Abstract. Cerebellar liponeurocytoma is rare, and the clinical characteristics and treatment strategy remain unclear. In the present study, a case of cerebellar liponeurocytoma was retrospectively reported and a literature review was performed. A 45-year-old female presented due to occipital headaches, exhibiting a hoarse voice and a broad-based gait. Pre-operative magnetic resonance images revealed a lesion occupying the right hemisphere of the cerebellum and the inferior vermis, compressing the medulla oblongata from the right side, and extending through the foramen magnum to the C2 level. A total resection was performed, and pathological analysis of the lesion showed positivity for synaptophysin, S-100 and neuronal nuclear antigen, partial positivity for Olig-2, and negativity for glial fibrillary acidic protein and epithelial membrane antigen. In addition, the Ki-67 index was low (<5%). Thus, a diagnosis of cerebellar liponeurocytoma was determined. Total resection was successful and the patient was followed up closely. A review of the literature showed that cerebellar liponeurocytoma is mainly located in the cerebellum, with rare extra-cerebellar cases. Certain studies have suggested that the tumor may be located supratentorially and subtentorially, and should be renamed as solely liponeurocytoma. Total resection of the tumor contributes to an improved prognosis, while a subtotal resection and high Ki-67 index lead to recurrence. The tumor is similar to a tumor of low malignancy, with long-term recurrence. Radiation is recommended when there is residual tumor, recurrence or when the Ki-67 is high.

Abstract. Familial skull base chordoma is a rare tumor derived from the remnants of the embryonic notochord. The present study describes the clinical presentation of 4 cases of skull base chordomas in a family. A 15-year-old female received staged surgeries and was pathologically confirmed with a diagnosis of skull base chordoma. Among the patient's family, 2 members had previously undergone surgery and were pathologically confirmed with chordomas; 1 family member had also received radiation therapy. Furthermore, the patient's cousin, an 18-year-old male, was confirmed to have this condition by epipharyngoscopy. All confirmed cases within the family remained alive with the condition. A literature review of familial chordoma was undertaken and 8 chordoma pedigrees were found. Familial chordoma was rare, with an estimated rate of 0.4% in all chordomas. The skull base was the predominant location for familial chordoma. Compared with sporadic chordoma, familial chordomas were diagnosed at a younger age. The brachyury gene was strongly associated with familial chordomas, however, the exact pathogenesis and genetics mechanisms remains unclear.