Cowden-Syndrom (Multiples Hamartom-Syndrom)

Zusammenfassung

Das Cowden-Syndrom ist ein Hamartom-Symptomkomplex mit individuell unterschiedlicher klinischer Ausprägung. Pathognomonische dermatologische Läsionen sind faziale mukokutane Papillome, faziale Tricholemmome und akrale Keratosis. Von größter prognostischer und therapeutischer Bedeutung ist das Auftreten von Malignomen, insbesondere Mamma- und Schilddrüsenkarzinomen. Die Diagnose wird anhand von klinischen Kriterien gestellt. Aufgrund des erhöhten Risikos sind Krebsvorsorgeuntersuchungen unerlässlich. Wir berichten über eine Patientin mit Cowden-Syndrom einschließlich fazialer Papillome, Schilddrüsenkarzinom und intestinaler hamartöser Polyposis.

Abstract

The Cowden syndrome is a complex hamartomous symptom with individually varying clinical manifestations. Diagnostic dermatological lesions include facial mucocutaneous papillomas, facial trichilemmoma and acral keratosis. Most important for prognostic and therapeutic reasons is the increased incidence of malignomas in Cowden-Syndrom such as breast and thyreoid cancer. The diagnosis of Cowden syndrome is based on clinical criteria. Due to the higher risk of cancer development in patients with Cowden syndrome, it is very important to perform complete medical follow-ups on a regular basis. We report a patient with Cowden syndrome including facial papillomatosis, thyreoid cancer and intestinal polyposis.

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