Cauda equina hemangioblastoma: case report

Abstract

-Hemangioblastomas of the spinal cord are rare lesions, and those located at the cauda equina are even rarer. Most commonly these tumors are present in patients with von Hippel-Lindau (VHL) syndrome. We describe here the case of a 48 years old woman with a pure radicular hemangioblastoma, not associated with VHL, presenting with radicular pain, diagnosed with magnetic ressonance imaging (MRI) and submitted to total resection with a very good outcome. To our knownledge, this is the second report to describe the MRI aspect of histologically proved hemangioblastoma of the cauda equina in a patient without clinical criteria for VHL.KEY WORDS: cauda eqüina, hemangioblastoma, Von Hippel-Lindau syndrome.Hemangioblastoma da cauda equina: relato de caso Hemangioblastoma da cauda equina: relato de caso Hemangioblastoma da cauda equina: relato de caso Hemangioblastoma da cauda equina: relato de caso Hemangioblastoma da cauda equina: relato de caso RESUMO -Hemangioblastomas da medula espinhal são lesões raras, sendo sua localização na cauda equina ainda mais incomum. Estes tumores são diagnosticados com mais frequência em pacientes portadores da síndrome de Von Hippel-Lindau. Descrevemos o caso mulher de 48 anos hemangioblastoma radicular na cauda equina, diagnosticado com ressonância nuclear magnética, não associado à síndrome de Von HippelLindau, tratado cirurgicamente com ótimo resultado. Em nossa revisão, este é o segundo relato de diagnóstico com ressonância nuclear magnética de hemangioblastoma da cauda equina em paciente sem critérios clínicos da síndrome de Von Hipppel-Lindau. PALAVRAS-CHAVE: cauda equina, hemangioblastoma, síndrome Von Hippel-Lindau. Hemangioblastomas represents about 1.6 to 2.1% of all spinal cord tumors. Extramedullary-intradural hemangioblastomas are rarer, representing only one fifth of all spinal hemangioblastomas. One recent report suggested that the number of reported cases of hemangioblastoma of the cauda equina may be as low as 40. Most commonly, these lesions are associated with VHL disease, an heredo-familial autosomal dominant genetic disease with incomplete penetrance [1][2][3][4][5][6] . We relate here a case of an hemangioblastoma of the cauda equina, diagnosed in a patient without clinical criteria to VHL disease, and show the aspects of MRI imaging of this rare disease. CASEA 40-years-old woman presented with left leg paresthesia for twenty days prior to hospital admission. Her physical examination showed a discrete weakness for left leg extension, patelar arreflexia and hypoestesia in the medial side of the same leg. The CT scan and eletromyography were unremarkable, and MRI showed an intradural, hyperintense lesion, with brightly enhancing after gadolinium infusion, at the aproximated level of the L2-L3 disc space (Figs 1 and 2). The patient was submitted to a L2-L3 laminectomy and complete ressection of a reddish-brown, highly vascular lesion. The tumor was attached to some radiculae of the fourth left lumbar root, but a total removal was acomplished. In the immediate p...

Intradural extramedullary hemangioblastoma

“…Browne et al 8 and Yasargil et al 12 reported an incidence of 21.3% and 25.3%, respectively, for this type of presentation of this tumor. In 2003, Costa Jr et al 15 described an exceptional case of cauda equina hemangioblastoma diagnosed by MRI, in a 48-years-old woman without VHL, who presented with paresthesia and discreet weakness in the left leg.…”

Cavernous angioma of the cauda equina: case report

“…Primary tumors of the filum terminale and conus medullaris, or both, comprise only 6% of spinal cord tumors 2 . Only 6.2% to 7.5% of all intraspinal tumors are vascular 3,4 .The cavernous angioma rarely occurs in the cauda equina. The literature reports eleven cases [5][6][7][8][9][10][11][12][13][14] .…”

Hemangioblastoma of the filum terminale

“…6 Extramedullary-intradural hemangioblastomas of the nerve roots are rare and represent one fifth of all spinal hemangiobalstomas. [7][8][9][10][11][12][13][14][15][16][17] Most of these are associated with von Hippel-Lindau disease. Michaud et al 18 reported a 6-month-old infant with a spinal cord hemangioblastoma located in the conus medullaris in association with an overlying congenital dermal sinus and cutaneous capillary hemangioma.…”