NCIt synonyms : Juvenile Type Granulosa Cell Neoplasm;
NCIt related terms : Granulosa cell tumor, juvenile; Juvenile Type Granulosa Cell Tumor;
NCIt definition : A sex cord-stromal tumor occurring in the ovary and testis. In females it occurs predominantly
in the first three decades of life and presents unilaterally as stage I disease in
the vast majority of cases. It is characterized by the presence of granulosa cells
forming macrofollicular structures. The majority of cases have a good prognosis. In
males it represents the most frequent congenital testicular neoplasm, and the vast
majority of cases occur in the perinatal period. It presents as a scrotal or abdominal
mass, and it more often affects the left testis. Approximately 20% of the patients
have ambiguous external genitalia. It is characterized by the presence of cystic spaces
lined by granulosa cells and cells resembling theca cells. Metastases have not been
reported.;
Alternative definition : NICHD: A rare type of tumor derived from the granulosa cells in the ovary or testis.
Ninety percent of girls with the tumor will have low stage disease, which is curable
with surgery alone. In males, it usually presents in infancy (median age, 6 days),
which is treated with surgery alone.;