Granulosa Cell Tumour and Sex Cord Stromal Tumours
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Direct answer
Granulosa cell tumour is the commonest malignant sex cord-stromal tumour of the ovary, and it secretes oestrogen, causing irregular bleeding, endometrial hyperplasia or precocious puberty. Diagnosis is supported by Call-Exner bodies on histology and by raised inhibin and anti-Mullerian hormone levels. Sex cord-stromal tumours also include fibroma, which can cause Meigs syndrome, and Sertoli-Leydig tumours, which virilise.
What you must remember
- Adult granulosa cell tumour: the commoner of the two granulosa types, and the main malignant tumour of this group (fibroma-thecoma is the commonest sex cord-stromal group overall), usually seen in perimenopausal or postmenopausal women and typically unilateral.
- Juvenile granulosa cell tumour: occurs in children and causes precocious puberty, with a generally favourable prognosis.
- Call-Exner bodies: small rosette-like structures containing eosinophilic fluid, a characteristic histological feature.
- Oestrogen effects: endometrial hyperplasia, postmenopausal bleeding and precocious puberty, so endometrial carcinoma must be excluded.
- Markers: inhibin B and anti-Mullerian hormone track disease and recurrence better than CA-125.
- Fibroma: a benign tumour causing ascites and right-sided pleural effusion, which is Meigs syndrome and resolves after removal.
- Sertoli-Leydig cell tumour: androgen-secreting, causing virilisation such as hirsutism, deepening voice and clitoromegaly.
- Late recurrence: granulosa cell tumours can recur many years after apparently complete treatment, so follow-up is prolonged.
Sex cord-stromal tumours by hormone and clue
Sex cord-stromal tumours are classified by the hormone they secrete, and each has a characteristic clinical clue. The clue usually points to the hormone, and the hormone points to the work-up, the surgery and the follow-up plan.
| Tumour | Hormone or feature | Clinical clue | Management focus |
|---|---|---|---|
| Adult granulosa cell tumour | Oestrogen, inhibin B, anti-Mullerian hormone | Postmenopausal bleeding with endometrial hyperplasia on biopsy | Staging surgery and long follow-up, since recurrence can occur many years later |
| Juvenile granulosa cell tumour | Oestrogen | Precocious puberty in a young child | Fertility-sparing surgery is often possible in early disease |
| Thecoma | Oestrogen | Usually benign, postmenopausal | Surgical removal, with assessment of the endometrium for hyperplasia |
| Fibroma | No hormone | Ascites and right-sided pleural effusion, Meigs syndrome | Removal, after which the effusion and ascites usually resolve, confirming the diagnosis |
| Sertoli-Leydig cell tumour | Androgens | Virilisation in a young woman | Surgery guided by stage and histological grade, with hormone monitoring afterwards |
Fibromas and thecomas are usually benign, whereas granulosa and Sertoli-Leydig tumours need full staging, long follow-up and marker monitoring. Hormonal clues guide the workup, but histology confirms the diagnosis and the grade.
Recognising sex cord-stromal tumours
Questions in this area test the link between the hormone, the clinical picture and the marker.
- Trap: Attributing postmenopausal bleeding to atrophy alone Correct: check the ovaries and endometrium, since an oestrogen-secreting granulosa cell tumour causes hyperplasia.
- Trap: Relying on CA-125 to follow granulosa cell tumour Correct: inhibin B and anti-Mullerian hormone are the more useful markers.
- Trap: Calling Meigs syndrome malignant ascites Correct: a benign fibroma causes Meigs syndrome, and the ascites and effusion resolve after removal.
- Trap: Treating a virilising ovarian tumour as benign Correct: Sertoli-Leydig tumours vary in behaviour, so histological grade determines prognosis.
Frequently asked questions
What are Call-Exner bodies in granulosa cell tumour?
Call-Exner bodies are small rosette-like structures containing eosinophilic fluid, resembling primordial follicles. They are a characteristic histological feature of granulosa cell tumour, supporting the diagnosis on biopsy or resected specimen.
Which tumour marker is preferred for granulosa cell tumour?
Inhibin B and anti-Mullerian hormone are the preferred markers for granulosa cell tumour, as they correlate with tumour burden and recurrence. CA-125 is less reliable for this tumour type and does not replace these markers.
Why does granulosa cell tumour cause endometrial hyperplasia?
Granulosa cell tumour secretes oestrogen, and unopposed oestrogen stimulates endometrial proliferation, causing hyperplasia and raising the risk of endometrial carcinoma. Endometrial sampling is therefore part of the workup of postmenopausal bleeding in these women.
How does Sertoli-Leydig cell tumour present?
Sertoli-Leydig cell tumour usually presents with virilisation, including hirsutism, deepening voice, clitoromegaly and menstrual irregularity. Its behaviour ranges from benign to aggressive, so histological grade guides surgery and adjuvant treatment.