Abstract
INTRACRANIAL MALIGNANT PERIPHERAL NERVE SHEATH TUMOUR IN THE CEREBELLO-PONTINE ANGLE – A RARE CASE REPORT AND REVIEW OF LITERATURE

*Dr. Moumita Paul, Prof (Dr.) Mouchumee Bhattacharyya, Prof (Dr.) Apurba Kumar Kalita

ABSTRACT

Introduction: Malignant peripheral nerve sheath tumours (MPNSTs) are soft tissue sarcomas derived from peripheral nerves. MPNSTs arising in the intracranial cavity is rare and that occurring in the cerebello-pontine (CP) angle is even more sporadic. The diagnosis is primarily based on conventional pathology and immunohistochemistry. The prognosis of these tumours is extremely poor. MPNSTs are typically treated with surgery and adjuvant radiotherapy and chemotherapy should be considered in cases of incomplete resection. Here, we present a case of a 55 years old male with Intracranial Malignant Peripheral Nerve Sheath Tumour in the left Cerebello-pontine angle. Case report: A 55 year old male presented with history of decreased hearing which worsened for the last 2 years alongwith tinnitus and dizziness. On examination, VIIth and VIIIth cranial nerve deficits were found. MRI Brain showed a mixed hyper and hypointense space occupying lesion in the left cerebello-pontine angle. Ventriculo- peritonial shunt was done which was followed by left retromastoid craniotomy and debulking of left CP angle lesion. Post-operative HPE showed Spindle cell sarcoma suggestive of malignant peripheral nerve sheath tumour. IHC showed S-100 positive. He was then taken up for adjuvant radiotherapy (60 Gy in 30#) with conformal technique. Post-radiotherapy, his symptoms started improving. He was on follow-up but after 6 months he started developing same symptoms. He was advised radiological investigation subsequently but unfortunately the patient expired before undergoing that. Conclusion: Because of poor prognosis and high chances of recurrence, close follow up is crucial in such tumours. Complete resection in these tumours is quite difficult because of close proximity with critical brain structures. The median survival of patients with intracranial MPNSTs is hardly a year. Studies with large number of patients will be required to ascertain the role and regimen of chemotherapy to be used in these tumours.

Keywords: Malignant peripheral nerve sheath tumour, Intracranial malignant peripheral nerve sheath tumour, Cerebello-pontine angle, Cranial nerve deficits, Radiotherapy.


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