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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.
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.
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.
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.
Same organ: Adenosquamous carcinoma of the cervix, Small cell neuroendocrine carcinoma of the cervix, Bartholin gland carcinoma, Vulvar melanoma, Vaginal melanoma, 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, POLE-ultramutated endometrial cancer, Mismatch-repair-deficient endometrial cancer, p53-abnormal endometrial cancer, including uterine serous carcinoma, Endometrial cancer with no specific molecular profile, Advanced or recurrent endometrial cancer, Uterine carcinosarcoma, Early cervical cancer and fertility-sparing surgery, Locally advanced cervical cancer, Recurrent or metastatic cervical cancer, Platinum-sensitive ovarian cancer, Platinum-resistant ovarian cancer, HPV-associated vulvar squamous cell carcinoma, HPV-independent vulvar squamous cell carcinoma (p53-mutant), Vaginal squamous cell carcinoma (HPV-associated), Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma), Low-risk gestational trophoblastic neoplasia (FIGO score 0 to 6), High-risk gestational trophoblastic neoplasia (FIGO score 7 or more, including ultra-high-risk), Placental-site trophoblastic tumour and epithelioid trophoblastic tumour
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.
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The observation after complete surgery for stage I granulosa cell tumour, lifelong inhibin monitoring and endocrine or bevacizumab-based options at relapse follow this guideline.
FOXL2 mutation testing is now used to confirm the diagnosis of adult granulosa cell tumour, and the mutation is central to understanding and treating the disease.
Query 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.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
A swollen painful calf, or sudden breathlessness with chest pain; the tamoxifen boxed warning covers pulmonary embolism and stroke.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Dose by Calvert formula using GFR (see the calculators).
During chemotherapy a temperature over 37.5 C or below 36 C, shivering, or feeling unwell even with a normal temperature means ringing the hospital's 24-hour line straight away; breathing very fast, confusion, mottled skin or no urine in a day means 999.
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
See all on the product pages:BevacizumabCarboplatinLetrozole (and other aromatase inhibitors)Paclitaxel / nab-paclitaxel·Printable cards in the navigator
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