Innv.19. Anaplastic cerebellar ependymoma in an adult female presenting with tonsillar herniation successfully treated with chemotherapy : a case report

Publication Date

2020

Document Type

Article

Publication Title

Neuro-Oncology

Abstract

INTRODUCTION

Ependymomas are slow-growing neuroectodermal tumors that may arise from various parts of the central nervous system. Anaplastic ependymoma represents 3–5% of ependymomas and is rarely found in the cerebellum in adults. In this paper, we discuss a case of an adult female with anaplastic cerebellar ependymoma who underwent surgery and chemotherapy for tonsillar herniation.

CASE PRESENTATION

This is a case of a 58-year-old Filipino female with a five-month history of dizziness, headache, nausea and vomiting. Cranial computed tomography (CT) scan revealed presence of hydrocephalus with enhancing lesions at the right cerebellum. The patient underwent ventriculoperitoneal shunting (VPS) with sub-total excision and biopsy of the right cerebellum. Histology and immunochemistry were consistent with a high-grade anaplastic ependymoma (WHO Grade III). Cerebrospinal fluid and spinal magnetic resonance imaging (MRI) were all negative for tumor. The initial plan was to undergo limited field external beam radiation therapy to the cerebellum, however the patient was lost to follow-up. Two months after surgery, she presented with similar symptoms and MRI revealed tonsillar herniation and recurrence of mass compressing the fourth ventricle, pons, and medulla oblongata, thus medical decompression and urgent chemotherapy with cisplatin and etoposide was started. After 4 sessions of chemotherapy, repeat cranial MRI revealed resolution of tonsillar herniation and interval regression of the mass.

CONCLUSION

This paper presented a rare case of anaplastic cerebellar ependymoma with tonsillar herniation who was successfully treated with chemotherapy. Radiotherapy is the standard of care following surgical resection, but the management of our case showed that chemotherapy is also effective in cases when the presentation is herniation.

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