Liponeurocytoma of the Cerebellopontine Angle

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Carmela Chiaramonte, Sylvain Rabaste, Timothee Jacquesson, David Meyronet, François Cotton, Emmanuel Jouanneau, Moncef Berhouma
BackgroundLiponeurocytoma is a very rare tumor classified as grade II (neuronal and mixed neuronal-glial tumors) according to 2016 World Health Organization classification of tumors of the central nervous system. The median age at detection is 50 years, and the most frequent location is the posterior cranial fossa, especially within the cerebellar hemispheres; liponeurocytomas arising in the cerebellopontine angle (CPA) are exceptional.Case DescriptionHere we report the clinical, radiological, and pathological characteristics of a CPA liponeurocytoma in a 35-year-old woman, as well as a review of the literature. This unusual cisternal location raises the issue of the differential imaging diagnosis with much more common CPA tumors (e.g., meningiomas, vestibular schwannomas, ependymomas, epidermoid cyst, hemangioblastomas, medulloblastomas).ConclusionTo the best of our knowledge, 59 cases of cerebellar liponeurocytomas have been reported to date, which include only 6 cases of CPA liponeurocytomas. Treatment relies on total removal whenever possible, with an excellent prognosis, but a high MIB-1 index (>10%) and/or incomplete tumor resection are the main adverse prognostic factors.



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