A case of Cowden’s syndrome associated with Lhermitte-Duclos disease

Dae Kwan Lim,; Sung Eun Kim,; Jung Young Song,; Do Hyun Kim,; Seon Jeong Kim.,; Moo In Park,; Seun Ja Park,

Korean J Med > Volume 76(1); 2009 > Article

Original Article

The Korean Journal of Medicine 2009;76(1):31-34.

A case of Cowden’s syndrome associated with Lhermitte-Duclos disease

Dae Kwan Lim, Sung Eun Kim, Jung Young Song, Do Hyun Kim, Seon Jeong Kim., Moo In Park, Seun Ja Park

Lhermitte-Duclos병을 동반한 Cowden병 1예

임대관, 김성은, 송준영, 김도현, 김선정, 박무인, 박선자

Abstract

Cowden’s disease, or multiple hamartoma syndrome, is an uncommon autosomal dominant condition characterized by mucocutaneous lesions and systemic malignancies. Lhermitte-Duclos disease, or dysplastic gangliocytoma of the cerebellum, is a rare cerebellar lesion that can cause mass effects in the posterior fossa. It may occur sporadically, or in association with Cowden’s syndrome. We report a case of Cowden’s disease associated with Lhermitte-Duclos disease in a 49-year-old man who underwent a routine checkup. (Korean J Med 76:S31-S34, 2009)

Key Words: Cowden disease; Lhermitte-Duclos disease