblastoma is reported.
Gonadoblastoma is part of the mixed germ cell sex cord-stromal tumor group.
The macroscopic appearance of gonadoblastoma varies depending on size, amount of calcification, and presence or absence of a malignant germ cell-tumor component.
Calcification and hyalinization can ultimately lead to changes referred to as "burnt-out" gonadoblastoma in which tumor cells can be sparse or even completely absent, and the pattern of calcification or the presence of Leydig-like cells may be the only evidence of a gonadoblastoma.
Several scrotal masses, clinically present with calcifications, include mature teratomas, gonadoblastomas, calcifying clear cell Sertoli tumours, testicular microlithiasis, testicular torsion followed by hemorrhagic infarction, and metastatic neuroblastomas.
Few authors apply the term to patients who show testicular differentiation on either side, bilateral streak testis or bilateral dysgenetic testis or gonadoblastomas on other side.
Latter identification is must since it influences the development of gonadoblastomas in later age.
CONCLUSION: Asymmetric gonadal dysgenesis is rare phenomenon and it is important to diagnose this entity in early age because of proper gender assignment and 30% of these cases progress to Gonadoblastomas.