Adult granulosa cell tumour of the ovary
Granulosa cell tumours 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.
Overview
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.
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.
Anatomy and lymph node drainage
Most high-grade ovarian cancers begin at the tip of the fallopian tube; endometrial cancer lines the uterus, cervical cancer starts at the transformation zone; each drains to a different node group.
- Fallopian tube fimbria (origin of high-grade serous)
- Ovary (other histologies, germ cell)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)
- Endometrium
- Myometrium (uterine sarcoma)Other sex cord-stromal tumours (Sertoli-Leydig, DICER1-related)
- Placental site (gestational trophoblastic)
- Cervix, transformation zone
- Vulva
- obturator and external iliac
- internal iliac
- para-aortic (ovary, high uterus)
- inguinal (vulva)
Same organ: High-grade serous ovarian cancer, Low-grade serous ovarian cancer, Clear cell ovarian cancer, Mucinous ovarian cancer, Ovarian cancer, Endometrial cancer, Cervical cancer, Vulvar cancer, Gestational trophoblastic neoplasia, Uterine sarcoma, Vaginal cancer
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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Surgical staging with hysterectomy and bilateral salpingo-oophorectomy, or unilateral oophorectomy to preserve fertility; endometrial sampling because of oestrogen exposure; no adjuvant therapy.
Repeat cytoreduction; aromatase inhibitors such as letrozole; bevacizumab; carboplatin-paclitaxel or BEP chemotherapy.
Lifelong monitoring with inhibin B and imaging because relapses occur decades later.
Subtypes & biomarkers
top- 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)
- FOXL2 C134W mutation (diagnostic)
- Inhibin B and anti-Mullerian hormone (monitoring)
- Oestradiol
- Stage and rupture at surgery (prognosis)
How often this target appears
- 1855Rokitansky describes granulosa cell tumour
- 2009FOXL2 C134W mutation found in almost all adult granulosa cell tumours
What is in development for Adult granulosa cell tumour of the ovary, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
No randomised trials guide adjuvant or relapse treatment.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Late relapse makes follow-up long and uncertain.
and how the field plans to fix it →What is being done about thisRecurrence and residual diseaseAvailable now- Liquid biopsy (ctDNA)Standard of care
- MRD / molecular residual disease testingEstablished
- Multiparameter flow cytometry MRDStandard of care
- NGS-based MRD (clonoSEQ and molecular MRD)Standard of care
- SignateraEstablished
- Structured exercise programmes after curative treatmentEstablished
In trials- ADAURAPositive
- CAMBRIA-1 & CAMBRIA-2Active
- CIRCULATE-Japan (GALAXY / VEGA / ALTAIR)Active
- ctDNA monitoring in lymphoma (PhasED-seq, clonoSEQ)Emerging
- DYNAMICPositive
- IMvigor011Positive
Ideas and roadmaps- A blood test for the pre-metastatic niche
- A bone marrow niche on a chip to study human dormancy
- A dedicated clinic for people whose blood test says the cancer is back
- A drug screen that only rewards killing sleeping cancer cells
- A national platform trial that every ctDNA-positive patient can join
- A national residual-disease weather service: serial blood tests for every curatively treated patient, pooled
Background: Circulating tumour DNA (ctDNA), Disseminated tumour cells (DTCs), Late recurrence, Minimal / molecular residual disease (MRD), MRD negativity (myeloma, 10⁻⁵ / 10⁻⁶). Also on OnCo: Treatment journeys · Survivorship planner.
FOXL2 is not yet druggable.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Expert centres
topExpert centres
- via Ovarian cancer
- Alliance for Clinical Trials in OncologyChicago, IL, USvia Carboplatin
- ARCAGY-GINECOParis, FRvia Ovarian cancer
- BC CancerVancouver, BC, CAvia Ovarian cancer
- via Ovarian cancer
- Centre hospitalier de l'Université de Montréal (CHUM)Montréal, QC, CAvia Ovarian cancer
- Centre Oscar LambretLille, FRvia Ovarian cancer
- Chris O'Brien LifehouseSydney, AUvia Ovarian cancer
- Edinburgh Cancer Centre / CRUK Scotland CentreEdinburgh, GBvia Ovarian cancer
- via Ovarian cancer
- German Breast Group (GBG)Neu-Isenburg, DEvia Carboplatin
- GOG FoundationPhiladelphia, PA, USvia Ovarian cancer
- Institut BergoniéBordeaux, FRvia Ovarian cancer
- Institute of Oncology LjubljanaLjubljana, SIvia Carboplatin
- via Ovarian cancer
- via Ovarian cancer
- via Ovarian cancer
- Newcastle Cancer Centre / Northern Centre for Cancer CareNewcastle upon Tyne, GBvia Ovarian cancer
- Northwell Health Cancer InstituteNew Hyde Park, NY, USvia Ovarian cancer
- via Ovarian cancer
- Ontario Institute for Cancer ResearchToronto, ON, CAvia Ovarian cancer
- via Ovarian cancer
- Peking Union Medical College HospitalBeijing, CNvia Ovarian cancer
- QIMR Berghofer Medical Research InstituteBrisbane, AUvia Ovarian cancer
- Shaare Zedek Medical CenterJerusalem, ILvia Ovarian cancer
- Society of Gynecologic OncologyChicago, IL, USvia Ovarian cancer
- via Ovarian cancer
- via Ovarian cancer
- via Ovarian cancer
- via Ovarian cancer
- UZ Leuven / Leuven Cancer InstituteLeuven, BEvia Ovarian cancer
Questions to ask
topQuestions to ask your oncologist about Adult granulosa cell tumour of the ovary
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example FOXL2 C134W mutation, Inhibin B and anti-Mullerian hormone, Oestradiol, Stage and rupture at surgery), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Adult granulosa cell tumour, Juvenile granulosa cell tumour, Other sex cord-stromal tumours.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Stage I
- For my situation (stage i), which of the standard options do you recommend and why?Why: Guideline options include: Surgical staging with hysterectomy and bilateral salpingo-oophorectomy, or unilateral oophorectomy to preserve fertility; endometrial sampling because of oestrogen exposure; no adjuvant therapy.
Advanced or relapsed
- For my situation (advanced or relapsed), which of the standard options do you recommend and why?Why: Guideline options include: Repeat cytoreduction; aromatase inhibitors such as letrozole; bevacizumab; carboplatin-paclitaxel or BEP chemotherapy.
- Am I a candidate for Letrozole (and other aromatase inhibitors), Bevacizumab, Carboplatin or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Follow-up
- For my situation (follow-up), which of the standard options do you recommend and why?Why: Guideline options include: Lifelong monitoring with inhibin B and imaging because relapses occur decades later.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No randomised trials guide adjuvant or relapse treatment”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Late relapse makes follow-up long and uncertain”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
5targets
2drugs
4companies
3Latest papers
topQuery for this cancer: (TITLE:"Adult granulosa cell tumour of the ovary" OR ABSTRACT:"Adult granulosa cell tumour of the ovary" OR TITLE:"Granulosa cell tumor" OR ABSTRACT:"Granulosa cell tumor" OR TITLE:"AGCT" OR ABSTRACT:"AGCT" OR TITLE:"Sex cord-stromal tumour of the ovary" OR ABSTRACT:"Sex cord-stromal tumour of the ovary") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Adult granulosa cell tumour of the ovary, not a curated reading list.
Similar pages
not linked directly; found by shared links- CancerLow-grade serous ovarian cancer
Shares Letrozole (and other aromatase inhibitors), Paclitaxel / nab-paclitaxel, Carboplatin, Ovarian cancer and the tag subtype-page.
- CancerClear cell ovarian cancer
Shares Paclitaxel / nab-paclitaxel, Carboplatin, Ovarian cancer and the tag subtype-page.
- CancerMucinous ovarian cancer
Shares Carboplatin, Ovarian cancer and the tag subtype-page.
- CancerHigh-grade serous ovarian cancer
Shares Bevacizumab, Paclitaxel / nab-paclitaxel, Carboplatin, Ovarian cancer and the tag subtype-page.
- CancerLeiomyosarcoma
Shares Letrozole (and other aromatase inhibitors) and the tag subtype-page.
- CancerOesophageal squamous cell carcinoma
Shares Paclitaxel / nab-paclitaxel, Carboplatin and the tag subtype-page.
- CancerSeminoma
Shares Carboplatin and the tag subtype-page.
- CancerAnaplastic thyroid cancer
Shares Paclitaxel / nab-paclitaxel and the tag subtype-page.