Intradural Extramedullary Capillary Hemangioma in the Upper Thoracic Spine: A Review of the Literature

Abstract

Capillary hemangiomas are benign tumors found in the skin and soft tissues in younger people. They occur in the central nervous system only rarely, and intradural occurrence is extremely rare. We report here a 60-year-old man presenting with thoracic girdle pain and progressive gait disturbance. Magnetic resonance images of the thoracic spine showed a 12 × 8 × 20 mm, well-defined intradural mass at the T2 level, compressing the spinal cord laterally. Relative to the spinal cord, the mass was hypo- to isointense on T1-weighted images and relatively hyperintense on T2-weighted images, with strong enhancement on contrast-enhanced T1-weighted images. The patient underwent T1-2 hemilaminectomy with resection of the intradural extramedullary tumor, which showed characteristics of a capillary hemangioma on histologic examination. The patient's symptoms improved following the surgery and no clinical or radiological evidence of recurrence was noted at the 2-year follow-up. We present this case with a review of the literature, highlighting features for differential diagnosis.

Intradural Extramedullary Capillary Hemangioma In the Upper Thoracic Spine with Simultaneous Extensive Arachnoiditis

“…Among these tumors, schwannoma and meningioma are the most common pathologies developing at thoracic spine, and show similar MR imaging features with CH21). Especially, CH arises from the inner surface of the dura mater, and it reveal similar feature such as a dural tail sign4,21). There are difficulties to differentiate CH from the other IDEM tumors, and still are risks of misdiagnosis1,9).…”

“…CH as intradural extramedullary (IDEM) tumor in spine is extremely rare and difficult to differentiate with other common pathologies10,21). Despite of the low incidence, prompt diagnosis and complete resection area important, because acute hemorrhage leading to sudden neurologic deterioration can be occurred17).…”

Intradural spinal, extramedullary, T4-T5 cavernous hemangioma - case report

“…Spinal cavernous hemangioma (1-22) can be found in several topographic locations: confined to the vertebrae, extended epidurally from the vertebral lesions, entirely extradural lesions with no bone involvement, and also purely intradural, extramedullary from the inner surface of the dura or the pial surface of the spinal cord; very rarely, intramedullary in about 3% of cases (21)(24), adherent to the blood vessels of the nerve roots in the cauda equina (16). Intradural spinal, extramedullary, cavernous hemangiomas are most frequently found in the adult population (only 4 pediatric cases cited in literature), mostly in women (23), in the thoracic spine -80% of cases (9)(12) or lower thoraco-lumbar region (13) (20), with posterior location within the spinal canal in 93% of cases; also with possible lateral recesses extensions; less common in cervical location (7)(8) and mostly adherent to the nerve root or spinal cord (8) (24).…”

“…Intradural spinal, extramedullary, cavernous hemangiomas are most frequently found in the adult population (only 4 pediatric cases cited in literature), mostly in women (23), in the thoracic spine -80% of cases (9)(12) or lower thoraco-lumbar region (13) (20), with posterior location within the spinal canal in 93% of cases; also with possible lateral recesses extensions; less common in cervical location (7)(8) and mostly adherent to the nerve root or spinal cord (8) (24). Capillary hemangiomas are found in the skin and soft tissues in younger people, and rarely occur in the central nervous system; more frequent they are located around or attached to nerve roots of cauda equina and conus medullaris (25), extremely rarely intradurally (16) 16,25) Clinical symptomatology at presentation is variable, depending on the size and topography, with the more frequent symptoms beeing: acute spinal pain, radiculopathy and/or myelopathy, progressive gait disturbance, slowly progressive paraparesis, or even asymptomatic -very rare, as in our case (5,18,(26)(27)(28). Cavernous hemangiomas may present in four major clinical patterns: acute episodes of step wise deterioration, slow progression, acute onset with rapid deterioration, and acute onset with gradual decline (10,12,14,15,18).…”

“…Either capillary or cavernous, intradural, spinal, extramedullary hemangiomas can be solitary or multiple; they are well-defined lesions composed of blood-filled endothelial spaces lined by thickened, hyalinised walls without elastic fibers or smooth muscle; they are also very rare (8)(9)(10)(11)(12)(13)(14)(15)(16). We report an intra dural spinal extramedullary, T4-T5 cavernoma in a 56-years-old woman, who underwent microsurgical resection.…”