# Adult granulosa cell tumour of the ovary

Source: https://onco.cc/cancers/granulosa-cell-tumour/  
OnCo record `granulosa-cell-tumour` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Granulosa cell tumours, a rare form of ovarian cancer, make oestrogen, so they often announce themselves with abnormal bleeding, and almost all carry the same single FOXL2 mutation. Surgery cures most; relapses come late and are treated with further surgery, hormone-blocking drugs, bevacizumab or chemotherapy.

## Summary

Adult granulosa cell tumours arise from the hormone-producing cells of the ovarian follicle and carry a FOXL2 C134W mutation in about 97 percent of cases, one of the most specific mutations in oncology. Oestrogen production causes irregular bleeding, endometrial hyperplasia and occasionally endometrial cancer, and inhibin B and anti-Mullerian hormone serve as tumour markers. Surgery, fertility-sparing where appropriate, cures most stage I disease; adjuvant chemotherapy for higher stages is debated. Relapse, typically in the pelvis and abdomen years or decades later, is managed with repeat surgery, aromatase inhibitors or other hormonal therapy, bevacizumab, or platinum-based chemotherapy such as carboplatin-paclitaxel or BEP.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Granulosa cell tumor; AGCT; Sex cord-stromal tumour of the ovary
- Tags: subtype-page
- Group: gynaecologic
- Burden: The commonest malignant sex cord-stromal tumour but only two to five percent of ovarian cancers; most are found at stage I and cured, yet a third relapse, sometimes twenty or thirty years later, so follow-up is lifelong.
- Subtypes: Adult granulosa cell tumour (FOXL2 C134W); Juvenile granulosa cell tumour (children and young women, different biology); Other sex cord-stromal tumours (Sertoli-Leydig, DICER1-related)
- Biomarkers: FOXL2 C134W mutation (diagnostic); Inhibin B and anti-Mullerian hormone (monitoring); Oestradiol; Stage and rupture at surgery (prognosis)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/granulosa-cell-tumour/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/granulosa-cell-tumour/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/granulosa-cell-tumour/#what-it-is [3 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/granulosa-cell-tumour/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/granulosa-cell-tumour/#treating-it [3 settings, 1 decision with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/granulosa-cell-tumour/#evidence [2 key papers, 3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/granulosa-cell-tumour/#science [2 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/granulosa-cell-tumour/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/granulosa-cell-tumour/#living-with-it [13 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/granulosa-cell-tumour/coming/ [4 medicines, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/granulosa-cell-tumour/data/ [19 connected records]

## Standard of care

- Stage I: Surgical staging with hysterectomy and bilateral salpingo-oophorectomy, or unilateral oophorectomy to preserve fertility; endometrial sampling because of oestrogen exposure; no adjuvant therapy. ([Ovarian cancer](https://onco.cc/cancers/ovarian/))
- Advanced or relapsed: Repeat cytoreduction; aromatase inhibitors such as letrozole; bevacizumab; carboplatin-paclitaxel or BEP chemotherapy. ([Letrozole (and other aromatase inhibitors)](https://onco.cc/drugs/letrozole/), [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/))
- Follow-up: Lifelong monitoring with inhibin B and imaging because relapses occur decades later. ([Ovarian cancer](https://onco.cc/cancers/ovarian/))

## State of the art

- The FOXL2 mutation, found in 2009, gives a definitive diagnosis and a target for research.
- Hormonal therapy and bevacizumab have added low-toxicity options for relapse.
- Registries and rare-tumour networks are replacing case series as the evidence base.

## Open problems

- No randomised trials guide adjuvant or relapse treatment.
- Late relapse makes follow-up long and uncertain.
- FOXL2 is not yet druggable.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Granulosa_cell_tumour
- Wikipedia: https://en.wikipedia.org/wiki/Granulosa_cell_tumour

## Connected records

- cancers: [Clear cell ovarian cancer](https://onco.cc/cancers/clear-cell-ovarian-cancer/), [Leydig cell tumour of the testis](https://onco.cc/cancers/leydig-cell-tumour/), [Low-grade serous ovarian cancer](https://onco.cc/cancers/low-grade-serous-ovarian-cancer/), [Mucinous ovarian cancer](https://onco.cc/cancers/mucinous-ovarian-cancer/), [Ovarian cancer](https://onco.cc/cancers/ovarian/), [Pleuropulmonary blastoma (types I, Ir, II and III)](https://onco.cc/cancers/pleuropulmonary-blastoma-dicer1/), [Sertoli cell tumour of the testis](https://onco.cc/cancers/sertoli-cell-tumour/)
- key papers: [Mutation of FOXL2 in granulosa cell tumours of the ovary](https://onco.cc/key-papers/paper-shah-foxl2-granulosa-nejm-2009/), [Non-epithelial ovarian cancer: ESMO clinical practice guidelines](https://onco.cc/key-papers/paper-esmo-non-epithelial-ovarian-ray-coquard-ann-oncol-2018/)
- drugs: [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Letrozole (and other aromatase inhibitors)](https://onco.cc/drugs/letrozole/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/)

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JSON: https://onco.cc/api/v1/entities/granulosa-cell-tumour.json