Lhermitte-Duclos disease and giant meningioma as manifestations of Cowden's disease.

A 43-year-old man with synchronous occurrence of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) and a giant meningioma with subcutaneous infiltration is reported. Subsequent examinations revealed typical manifestations of Cowden's disease (multiple hamartomaneoplasia syndrome), i.e. mucocutaneous lesions and increased head circumference. Our findings support the view that there is a correlation between Lhermitte-Duclos disease and Cowden's disease. We conclude that all patients with Lhermitte-Duclos disease should be thoroughly examined with regard to Cowden's disease, which necessitates lifelong follow-up with screening for neoplasia in various organ systems. The occurrence of both Lhermitte-Duclos disease and a meningioma as part of the Cowden's symptom complex is very rare and to our knowledge only one case with this combination of lesions has previously been reported.