Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review

Intra-extradural Dumbbell-Shaped Hemangioblastoma Manifesting as Subarachnoid Hemorrhage in the Cauda Equina

“…11) Furthermore, the tumor usually affects only the dorsal root fascicles. 1,10,12,19) However, in our patient, the entire L2 nerve root was fanning out and completely encased by the tumor, and the tumor was tightly attached to the surrounding tissue due to bleeding at the onset. We were forced to resect the entire L2 nerve root to accomplish extracapsular removal, but the patient's postoperative neurological symptoms were minimal as in previous cases (Table 2), because the function of the fanning origin nerve might be compensated by other nerves during tumor growth, as occasionally experienced in schwannoma.…”

“…12) Intra-extradural dumbbell-shaped hemangioblastoma is extremely rare; previous reports of this tumor are listed in Table 2. 1,4,7,10,12,19,23,26) Hemangioblastomas are certainly a diagnostic challenge and may be preoperatively misdiagnosed as metastases, schwannomas, or neurofibromas, all of which should be treated by distinct strategies. It is imperative to identify hemangioblastomas preoperatively because these tumors tend to bleed significantly at surgery because of their vascular nature.…”

“…It is imperative to identify hemangioblastomas preoperatively because these tumors tend to bleed significantly at surgery because of their vascular nature. 10) Hemangioblastomas have an inhomogeneous appearance on T 2 -weighted MR imaging, reflecting the remarkable flow void resulting from the highly vascular mass. Intense enhancement also results from prominent tumor vessels with dilated feeding arteries and draining veins.…”

Spinal Hemangioblastoma of Cauda Equina Origin Not Associated With Von Hippel-Lindau Syndrome -Case Report-

“…8,14) Spinal hemangioblastoma is rare and accounts for 1-5% of all spinal cord tumors. 2,5,12,14,23) Spinal hemangioblastoma usually occurs in the cervical or thoracic spine, and located in the posterior aspect of the spinal cord, with subpial growth, often associated with formation of an intraspinal cyst.…”

“…Hemangioblastoma in the lumbosacral region is exceptional. 1,8,11,14) Analysis of 56 patients with central nervous system hemangioblastomas found that spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%). 20) Extramedullary hemangioblastomas account for only one fifth of spinal hemangioblastomas, 3) and are usually associated with VHL syndrome.…”