Reappraisal of cerebellopontine angle medulloblastomas: Report of a fatal case and lessons learned

摘要

CPA Medulloblastomas are rare in adults. We describe a case of medulloblastoma in the right CPA in a middle aged male patient who presented with short history and was preoperatively diagnosed as vestibular schwannoma. Intra-operatively it turned out to be a highly vascular and malignant lesion. Histopathology demonstrated medulloblastoma with high ki67 index. Patient was re-explored for hematoma evacuation and remained vegetative postoperatively. This is a unique case considering the older age at presentation, short duration of symptoms with atypical presentation of only 7th, 8th and lower cranial nerve involvement. MRI showed a lobulated heterogeneous extra axial right CPA mass lesion extending in the right acoustic meatus and causing mass effect and effacement of the fourth ventricle with heterogeneous enhancement post-gadolinium, suggestive of vestibular schwannoma. The aggressive behaviour of this tumor with short history has to prompt one to keep less common lesions like medulloblastomas as differential diagnosis in the CPA region. Though medulloblastomas are rare lesions in the CPA they have to be considered in the differential diagnosis based on atypical clinical behaviour, a suspicion of such diagnosis preoperatively can alter the treatment strategy, focusing on tumor embolization, safe surgical excision and adjuvant chemoradiation, aiming towards a better quality of life, in the post-operative period. Highlights ? CPA medulloblastomas are rare in adults. ? Medulloblastomas are aggressive and have a short history of presentation. ? Medulloblastomas have to be considered in the differential diagnosis based on atypical clinical behaviour. ? Better planning based on suspicion, different treatment strategy.