Juvenile granulosa cell tumor (ovary)

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.

Epidemiology

It typically occurs in premenarchal girls and young women. The mean age of presentation is 13 years.

Clinical presentation

JGCTs typically produce precocious puberty as a consequence of estrogen secretion. Rarely, they produce androgenic hormones.

Pathology

Associations

Radiographic features

Ultrasound
  • 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
  • "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 JGCTs (~90%) are detected at a low stage and surgery is curative. Higher stage disease has a worse prognosis and may require chemotherapy .

Inhibin can be used as a serum marker for diagnosis and post-treatment follow up.

See also