Malignant granulosa cell tumor of the…

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Malignant granulosa cell tumor of the ovary

ORPHA:99915Disease

Also called Granulosa cell cancer · Granulosa cell malignant tumor

What it is

A rare malignant sex cord stromal tumor of ovary arising from the granulosa cells of the ovary, which occurs in peri and post menopausal women, and that presents with abnormal vaginal bleeding, abdominal pain and distension. The tumor is frequently unilateral, estrogen secreting, and has a slow natural history and a tendency to relapse long after the initial diagnosis.

Key facts

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)
Age of onset
Adolescent, Adult, Elderly
Inheritance
Multigenic/multifactorial
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

DICER1Major susceptibility factor
FOXL2Disease-causing somatic mutation(s)

ICD-10 codes

C56filed under a broader ICD-10 category — shared with 14 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MESH D006106MONDO 0020541UMLS C0346175

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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