Juvenile granulosa cell tumors of the ovary (JGCT) are a less common subtype of granulosa cell tumor of the ovary (~5% of cases). They are classified as ovarian sex cord / stromal tumors.
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Epidemiology
It typically occurs in premenarchal girls and young women. The mean age of presentation is 13 years.
Associations
Clinical presentation
Juvenile granulosa cell tumors of the ovary typically produce precocious puberty as a consequence of estrogen secretion. Rarely, they produce androgenic hormones.
Radiographic features
Ultrasound
the appearance varies widely: may appear anywhere from a solid ovarian mass, to a multiloculated solid and cystic mass, to a purely cystic lesion
varying degrees of hemorrhage and/or fibrosis
typically unilateral
less likely to have intracystic papillary projections than epithelial ovarian tumors
MRI
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"sponge-like" appearance
solid areas of intermediate T2 signal intensity
multilocular cystic component
increased T1 signal in multiple locules, compatible with hemorrhage into the cystic locule
Uterine enlargement and/or endometrial thickening may also be present.
Treatment and prognosis
Most juvenile granulosa cell tumors of the ovary (~90%) are detected at a low stage and surgery is curative. Higher-stage disease has a worse prognosis and may require chemotherapy 7.
Inhibin can be used as a serum marker for diagnosis and post-treatment follow-up.
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