Cervicomedullary ependymoma with hemorrhage: A case report and review of literature

Abstract

Ependymoma is a rare tumor central nervous system that arises from the ependymal lining of the ventricles or the central canal of the spinal cord. They are of neuroectodermal in origin and constitute about 30%–86% of tumors arising in the spinal cord. The occurrence of these tumors in the cervicomedullary region is very rare. Sudden symptomatic neurologic presentations due to hemorrhage in cervicomedullary ependymoma is very rare and so far have never been reported. Mostly presenting as neurologic deficits involving limbs, these tumors pose a technical challenge in their removal. We present a patient who presented with sudden-onset dysesthesia of the upper and lower limbs. On imaging, he was found to have a cystic medullary tumor extending to the cervical region with hemorrhage. We discuss the epidemiology, surgical challenges, and outcome along with review of literature of these rare tumors located in this precarious location.

Ependymoma Presenting as a ­Rim-Enhancing Lesion in the Brainstem

“…They rarely invade or arise within the brainstem parenchyma [3]. There are multiple reports of patients with intramedullary cervicomedullary ependymomas in children and adults [6-9]. However, for ependymomas isolated to the brainstem, there have only been a total of 7 cases previously reported [10-16].…”

Treatment strategy for multisegmental cervicomedullary ependymoma: illustrative case

“…In cases of predominant spread of the tumor along the cervical spinal cord, symptoms include signs of cervical myelopathy. 2 , 4 , 5 Local neck pain is also very common, and hypertension symptoms are less common. The patient in this case had significant neurological deficits, spinal fragments of the tumor prevailed, and motor disorders of the peripheral type in the proximal parts of the arms dominated.…”

“…Acute occurrence or increase in symptoms that could indicate a hemorrhage in the tumor is not typical for CME. However, Kutty et al 5 described a clinical case of a patient with an acute onset of the disease in the form of sudden dysesthesia in the arms and legs in which MRI of the brain and cervical spinal cord showed a solid cystic CME with signs of hemorrhage.…”

“…Several studies have shown that total removal of these tumors is the most significant factor associated with a favorable prognosis of the disease. 2 , 4 , 5 In the case of a high risk of neurological deficit with dense adhesion of the tumor to the brain and with significant deterioration in IONM parameters, subtotal removal of the tumor is preferable to prevent the increase in neurological symptoms in the postoperative period. 2 , 5 , 11 In our case, there was an intense adhesion of the rostral fragment of the tumor more to the right.…”

“… 2 , 4 The prevalence of CME is relatively low: 3.8–10.0% of all spinal cord ependymomas and up to 23.7% of all cervicomedullary tumors. 5–7 CME constitutes a complex surgical pathology, especially in patients with multisegmental distribution in the spinal cord.…”