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.
Chapman
M S
MS
Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03756, USA.
Perry
A E
AE
Baughman
R D
RD
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*