Lhermitte duclos disease and Cowden disease: clinical, pathological and neuroimaging study of a case.

The authors report the case of a 26-year-old female patient affected by Lhermitte Duclos disease and Cowden disease. Preoperative MRI allowed a correct diagnosis which was confirmed by pathological examination. The authors stress the possibility that Lhermitte Duclos and Cowden disease be a single phakomatosis; for this reason all the patients affected by Lhermitte-Duclos should be screened for the presence of multiple hamartomas or malignant neoplastic lesions typical of Cowden disease.