Unique association of Lhermitte–Duclos disease and frontal encephalocele

Abstract

Lhermitte–Duclos disease, also known as dysplastic gangliocytoma of the cerebellum, is an extremely rare cerebellar tumor characterized by an enlargement of cerebellar folia. Its etiology remains controversial. Imaging modalities, especially magnetic resonance imaging, contribute to establish a correct and accurate diagnosis with pre and postoperative assessment. The characteristic “tiger striping” appearance is pathognomonic. The recognition of this unusual and benign condition is particularly important, given the possible association with Cowden’s syndrome and therefore the need to identify concurrent malignancies.To our knowledge, we describe a rare and unique presentation of histologically proven Lhermitte–Duclos disease associated with a frontal encephalocele surgically treated.