Familial ossifying fibroma

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Familial ossifying fibroma

ORPHA:435329Disease

Also called Familial Gigantiform cementoma · Multiple ossifying fibroma

What it is

A rare genetic bone disease characterized by multifocal, painless, benign fibrocemento-osseous lesions of the jaws which expand progressively and can cause severe facial deformity. It usually manifests at an early age and is often associated with abnormalities of the long bones and pathologic fractures. Radiologically, the lesions are of mixed radiopaque/radiolucent appearance. Incomplete surgical removal may lead to more rapid growth of the residual lesion.

Key facts

Age of onset
Adolescent, Adult, Childhood
Inheritance
Autosomal dominant
Classified as
Disease

ICD-10 codes

D16.4filed under a broader ICD-10 category — shared with 5 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

MEDDRA 10081225MESH C563017MONDO 0007660OMIM 137575UMLS C3495361

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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