juvenile granulosa cell tumour of the 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

• Maffucci syndrome
• Ollier disease

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

• ovarian tumors

Siehe auch:

• Neoplasien des Ovars
• adulter Granulosazelltumor des Ovars
• Maffucci-Syndrom
• Pubertas praecox
• Granulosazelltumor
• ovarian sex cord-stromal tumours
• Granulosazelltumor des Ovars

und weiter:

• sex cord - stromal ovarian tumours