Granulosa cell tumour and sex cord-stromal tumours

Exam relevance: in 3 of 197 board exam reports · rank 125

Specialty: Gynaecology · Ovary & adnexa
Images: Histology & cytology 2 · Mammography/MRI 1
Exam relevance: 3 of 197 reports · rank 125
Last updated: 10/2026 · Dr. Pascal Bafteh

Contents

Images (3)
Definition
Classification
Aetiopathogenesis
Clinical features
Histology
Keep learning in the app
Further reading (selection)
Cross-references

Images (3)

Granulosa cell tumour and sex cord-stromal tumours – Histology & cytology: Histology (H&E): adult granulosa cell tumour – diffuse sheets of small cells with Call-Exner bodies (rosette-like spaces, circled in black) — Histology (H&E): adult granulosa cell tumour – diffuse sheets of small cells with Call-Exner bodies (rosette-like spaces, circled in black)Image: Mikael Häggström and Nephron (Wikimedia Commons) · CC BY-SA 3.0 · Source

Granulosa cell tumour and sex cord-stromal tumours – Mammography/MRI: CT (axial, contrast-enhanced): adult granulosa cell tumour of the ovary in a 78-year-old woman – very large, cystic, multiseptate tumour with solid components — CT (axial, contrast-enhanced): adult granulosa cell tumour of the ovary in a 78-year-old woman – very large, cystic, multiseptate tumour with solid componentsImage: Hellerhoff (Wikimedia Commons) · CC BY-SA 3.0 · Source

Granulosa cell tumour and sex cord-stromal tumours – Histology & cytology: Inhibin immunohistochemistry: cytoplasmic positivity of the tumour cell nests of a granulosa cell tumour, stroma negative — Inhibin immunohistochemistry: cytoplasmic positivity of the tumour cell nests of a granulosa cell tumour, stroma negativeImage: Ed Uthman from Houston, TX, USA (Wikimedia Commons) · CC BY 2.0 · Source

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Definition

Granulosa cell tumours belong to the sex cord-stromal tumours and consist of granulosa cells, theca cells and fibroblasts in varying combinations.
Sex cord-stromal tumours account for about 8% of all ovarian tumours.

Classification

Adult and juvenile types are distinguished: the adult type accounts for about 95% of granulosa cell tumours and mostly occurs peri- or postmenopausally, the juvenile type affects prepubertal girls and young women.
Thecomas are uncommon and usually benign.

Aetiopathogenesis

Pathogenesis

The somatic FOXL2 variant c.402C>G (p.C134W) is characteristic of adult granulosa cell tumour and was shown experimentally to be sufficient to trigger it.
Sertoli-Leydig cell tumours are associated with DICER1 syndrome, sex cord tumours with annular tubules with Peutz-Jeghers syndrome.

Clinical features

Typical are abdominal pain and swelling as well as oestrogen-related uterine bleeding; androgenic signs are less common (about 10%), and the tumours are usually detected at FIGO stage I.
Endometrial hyperplasia or endometrioid carcinoma may coexist, especially after the age of 40.
Granulosa cell tumours tend to recur late, sometimes decades after the initial diagnosis; depending on the series, up to a quarter to a third recur.
Thecomas often present with oestrogenic, less commonly androgenic, effects, sometimes with endometrial neoplasms.

Histology

Histology and diagnosis

Pale, longitudinally grooved nuclei with a coffee-bean appearance are typical; Call-Exner bodies are a hallmark but are present in only about 10% of tumours.
Sex cord-stromal tumours express markers such as inhibin, calretinin, SF1, WT1 and FOXL2; molecular pathology helps with classification.
Serum inhibin A and inhibin B may be raised.

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