LHERMITTE-DUCLOS DISEASE AND GIANT MENINGIOMA AS MANIFESTATIONS OF COWDENS-DISEASE

A 43-year-old man with synchronous occurrence of Lhermitte-Duclos dise ase (dysplastic gangliocytoma of the cerebellum) and a giant meningiom a with subcutaneous infiltration is reported. Subsequent examinations revealed typical manifestations of Cowden's disease (multiple hamartom a-neoplasia syndrome), i.e. mucocutaneous lesions and increased head c ircumference. Our findings support the view that there is a correlatio n between Lhermitte-Duclos disease and Cowden's disease. We conclude t hat all patients with Lhermitte-Duclos disease should be thoroughly ex amined with regard to Cowden's disease, which necessitates lifelong fo llow-up with screening for neoplasia in various organ systems. The occ urrence of both Lhermitte-Duclos disease and a meningioma as part of t he Cowden's symptom complex is very rare and to our knowledge only one case with this combination of lesions has previously been reported.