[The multiple hamartoma syndrome (Cowden syndrome)].
The multiple hamartoma syndrome is a genetic disorder of autosomal dominant inheritance. It is characterized by an impressive diversity and multitude of both mesodermal and epithelial hamartomas and tumors of all organ systems. The dermatological hallmarks of this probably not too rare syndrome are lichenoid centrofacial and akral papular lesions and a marked papillomatosis of the entire oral mucosa of a highly characteristic morphology which usually extends throughout the entire length of the gastrointestinal tract. The importance of this syndrome is its frequent association with malignant tumors, predominantly carcinomas of the mammary and thyroid glands. In this paper, we describe the first three cases of the German literature. No malignancies were detected; in one case, a meningeoma causing severe increase of intracranial pressure was discovered.
D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005350:Fibroma / Q000175:diagnosis; D006222:Hamartoma / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D008297:Male; D009062:Mouth Neoplasms / Q000175:diagnosis; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000235:genetics; D010212:Papilloma / Q000175:diagnosis; D012878:Skin Neoplasms / Q000175:diagnosis*; D013274:Stomach Neoplasms / Q000175:diagnosis; D013577:Syndrome
Das Multiple-Hamartome-Syndrom (Cowden-Syndrom).