Spontaneous Hemorrhage of a Spinal Ependymoma in the Filum Terminale Presenting with Acute Cauda Equina Syndrome: Case Report

Abstract

We present a rare case of spontaneous hemorrhage of a spinal ependymoma in the filum terminale presenting with acute cauda equina syndrome. A 16-year-old male presented with a sudden onset of severe back pain that began 10 days before hospitalization. This symptom progressed, followed by development of decreased sensation in the lower extremities, bladder dysfunction, and motor weakness that advanced to an inability to walk. Spinal magnetic resonance imaging revealed a hemorrhagic mass from Th12 to L2 and L4 to L5, and clinical signs indicated acute cauda equina compression. One day after admission to the hospital, emergency surgery was performed. A spinal tumor in the conus portion with a spinal subarachnoid hemorrhage was seen. Gross total excision of the massive hematoma mixed with the underlying tumor was performed. Pathological findings of the excised tumor demonstrated a WHO Grade II cellular ependymoma of the non-myxopapillary type. The patient made a significant recovery. The ability to walk was restored, and impaired bladder function improved at follow-up. Early diagnosis and suitable treatment are associated with a more favorable outcome.

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

“…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 ].…”

“…Furthermore, vascular insult has been suggested to be predisposed by abuse of anabolic steroids [ 8 ]. The average age of presentation has been reported as 34.9 years (range, 15–65 years), and the main presenting symptom is predominantly severe back pain [ 11 ]. Our patient was relatively young and physically active, with no history of any proposed risk factors, such as trauma, heavy lifting, or medication use.…”

“…In the majority of cases, RT is used for recurrence or subtotal tumor removal [ 1 ]. In a study conducted between 1972 and 2005 by Nakamura and his group, no tumor recurrence was found during a mean postoperative follow-up period of 10.4 years among 14 of 15 patients who underwent GTR followed by either whole brain and spinal cord radiation or local irradiation [ 11 , 15 ]. Feldman et al [ 16 ] found that the recurrence rate of MPE after GTR alone was 15.9%, which was similar to the recurrence rate for patients who underwent GTR with adjuvant RT.…”

“…Feldman et al [ 16 ] found that the recurrence rate of MPE after GTR alone was 15.9%, which was similar to the recurrence rate for patients who underwent GTR with adjuvant RT. However, the recurrence rate for patients who underwent subtotal resection was 35.1% versus 29.3% for patients who underwent subtotal resection with adjuvant RT [ 11 , 16 ]. A similar conclusion was found by Tsai and his group [ 17 ].…”

Freiburg Neuropathology Case Conference

“…Due to its rich mucin content it is almost always hyperintense on T2-weighted images and T2-hypointense margins of the tumor can indicate superficial siderosis. Myxopapillary ependymomas have the greatest bleeding tendency of all ependymoma subtypes and may appear with proliferated vessels subsequent to hypervascularity [4]. Usually MEs show avid contrast enhancement.…”