Intradural extramedullary cavernous hemangioma of the cervicothoracic junction: A case report and review of the literature

Abstract

Background: Intradural extramedullary cavernous hemangiomas of the spine are rare, benign lesions with only 40 published cases to date. Case Description: The authors report a rare case of a histologically diagnosed intradural extramedullary cavernous hemangioma of the spine involving the cervicothoracic junction and causing sudden gait disturbances and urinary retention in a 24-year-old male. Gross total tumor removal allowed complete spinal decompression and sensible improvement of the clinical condition with no evidence of tumor relapse at 12-month follow-up examination. Conclusion: More frequently found in the lower thoracic and lumbar spine, these tumors often cause subtle clinical manifestations including sensory and motor dysfunction secondary to nerve root compression; nonetheless, occasional cases of rapidly progressive worsening of the neurological condition with evidence of myelopathy and autonomic dysfunction have been described. In such cases, urgent surgical resection is crucial since the degree of neurological impairment and the time spanned from the onset of the symptoms are paramount for a good recovery.

Spinal intradural extramedullary cavernoma: A case report

“…The majority of spinal cavernomas arise from the vertebral body or the epidural spaces [ 7 ]. However, only 3 % of spinal cavernomas are located intraduraly and only a fraction of intradural spinal cavernomas are extramedullary as only 40 cases have been reported in the literature [ 4 ].…”

“…The pathogenesis of these uncommon lesions remains unknown [ 4 ]. None the less, some studies have suggested that these lesions can occur as a result of impaired migration and differentiation of the fetal mesoderm from the embryonic mesodermal plate during angioblastic differentiation [ 5 ].…”

“…The most common symptoms of spinal cavernomas are back pain accompanied by signs of progressive nerve root compression; however, sudden onset myelopathy and autonomic dysfunction accompanied by malaise were also reported [ 4 ].…”

“…Only 3 % of spinal cavernomas are located intraduraly [ 4 , 5 ] and only 40 cases of intradural spinal cavernomas are reported to be extramedullary [ 1 , 4 ]. Here we report a case of an intradural extramedullary cavernoma which is located at the T8-T9 level making it the 41st reported case in the literature.…”

Cervical intra and extramedullary hemangioblastoma with associated syringomyelia: A case report and review of the literature

“…They can arise as an isolated lesion or as multiple tumors spread throughout the central nervous system in association with syringomyelia, intramedullary hemorrhages, or Von Hippel-Lindau (VHL) syndrome (in 20–30% of the cases). [ 2 , 4 ] Clinically, they contribute to varying degrees of myelopathy that correlate with the location of the tumor mass and accompanying abnormalities. Here, we describe a 32-year-old female with a cervical, intra- and extra-medullary HB extending from the obex to C2 resulting in headaches and numbness in the upper extremities.…”