A A Rare Case Report on Lhermitte-Duclos Disease: An Intraoperative and Radiopathological Diagnosis

Abstract

Background: Lhermitte-Duclos Disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a very rare tumor of the cerebellum, with only about 300 cases reported worldwide. It is a slow-growing benign tumor causing diffuse enlargement of the cerebellum due to granular layer hypertrophy.

Case Report: We present a case of a 30-year-old female with symptoms of giddiness, focal convulsions, and loss of consciousness, along with positive cerebellar signs. On MRI, an ill-defined intra-axial lesion showed classical alternating light and dark “tiger stripe†patterns with no contrast enhancement. Frozen and histopathological evaluations confirmed the diagnosis, as there was the presence of atypical ganglion cells replacing the internal molecular layer. The patient underwent total tumor resection.

Conclusion: Lhermitte-Duclos disease is a rare lesion that unilaterally enlarges the cerebellum but maintains the foliar architecture. It is identified as a component of Cowden syndrome, an autosomal dominant cancer predisposition disorder. Recognition of the histological characteristics of this uncommon condition and maintaining a heightened suspicion are imperative for an accurate diagnosis. This should prompt comprehensive examinations to rule out manifestations of associated Cowden syndrome. Lhermitte-Duclos Disease is an infrequent occurrence, and understanding its histological attributes and aligning them with radiological findings is crucial, particularly in small biopsy samples, to ensure a precise diagnosis.