Dysplastic cerebellar gangliocytoma

Abstract

Background Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) is benign tumor with atypical clinical manifestations and relatively rare incidence. This paper reported one case of dysplastic cerebellar gangliocytoma and reviewed related literatures, so as to explore its clinical and pathological characteristics and improve the diagnosis and differential diagnosis of dysplastic cerebellar gangliocytoma.  Methods and Results Head MRI of a 34-year-old female patient showed round space-occupying lesion in right cerebellar hemisphere. The lesion was totally removed by operation Intraoperative findings: the lesion had widened gyri on the surface with yellow or white color; it was similar to brain tissue, soft, rich blood supply, nonencapsuled, and was not adhered to surrounding brain tissues. Histologically, granular layers and Purkinje cell layers were replaced by parallel arranged abnormal myelinated fibers and abnormal neurons in disordered structures associated with vascular malformation. Immunohistochemical staining showed the cytoplasm of abnormal neuronal cells was positive for neuronal nuclei (NeuN), synaptophysin (Syn), S-100 protein (S-100) and neuron-specific enolase (NSE); glial fibers were positive for glial fibrillary acidic protein (GFAP); nerve fibers were positive for neurofilament protein (NF). Ki-67 labeling index was less than 1% . Final pathological diagnosis was dysplastic cerebellar gangliocytoma. The patient was in deep coma after operation, and was discharged after 24 d, but was lost after discharge.  Conclusions Dysplastic cerebellar gangliocytoma is a rare, benign tumor, having certain specific imaging features. Early diagnosis and surgical treatment is necessary, and it should be differentiated from gangliocytoma, ganglioglioma and low-grade astrocytoma. The systemic examination for patients with dysplastic cerebellar gangliocytoma and their family members is needed for the detection of pathological changes in other organs or other tumors.

DOI: 10.3969/j.issn.1672-6731.2017.04.008