Cowden's syndrome, Lhermitte-Duclos disease, and sclerotic fibroma.
The authors report a case of Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma, in which a cutaneous sclerotic fibroma was found incidentally during the second resection of a recurrent cerebellar hamartoma. The association of Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome led to a dermatologic examination and confirmation of the diagnosis of Cowden's syndrome. The combination of both Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome has not previously been reported. A review of the 15 cases of Lhermitte-Duclos disease associated with Cowden's syndrome shows no significant gender predilection. Sclerotic fibromas, once thought to be specific for Cowden's syndrome, also are reviewed.
Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03756, USA.
M S
MS
Chapman
A E
AE
Perry
R D
RD
Baughman
D000328:Adult; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology*; D003937:Diagnosis, Differential; D005350:Fibroma / Q000150:complications / Q000473:pathology*; D005729:Ganglioneuroma / Q000150:complications / Q000473:pathology*; D006223:Hamartoma Syndrome, Multiple / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D012871:Skin Diseases / Q000150:complications / Q000473:pathology*; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology*