Fact-sheet: Granulosa cell tumor
Updated on 04/16/2025 at 11:18 AM
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Definition
Ovarian granulosa cell tumors are a type of sex cord-stromal ovarian tumor. They are considered malignant but with low metastatic potential (lung, liver, bone). They arise from proliferating granulosa cells of the late preovulatory follicle.
They are rare and account for 1 to 3% of all ovarian tumors. They are the most common tumors among sex cord-stromal tumors (70%) and among hormone-secreting tumors (80%).
Two main subtypes are distinguished:
· the "adult" granulosa cell tumor, much more common, occurring after age 30 and accounting for approximately 95% of cases,
· the "juvenile" granulosa cell tumor, occurring before age 20, most often between 0 and 10 years.
Clinical features
Tumor-related symptoms: abdominal pain, distension or bloating, or even an acute presentation in cases of hemorrhage or rupture.
Endocrine syndrome related to the tumor's estrogenic activity:
· vaginal bleeding or precocious pseudopuberty in children.
· menorrhagia and breast swelling in adolescents and adults.
· uterine abnormalities: endometrial hyperplasia, endometrial polyps or endometrial carcinoma, adenomyosis
· external genital abnormalities
Association between juvenile granulosa cell tumor and Maffucci syndrome (Enchondromatosis, cutaneous hemangiomas).
Laboratory findings
Granulosa cell tumors are distinguished from other ovarian carcinomas by their hormonal activity; their capacity to secrete estrogen, inhibin, and Müllerian inhibiting substance accounts for some of the clinical manifestations and also provides useful tumor markers for disease surveillance.
Hyperestrogenism is a key feature.
Ultrasound
Highly variable sonographic appearance: solid mass, multilocular solid and cystic mass, or purely cystic lesion.
Variable degrees of hemorrhage or fibrosis may be observed.
Intracystic papillary projections are less commonly found than in epithelial ovarian tumors.
Two main patterns:
- large solid tumor with fluid areas of necrotic appearance.
- large multilocular tumor with solid components, sometimes bulky but rarely papillary excrescences. The locules are numerous and small ('sponge-like' appearance).
Both types show moderate to marked vascularity on Doppler.
Ascites (approximately 20% of cases).
CT
Large solid-cystic mass arising from an ovary with multiple fluid-filled loculations, well-defined, low density.
Heterogeneous enhancement.
Staging workup to search for pulmonary, hepatic, and bone metastases (rare)
MRI
Key MRI findings:
· Solid lesion with T2 hyposignal and central necrosis
· Diffusion hyperintensity
· Type 2 post-contrast enhancement curve
· Thickened endometrium and adenomyosis related to its secretory nature
Management
Pathological diagnosis
Surgery
Multidisciplinary tumor board and oncologic follow-up