Epidemiology

Etiology

Hyperestrogenic manifestations:
- Juvenile / Prepubertal: Peripheral precocious puberty (isosexual: breast buds, pubic/axillary hair, early vaginal bleeding, accelerated linear growth with premature epiphyseal closure).
- Reproductive age: Abnormal uterine bleeding (AUB), heavy menstrual bleeding, oligomenorrhea/amenorrhea.
- Postmenopausal: Postmenopausal bleeding (PMB), breast tenderness, vaginal discharge.
Mass effect & local symptoms:
- Unilateral pelvic pain, fullness, pressure, or palpable adnexal mass on pelvic examination.
- Acute abdomen secondary to ovarian torsion or tumor rupture with hemoperitoneum.

Initial / Screening Imaging:
- Transvaginal Ultrasound (TVUS): Large, complex, solid-cystic adnexal mass with a multilocular or “Swiss cheese” appearance; thickened endometrial stripe (> 4 mm in postmenopausal women).
Key Labs / Tumor Markers:
- ↑ Inhibin (Inhibin A and B): Sensitive and specific tumor marker; gold standard for monitoring treatment response and detecting recurrence.
- ↑ Serum Estradiol (E2).
- Serum CA-125: Non-specific, may be mildly elevated.
Essential Adjunctive Procedure:
- Endometrial Biopsy (EMB): Mandatory in patients with AUB/PMB or thickened endometrium to rule out concurrent endometrial hyperplasia or cancer. c
Confirmatory / Gold Standard:
- Surgical Staging & Histopathology:
  - Call-Exner bodies: Rosette-like arrangement of granulosa cells around central eosinophilic, fluid-filled spaces.
  - “Coffee-bean” nuclei: Pale, round-to-oval nuclei with longitudinal grooving.
  - Immunohistochemistry (IHC): (+) Calretinin, (+) Inhibin, (+) WT-1, (+) FOXL2.

Mnemonic

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