Juvenile granulosa cell tumor (ovary)
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A juvenile granulosa cell tumour of the ovary (JGCT) is a less common subtype of granulosa cell tumour of the ovary (~5% of cases). They are classified as ovarian sex cord / stromal tumours.
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 oestrogen secretion. Rarely, they produce androgenic hormones.
Pathology
Associations
Radiographic features
Uterine enlargement and/or endometrial thickening may be present
Ultrasound
- appearance varies widely: may appear anywhere from a solid 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 tumours
MRI
- "sponge-like" appearance
- solid areas of intermediate T2 signal intensity
- multilocular cystic component
- increased T1 signal in multiple locules, compatible with haemorrhage into the cystic locule
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 7.
Inhibin can be used as a serum marker for diagnosis and post-treatment follow up.
See also
-<a title="Maffucci syndrome" href="/articles/maffucci-syndrome">Maffucci syndrome</a> <sup>5</sup>- +<a href="/articles/maffucci-syndrome">Maffucci syndrome</a> <sup>5</sup>
-<a title="Ollier disease" href="/articles/enchondromatosis">Ollier disease</a> <sup>7</sup>- +<a href="/articles/enchondromatosis">Ollier disease</a> <sup>7</sup>
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