Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report

• Published in: Brain tumor pathology 2007-05, Vol.24 (1), p.25-29
• Main Authors: Inoue, Tomoo, Nishimura, Shinjitsu, Hayashi, Nakamasa, Numagami, Yoshihiro, Kaimori, Mitsuomi, Nishijima, Michiharu
• Format: Article
• Language: English
• Subjects: Brain Diseases - metabolism; Brain Diseases - pathology; Brain Diseases - surgery; Female; Hamartoma Syndrome, Multiple - metabolism; Hamartoma Syndrome, Multiple - pathology; Hamartoma Syndrome, Multiple - surgery; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Middle Aged
• ISSN: 1433-7398; 1861-387X
• DOI: 10.1007/s10014-006-0211-z

Lhermitte-Duclos disease is a rare clinical entity characterized by slow deformation of the cerebellar lesion. A 53-year-old woman presented with Lherimitte-Duclos disease manifesting as ataxic gait, occipital headache, and loss of consciousness. Magnetic resonance imaging demonstrated striated and laminar pattern lesions in the right cerebellar hemisphere and vermis. She underwent subtotal removal of the vermis compressing the brainstem, but the cerebellar hemisphere appeared normal and was preserved. Histological findings were consistent with Lherimitte-Duclos disease. Two years later, magnetic resonance imaging revealed enlargement of the right hemispheric lesion. Intraoperative appearance of the hemisphere demonstrated marked enlargement of the folia. Subtotal removal was performed. Histological examination showed no malignant findings. Lhermitte-Duclos disease may recur, and should be carefully followed up as a low-grade neoplasm, not as a hamartomatous disorder.