The Extra-axial Cerebellopontine Angle Medulloblastoma in an Adult Patient: A Case Report and Review of Literature
Authors:
- Mohammad Mirahmadi EraghiMohammad Mirahmadi Eraghi
- Senior Independent Researcher MbaSMSenior Independent Researcher Mba
Open Access
- 28 December 2022
- journal article
- review article
- Published by Negah Scientific Publisher in Iranian Journal of Neurosurgery
- Vol. 8 (Continuous) , 19
Abstract
Background and Importance: Medulloblastoma (MB) is regarded as a scarce primary brain neoplasm in adulthood, originating from the fourth ventricle or the cerebellum, and cerebellopontine angle (CPA)-MB has been described less often in the literature. Few cases of CPA MB have been reported in the English-written literature, most of which are intra-axial, mainly in children; adults' extra-axial CPAMB is even scarcer. To the best of the authors’ knowledge, 12 cases of extra-axial CPA MBs have been reported in the English-written literature. Case Presentation: A 23-year-old man presented to our center complaining of a generalized pulsatile headache, imbalance, swallowing impairment, and right-sided hearing loss for the past 20 days. Computed tomography (CT) scan of the brain showed a hyper-dense extra-axial mass lesion (41*37mm) in the right CPA with a significant compression effect on the fourth ventricle, causing a three ventricular obstructive hydrocephalus. Magnetic resonance imaging (MRI) showed a well-defined heterogeneous extra-axial, lobulated, dural-based mass lesion in the right CPA, hypointense on the T1 sequence, and hyperintense on the T2 sequence compared with the adjacent parenchyma, which has a bright heterogeneous enhancement during gadolinium injection. A significant mass effect was observed on the adjacent parenchyma, brain stem, and fourth ventricle without evidence of parenchymal edema. The patient underwent emergent surgery the next day regarding the mass effect and hydrocephalus. On the postoperative examination in the intensive care unit, no new neurological deficit was detected, and the swallowing and gag reflex significantly improved. Conclusion: Though rare, clinical considerations, along with early supportive radiologic measures, should be considered in subjects with suspected MB. A total tumor excision approach followed by aggregative chemotherapy/radiotherapy is designed to hinder tumor relapse.Keywords
This publication has 26 references indexed in Scilit:
- Medulloblastomas in cerebellopontine angle: Epidemiology, clinical manifestations, imaging features, molecular analysis and surgical outcomeJournal of Clinical Neuroscience, 2019
- Updates on Management of Adult MedulloblastomaCurrent Treatment Options in Oncology, 2019
- MedulloblastomaNature Reviews Disease Primers, 2019
- Reappraisal of cerebellopontine angle medulloblastomas: Report of a fatal case and lessons learnedInterdisciplinary Neurosurgery, 2018
- Posterior fossa medulloblastoma in an atypical extra-axial location: A case reportRadiology Case Reports, 2018
- Maml1 acts cooperatively with Gli proteins to regulate sonic hedgehog signaling pathwayCell Death & Disease, 2017
- Cerebellopontine angle medulloblastoma with extensive nodularity in a child: case report and review of the literatureChild's Nervous System, 2016
- Subtypes of medulloblastoma have distinct developmental originsNature, 2010
- Adult medulloblastoma and the “dural-tail” sign: Rare mimic of a posterior petrous meningiomaClinical Neurology and Neurosurgery, 2009
- Uncommon presentation of medulloblastomaChild's Nervous System, 2001