Abstract. Subtentorial ependymoma is a common central nervous system tumor in young children, but is uncommon in adults. Ependymoma often arises from the cells lining the fourth ventricle. The present study reports a rare case of primary ependymoma that originated from the cerebellopontine angle, with local extension to the two internal auditory canals and remote spinal metastasis, in an adult male. A 50-year-old male presented with headache, tinnitus and bilateral hearing loss that had persisted for 4 months. Magnetic resonance imaging (MRI) revealed a mass in each of the cerebellopontine angles, which had spread to each internal auditory canal and wrapped the VII/VIII cranial nerve complex. A gross total resection was performed to remove the mass in the right side. Histological examination confirmed that the tumor was a World Health Organization grade II papillary ependymoma. Notably, the patient complained of urine retention post-surgery and massive occupational lesions in T3-T4 and L5-S2 were found on full spinal cord MRI. The patient then received combination therapy consisting of temozolomide, and whole-brain and spinal cord radiation. In the final follow-up examination, performed 13 months after treatment, slight shrinkage of the T3 lesion was observed, and no progression of the left cerebellopontine angle and S5-L2 lesions were identified on MRI. In summary, although this clinical entity is rare, the diagnosis of ependymoma and the possibility of spinal cord metastasis should be considered in subtentorial tumors.
IntroductionSubtentorial ependymoma mainly occurs in young children and usually arises in the fourth ventricle (1). Although it is a relatively benign tumor, cerebrospinal fluid (CSF) spread is found in 8-33% of patients (2,3). Moreover, subtentorial ependymoma is more inclined to exhibit CSF metastasis compared with supratentorial ependymoma (4).Bilateral primary posterior fossa ependymoma originating from the cerebellopontine angle, termed primary cerebellopontine angle ependymoma, is a rare form of subtentorial ependymoma that predominantly occurs in infants and young children. The main symptom is headache due to progressively increasing intracranial pressure (5), while cranial nerve deficit is relatively uncommon. Surgical resection with subsequent radiotherapy is the primary treatment strategy for patients with ependymoma (6). The efficacy of conventional chemotherapy for this disease remains uncertain; however, temozolomide appears to be a promising adjuvant therapeutic approach for multifocal anaplastic ependymoma following surgical resection (7). The extent of surgical resection is the major determinant of overall survival in pediatric patients (8). However, the prognosis varies in different reports, with 5-year overall survival ranging between 50 and 64%, and progression-free survival ranging between 23 and 64% (5,9-11).The present study reports a rare case of primary cerebellopontine angle ependymoma extending to the internal auditory canals and spinal cord in an adult man. To the best...