Granulosa cell tumours: adult and juvenile
- Lesion
An ovarian tumour that makes estrogen: it causes bleeding and endometrial cancer in older women, early puberty in girls, and can come back decades later.
What it is
Granulosa cell tumours are the most common malignant sex cord–stromal tumours. The adult type, which occurs around and after menopause, is defined by a somatic FOXL2 mutation and forms small rosettes called Call–Exner bodies among cells with grooved nuclei. Many tumours secrete estrogen, causing endometrial hyperplasia or carcinoma, and inhibin, a useful serum marker. The juvenile type affects children and young women and causes isosexual precocious puberty. Both are low-grade malignancies that can recur many years after removal.
Structure
- Gross: unilateral, solid and cystic, yellow to white tumour, often with haemorrhage; can rupture and cause haemoperitoneum.
- Adult: diffuse, trabecular, insular or microfollicular patterns of small cuboidal cells with scant cytoplasm and grooved nuclei.
- Juvenile: macrofollicles filled with secretion, cells with more cytoplasm and luteinisation, more mitoses.
Step by step
- A granulosa cell acquires a FOXL2 mutation that alters its transcriptional program (adult type).
- The clone proliferates and keeps granulosa functions: aromatase converts androgens to estrogen and the cells make inhibin.
- Estrogen stimulates the endometrium and breast.
- The tumour grows slowly; cells left in the peritoneum may form recurrences years later.
Markers
- Inhibin
- Calretinin
- SF1
- FOXL2
- Serum inhibin and anti-Müllerian hormone
Clinical notes
- Postmenopausal bleeding with a solid adnexal mass; always sample the endometrium, since hyperplasia or carcinoma is often present.
- In a girl, breast development and vaginal bleeding before the expected age with an abdominal mass suggest a juvenile granulosa cell tumour.
- Long follow-up is needed because of late recurrence.
Test yourself
Think of the answer, then tap the question to see it.
Which mutation defines the adult granulosa cell tumour?
A somatic FOXL2 mutation (C134W).
What are Call–Exner bodies?
Small rosette-like spaces containing eosinophilic material surrounded by granulosa cells.
Why should the endometrium be sampled in a woman with a granulosa cell tumour?
Estrogen from the tumour can cause endometrial hyperplasia or carcinoma.
Elsewhere in DurgiPedia
- Puberty
- Actions of estrogen and progesterone
- Aromatase (CYP19A1)
- Ovary: follicles, ovulation and corpus luteum
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: FOXL2 gene: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2026).
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Representative model, for educational purposes only.