Abstract
Gonadoblastoma emerges as a benign gonadal neoplasm composed of germ cells and descendants of sex cord-stromal cells reminiscent of immature granulosa cells and Sertoli cells. Neoplasm is preponderantly associated with disorders of sexual development wherein majority (~80%) of neoplasms articulate phenotypic females. Tumefaction expounds primary amenorrhea and delayed sexual maturation with delayed development of genitalia and emergence of secondary sexual characters. Gonadoblastoma locus appears to be situated adjacent to Y centromere wherein TSPY gene is implicated in carcinogenesis. Commonly discerned distinct morphological configurations appear comprised of spherical or irregular clusters of immature Sertoli cells admixed with germ cells and circumscribing basement membrane. Intervening stroma exhibits enlarged, polygonal cells simulating Leydig cells admixed with focal calcification. Germ cells appear immune reactive to p53, placental alkaline phosphatase (PLAP), CD117/c-KIT and VASA protein or encoded testis specific Y protein. Testicular gonadoblastoma requires segregation from neoplasms such as Sertoli cell tumour. Genetic karyotyping is an essential investigation for distinguishing individuals with intersex disorders and potential occurrence of gonadoblastoma. Surgical extermination is a recommended mode of therapy for alleviating gonadoblastoma.
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