Malignant Sertoli-Leydig cell tumor of…

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Malignant Sertoli-Leydig cell tumor of the ovary

ORPHA:99916Disease

Also called Androblastoma · Arrhenoblastoma · Ovarian Sertoli-Leydig cell cancer · Ovarian malignant Sertoli-Leydig cell tumor · Virilizing ovarian tumor

What it is

A rare malignant sex cord stromal tumor of ovary occuring typically in young women and characterized by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea), when functional.

Key facts

Prevalence
<1 / 1 000 000 (annual incidence, Europe)
Age of onset
Adolescent, Adult, Childhood, Elderly
Classified as
Disease

Recorded for the broader condition

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

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.

Gene

DICER1Major susceptibility factor

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

GARD 5495MONDO 0020542UMLS C5574973

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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