Cerebellar Liponeurocytoma with an Unusually Aggressive Clinical Course: Case Report

Author:

Jenkinson Michael David1,Bosma Jark Jan Daniel1,Du Plessis Daniel2,Ohgaki Hiroko3,Kleihues Paul3,Warnke Peter14,Rainov Nikolai G.14

Affiliation:

1. Department of Neurosurgery, The Walton Centre for Neurology and Neurosurgery, National Health Service Trust, Liverpool, England

2. Department of Neuropathology, The Walton Centre for Neurology and Neurosurgery, National Health Service Trust, Liverpool, England

3. International Agency for Research on Cancer, Lyon, France

4. Department of Neurological Science, University of Liverpool, Liverpool, England

Abstract

Abstract OBJECTIVE AND IMPORTANCE Liponeurocytomas are rare cerebellar neoplasms in adults, with benign histological features and a favorable clinical prognosis. Current clinical opinion is based on a total of less than 20 published cases and suggests that gross total resection and long-term follow-up monitoring, with possible additional surgery and radiotherapy for treatment of recurrent tumors, represent the best treatment approach for this relatively benign tumor type. CLINICAL PRESENTATION A 51-year-old Caucasian woman presented with worsening unsteady gait and headaches, suggesting increased intracranial pressure. INTERVENTION The patient underwent subtotal resection of a cerebellar liponeurocytoma, followed by fractionated radiotherapy (total dose of 54 Gy). She experienced a local recurrence of the tumor 12 months later and underwent additional surgery for removal of the cerebellar mass. A second recurrent tumor was diagnosed on magnetic resonance imaging scans 3 months later and was surgically resected. The tumor histological findings were consistently devoid of atypical features, apart from leptomeningeal invasion noted in the first surgical specimen. CONCLUSION This unusual case demonstrated an atypical clinical course of a highly aggressive and radiation-resistant tumor, despite the consistent absence of aggressive histological features. Cerebellar liponeurocytomas may not be as benign as the current literature and typical low-grade cytological and histological features suggest.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Clinical Neurology,Surgery

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