Acute neurological deterioration as a result of two synchronous hemorrhagic spinal ependymomas

Abstract

Background:Ependymomas are the most common intramedullary tumors in adults and are the most common in mid-adult years. The presence of synchronous ependymomas in different sites of the spine is not common and it is even more infrequent to find hemorrhage from a spinal ependymoma as a cause of neurological deterioration.Case Description:A 32-year-old man presented with back pain and progressive paraparesia. Magnetic resonance (MR) showed two intradural extramedullary lesions on spinal canal with signs of acute hemorrhage. The patient underwent emergent surgical decompression and resection. Pathology revealed myxopapillary ependymomas.Conclusion:To our knowledge, we report the first case of a patient with acute neurological deterioration as a consequence of synchronous bleeding of two spinal ependymomas located at different levels in the spinal cord. This study illustrates the importance of recognizing the rare, but known occurrence of acute neurological deterioration after spontaneous hemorrhage in spinal ependymomas.

Atypical Imaging of Hemorrhagic Lumbosacral Myxopapillary Ependymoma with Histopathological Correlation: A Case Report

“…Spontaneous hemorrhagic MPE is rare. To date, only 14 case reports describing acute neurological compromise after hemorrhage of spinal MPEs have been published in the English medical literature [ 8 , 9 ]. In our case, the MPE was hemorrhagic and located at the L5–S2 level.…”

“…Among the previously published case reports, only 1 case report described a hemorrhagic MPE at the lumbosacral level [ 4 , 10 ]. MPE is a highly vascular tumor, and it has the highest risk of intratumoral bleeding relative to the other subtypes of ependymal tumors [ 9 , 11 ]. Multiple theories have been proposed to explain the tendency of an MPE to bleed [ 8 , 9 ].…”

Spontaneous Rupture and Hemorrhage of Myxopapillary Ependymoma of the Filum Terminale: A Case Report and Literature Review

“…Myxopapillary ependymoma is defined as a slowgrowing glioma and is considered a distinct variant of ependymomas classified as World Health Organization grade 1 (1-4). More than 50% of myxopapillary ependymomas are located in the conus medullaris or filum terminale (5,6), and the majority of myxopapillary ependymomas exhibit an insidious presentation with a gradual onset of symptoms and a prolonged subjective history, although clinical manifestations are frequently nonspecific (7,8). Therefore, it is rare for a patient with myxopapillary ependymoma to present with acute neurological decline after spontaneous hemorrhage, even if the myxopapillary ependymoma has a more vascular architecture than the other types of ependymomas (9)(10)(11).…”

“…We presented here a rare case of a 26-year-old woman with a ruptured myxopapillary ependymoma and accompanying hemorrhage that expanded from the L1 body to the L2-3 disc level. A review of the English literature revealed only 12 reports describing 15 cases of acute neurological decline caused by hemorrhage from myxopapillary ependymoma of the filum terminale or conus medullaris (Table 1) (7,8,(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). Clear descriptions of the tumors or MRI findings were documented in only 8 of these cases.…”

“…Its preferential manifestation is in young adults with an annual incidence of less than 0.01 per 100,000 (1)(2)(3)(4). The majority of myxopapillary ependymomas are found in the conus medullaris or filum terminale (5,6) and tend to have an insidious presentation with a gradual onset of symptoms and prolonged history (7,8). Therefore, it is rare for myxopapillary ependymoma to present with acute neurological deterioration secondary to rupture and hemorrhage.…”