Cowden syndrome 1

ORPHA: 201

Overview

inherited disorder causing tumor-like growth and increased cancer risk

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 Cowden syndrome 1, sourced from HPO and Orphanet clinical annotations.

Abnormal penis morphologyAbnormality of the kidneyAbnormality of the uterusMacroglossiaHigh palateFurrowed tongueMacrocephalyHearing impairmentCataractMyopiaAutismPectus excavatumGynecomastiaAbnormality of the thyroid glandGoiterPalmoplantar keratodermaMelanocytic nevusCavernous hemangiomaHypopigmented skin patchesBrachydactylyIntellectual disabilitySeizureAtaxiaGlobal developmental delayAbnormal cerebellum morphologySubcutaneous noduleFailure to thriveIncreased intracranial pressureScoliosisNeoplasmKyphosisMeningiomaMelanomaBreast carcinomaShort statureHamartomatous polyposisCellular immunodeficiencyRenal cell carcinomaGeneralized hyperkeratosisFollicular thyroid carcinomaMultiple cafe-au-lait spotsNeoplasm of the skinEnlarged polycystic ovariesAdenoma sebaceumFibromaLipomaBone cystEndometrial carcinomaMaculePapillomaNeoplasm of the central nervous systemNeoplasm of the thyroid glandCognitive impairmentMucosal telangiectasiaeConjunctival hamartomaPapuleColorectal polyposis

Classification & Codes

Orphanet Code

ORPHA:201
Cowden syndrome 1
OrphanetORPHA:201
Treatments0 drug(s)
Symptoms on record57 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO