Third ventricle liponeurocytoma: one case report and review of literature

Abstract

Objective To study clinicopathological features, diagnosis, differential diagnosis and prognosis of central nervous system (CNS) liponeurocytoma outside the cerebellum.  Methods One case of the third ventricle liponeurocytoma was reported focusing on the following aspects: clinical manifestations, histopathological features and immunophenotypes, and the relevant literatures were reviewed.  Results A 52-year-old female presented headache, and her head CT scan showed that there was a low-density lesion area at the former of the brain midline. The tumor was detected in the third ventricle during surgery. The size of tumor was 5.80 cm × 4.00 cm × 3.80 cm. Under optical microscopy, the tumor was biphasic in appearance, which consisted isomorphic small neuronal cells and mature adipose cells and adipose-like cells. Mature osseous tissue and intensive areas of calcification could also be seen. There was no necrosis or mitosis. By using immunohistochemical staining, the tumor cells were diffusely positive for S-100 protein (S-100) and β-catenin. A part of tumor cells were positive for neuronal nuclear antigen (NeuN), synaptophysin (Syn), murine double minute 2 (MDM2) and P53. Besides, the tumor cells were negative for glial fibrillary acidic protein (GFAP), oligodendrocyte transcription factor-2 (Oligo-2), isocitrate dehydrogenase 1 (IDH1), desmin (Des), CD68 and myelin basic protein (MBP). Ki-67 labeling index was about 1%.  Conclusions Central nervous system liponeurocytoma is a rare tumor, which is predominantly located in the cerebellar hemispheres, and those located out of cerebellum are much more seldom. Definite diagnosis could be made by typical histopathological characteristics and immunohistochemical expressions. Central nervous system liponeurocytoma has a very well prognosis. Most patients can survive over 5 years, and the longest sugvival is more than 18 years.

doi: 10.3969/j.issn.1672-6731.2014.09.012