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hereditary desmoid disease (familial infiltrative fibromatosis)
Genetics:
- autosomal dominant (100% penetrance)
- associated with defects in APC
Clinical manifestations:
- multifocal fibromatosis of the paraspinal muscles, breast, occiput, arms, lower ribs, abdominal wall
- mesentery desmoid tumors appear as a complication of familial adenomatous polyposis
Related
desmoid tumor
General
genetic syndrome (multisystem disorder)
Database Correlations
OMIM 135290
References
OMIM :accession 135290