desmoid disease, hereditary

ORPHA: 873

Overview

desmoid tumor (DT) is a benign, locally invasive soft tissue tumor associated with a high recurrence rate but with no metastatic potential

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with desmoid disease, hereditary, sourced from HPO and Orphanet clinical annotations.

HydronephrosisLimitation of joint mobilitySubcutaneous noduleMalabsorptionAbdominal painGastrointestinal hemorrhageOsteolysisArthralgiaAbnormality of the musculatureMyalgiaAbnormal abdominal wall morphologyIntestinal obstructionAbnormal retinal pigmentationNeoplasm of the skinFibromaAbnormality of the upper urinary tractGastrointestinal desmoid tumorChest painSepsisIntestinal polyposis

Classification & Codes

Orphanet Code

ORPHA:873
desmoid disease, hereditary
OrphanetORPHA:873
Treatments0 drug(s)
Symptoms on record20 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO