{"entity":{"id":"granulosa-cell-tumour","kind":"cancer","name":"Adult granulosa cell tumour of the ovary","aka":["Granulosa cell tumor","AGCT","Sex cord-stromal tumour of the ovary"],"tldr":"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.","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.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Granulosa_cell_tumour","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Granulosa_cell_tumour"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"gynaecological","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)"],"standardOfCare":[{"setting":"Stage I","approach":"Surgical staging with hysterectomy and bilateral salpingo-oophorectomy, or unilateral oophorectomy to preserve fertility; endometrial sampling because of oestrogen exposure; no adjuvant therapy.","refs":["ovarian"]},{"setting":"Advanced or relapsed","approach":"Repeat cytoreduction; aromatase inhibitors such as letrozole; bevacizumab; carboplatin-paclitaxel or BEP chemotherapy.","refs":["letrozole","bevacizumab","carboplatin","paclitaxel"]},{"setting":"Follow-up","approach":"Lifelong monitoring with inhibin B and imaging because relapses occur decades later.","refs":["ovarian"]}],"stateOfArt":["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."],"history":[{"year":1855,"title":"Rokitansky describes granulosa cell tumour","refs":[]},{"year":2009,"title":"FOXL2 C134W mutation found in almost all adult granulosa cell tumours","refs":[]},{"year":2020,"title":"ALIENOR: bevacizumab tested with paclitaxel in relapsed sex cord-stromal tumours","refs":["bevacizumab"]}],"pipeline":["bevacizumab","letrozole"],"openProblems":["No randomised trials guide adjuvant or relapse treatment.","Late relapse makes follow-up long and uncertain.","FOXL2 is not yet druggable."],"parent":"ovarian"},"route":"/cancers/granulosa-cell-tumour/","neighbours":{"drug":[{"id":"bevacizumab","kind":"drug","name":"Bevacizumab","route":"/drugs/bevacizumab/"},{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"letrozole","kind":"drug","name":"Letrozole (and other aromatase inhibitors)","route":"/drugs/letrozole/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"}],"cancer":[{"id":"ovarian","kind":"cancer","name":"Ovarian cancer","route":"/cancers/ovarian/"}]}}