Gonadoblastoma overview
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Gonadoblastoma Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
Historical Perspective
Gonadoblastoma was first discovered by Dr. Scully in 1953. The association between GBY gene (GonadoBlastoma on the Y chromosome) and gonadoblastoma was made in 1986. In 1995, Tsuchiya found that the GBY gene located near the centromere of Y chromosome and contains multiple genes including Testis-specific protein Y-encoded (TSPY) gene.
Classification
Gonadoblastoma may be classified according to pathological appearance into three sub-types including classical, dissecting, and burnt-out.
Pathophysiology
The exact pathogenesis of gonadoblastoma is not fully understood. Gonadal development starts at 5 weeks of gestation and continues according to sex chromosomes. Any defects in this complicated process leads to defective gonadal development and gonadal dysgenesis and subsequently, it may be converted to gonadoblastoma in 20% to 30% of the cases.
Causes
There are no established causes for gonadoblastoma. However, there are certain risk factors that predispose to increased risk of gonadoblastoma.
Differentiating Xyz from Other Diseases
Gonadoblastoma must be differentiated from other diseases that cause virilization, and primary amenorrhea and also must be differentiated pathologically from dysgerminoma, Sex-cord stromal tumors, and Sertoli-cell nodules.