Abstract
A chance finding of structures resembling gonadoblastomas in the ovaries of a child with lissencephaly prompted a detailed review of all ovarian histology obtained at autopsy over a 12 month period. Fifty-five stillbirths, infants and children were studied ranging from 20 weeks gestational age to 2.5 years post-natal age. In 19 infants structures mimicking gonadoblastomas and sex cord tumours with annular tubules were seen. In all but one case these structures were found in association with follicular cysts and they closely resembled the atretic follicles often seen in the stroma surrounding the follicular cysts. They differed from the atretic follicles only by virtue of their being larger. In addition, in several infants structures resembling Sertoli cell tubules or clusters of Leydig cells were found. When present, these structures always co-existed with sex cord tumours with annular tubules and gonadoblastoma-like lesions. The abnormal stromal lesions and follicular cysts were found most frequently at the stage of development when a massive ‘physiological’reduction of oocytes occurs. It is suggested that the ‘abnormal’structures identified in this report represent the ‘first hit’of oncogenesis and could serve as the precursor of many of the sex cord-stromal tumours, and possibly germ cell neoplasms, seen in childhood.

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