ovarian juvenile granulosa cell tumor
Thursday 26 November 2009
Definition: Ovarian sex cord stromal tumor showing differentiation towards follicular granulosa cells that can occur in adults (adult granulosa cell tumor) and in younger patients (juvenile granulosa cell tumor).
Juvenile granulosa cell tumors comprise only 5% of ovarian tumors of childhood or adolescence.
Typically, they present as sexual precocity in prepubertal girls due to excessive estrogen production, although androgens may rarely be produced.
≈80% during first two decades of life
Usually presents with isosexual precocity
especially if bilateral
enchondromatosis (Ollier disease)
Grossly, these tumors are partially solid and cystic.
Hemorrhage or necrosis may be present.
- cystic juvenile granulosa cell tumor
- solid juvenile granulosa cell tumor
This tumor is composed of macrofollicles with eosinophilic secretions, and intervening tumor cells with abundant eosinophilic cytoplasm, round/oval hyperchromatic nuclei with small nucleoli, and mildly irregular nuclear contours. There are rare nuclear grooves. Other features often present include high mitotic activity, nuclear atypia and occasionally hobnail-like cells.
nodular or diffuse growth of granulosa cells
prominent cyst / follicle formation
no classical Call–Exner bodies
diffuse or macrofollicular patterns of growth (former predominating)
mucin-positive intrafollicular secretion
cystic areas surrounded by granulosa type cells
solid areas composed of bland ovoid granulosa cells with eosinophilic cytoplasm.
larger tumor cells with extensive luteinization
- eosinophilic cytoplasm
- no nuclear grooves
- mitoses common
- inhibin+ and CD99+
paucity of nuclear grooves
variable but often high mitotic activity
follicle-like spaces on low-power examination
On high power the tumor cells lack the coffee-bean nuclei seen in the adult type.
Juvenile granulosa cell tumor shows round-to-oval follicles that contain eosinophilic secretion.
- The follicles are separated by luteinized granulosa cells.
- Round-to-oval follicles that contain eosinophilic secretion.
- The follicles are separated by luteinized granulosa cells.
- Follicles of varying sizes and shapes are separated by cellular areas.
- abundant eosinophilic cytoplasm
- lack grooves
Juvenile granulosa cell tumor demonstrates strong staining for inhibin.
Positive immunostains include inhibin and calretinin.
- They are not usually needed for diagnosis, but may be helpful in excluding germ cell tumors or melanoma.
Consistent trisomy 12
Greater percentage of aneuploidy than in adult variety.
adult-type granulosa cell tumor
- Adult granulosa cell tumors have more regularly shaped follicles that contain basement membrane material.
- Tumor cells have prominent nuclear grooves and less hyperchromasia.
- Thecomas almost never occur in patients 30 years or younger, and usually lack mitotic activity.
- Thecomas also lack follicles, which are usually found in juvenile granulosa cell tumors with thorough sampling.
clear cell carcinoma.
- Patients with clear cell carcinoma are typically older.
- Clear cell carcinomas lack follicles or areas of typical juvenile granulosa type cells.
Although hobnail-type cells may be seen in juvenile granulosa cell tumors, they are typically focal.
Treatment and prognosis
Excision is usually adequate therapy for limited disease, although late recurrence may occur.
granulosa cell tumor
- juvenile granulosa cell tumor*
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