Cerebellar liponeurocytoma in two siblings suggests a possible familial predisposition. (October 2016)

• Record Type: Journal Article
• Authors: Pikis, Stylianos; Fellig, Yakov; Margolin, Emil
• Is Part Of: Journal of clinical neuroscience. Volume 32(2016:Oct.)
• Page Start: 154
• Page End: 156
• Publication Date: 2016-10
• Subjects: Biology -- Cerebellar liponeurocytomas -- Familial -- Inheritance; Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2016.04.004
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: The molecular biology and genetic origin of cerebellar liponeurocytomas is yet to be defined. Cerebellar liponeurocytomas afflicting multiple members of the same family has been recently reported. The existence of a germline mutation predisposing only to cerebellar liponeurocytoma has been recently suggested. Abstract: There is limited data on the genetic origin and natural history of cerebellar liponeurocytoma. To the best of our knowledge there has been only one report of a familial presentation of this rare entity. We report a 72-year-old female with a posterior fossa tumor presenting with progressive cerebellar signs and symptoms. The patient underwent total tumor resection via an uncomplicated sub-occipital craniotomy. Histopathologic examination was diagnostic for cerebellar liponeurocytoma. Her sister was previously treated for a similar tumor. Our report provides further evidence for the possible existence of a hereditary abnormality predisposing afflicted families to cerebellar liponeurocytoma development.