Cauda equina hemangioblastoma: case report

Hemangioblastoma da cauda equina: relato de caso

Cauda equina hemangioblastoma: case report

Hemangioblastoma da cauda equina: relato de caso

Leodante Batista da Costa Jr^I; Agustinho de Andrade^II; Bruno Perocco Braga^III; Carlos Alberto Ribeiro^IV

^IAssistant Neurosurgeon, Benjamin Guimarães Foundation, Belo Horizonte MG, Brazil

^IIChief of Neurosurgery, Belo Horizonte Hospital

^IIIResident of Neurosurgery, Benjamin Guimarães Foundation

^IVProfessor of Pathology, Federal University of Minas Gerais Medical School (UFMG), Belo Horizonte MG, Brazil

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.

Keywords: cauda eqüina, hemangioblastoma, Von Hippel-Lindau syndrome.

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 Hippel-Lindau, 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^1-6.

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.

CASE

A 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 pos-operative period she showed a little worsening in the weakness of the left quadriceps, which improved over time. Six months after surgery she was asymptomatic, and showed no neurologic deficit. Histopathological examination demonstrated hemangioblastoma (Fig 3). Von Hippel-Lindau clinical screening was negative.

DISCUSSION

Spinal cord tumors account for about 15% of all central nervous system neoplasms, and only about 10% of these are found in the cauda equina. Primary tumors of the cauda equina are rare lesions. Most commonly, they are ependymomas and schwannomas. Hemangioblastomas account for 1.6% to 2.1% of all spinal cord tumors. The majority of these lesions are intramedullary, but hemangioblastomas have been described in all spinal levels and compartments, with cervical and thoracic lesions predominating. Extramedullary disease is distinctly uncommon, representing approximately one fifth of all spinal hemangioblastomas^1,2,6,7.

Histologically, hemangioblastomas are benign lesions, partly cystic, characterized by stromal cells containing foamy cytoplasm in endothelium-lined vascular channels. The central nervous system lesions are located mainly in the retina and cerebellum; less often they occur in the medulla and spinal cord, and very seldom supratentorially. Medullary and spinal cord lesions are more commom in patients with VHL disease, and sporadic lesions are mostly cerebellar. Von Hippel-Lindau disease is an heredofamilial autosomal dominant disease with incomplete penetrance, characterized by intracranial and intraspinal hemangioblastoma, often multiple, retinal hemangioblastoma, cystic lesions in the kidneys, liver, pancreas and epididymis, benign and malignant renal cell tumors. The vascular tumor may be the sole manifestation of this syndrome⁸. Althought histologically benign, in the central nervous system these lesions may be devastating, especially if presenting in the posterior fossa or intramedullary.

In our review, we found only three cases of intradural hemangioblastomas of the nerve root in patients without the stigmata of VHL syndrome, and only one was documented with magnetic ressonance imaging, as ours own^1,4,9. Some authors recommend the superseletive spinal angiography to distinguish between intradural hemangioblastoma and spinal arteriovenous fistulae¹, but in this case, the MRI was quite sufficient for surgical planning. MRI showed a small nodular lesion, attached to lumbar root, hyperintense in T1 and isointense in T2, markedly enhanced with gadolinium infusion. This is not the typical imaging finding in intracranial lesions, that usually appears as cystic lesions with enhancing intramural nodules. Nevertheless we agree that angiography could be very usefull in planning the surgical management of these lesions. Myelography could also suggest the diagnosis, showing the serpiginous vasculature and the mass interfering with the contrast filling, if MRI and angiography are not available.

The treatment of choice for these tumors is total surgical resection, which, if accomplished, is usually curative. At surgery, intralesional debulking should not be performed. These tumors should be dissected and removed en bloc, once the intralesional resection, even with the smaller lesions, will be associated with profuse bleeding^1,2,5,7,10.

Received 23 October 2002, received in final form 13 December 2002

Accepted 6 January 2003

Dr. Leodante Batista da Costa Jr - Rua Três Corações 13/302 - 30480-110 Belo Horizonte MG - Brasil. FAX: [phone]. E-mail: [e-mail]

• 1. Brisman J L, Borges L F, Ogivly C S. Extramedullary hemangioblastoma of the conus medullaris. Acta Neurochirur(Wien) 2000;142:1059-1062 .
• 2. Tibbs R E Jr, Harkey L, Raila F A. Hemangioblastoma of the filum terminale: case report. Neurosurgery 1999;44:221-226.
• 3. Wolbers JG, Ponssen H, Kamphorst W. Hemangioblastoma of the cauda equina. Clin Neurol Neurosurg 1985;87:5559.
• 4. Chazono M, Shiba R, Funasaki H, Soshi S, Hattori A, Fujii K. Hemangioblastoma of the L-5 nerve root - case illustration. J Neurosurg (Spine1) 1999;90:160.
• 5. Roonprapunt C, Silvera VM, Setton A, Freed D, Epstein FJ, Jallo GI. Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery 2001;49:321-328.
• 6. Browne TR, Adams RD, Roberson GH. Hemangioblastoma of the spinal cord: review and report of five cases. Arch Neurol 1976;33:435-441.
• 7. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001;48:55-62.
• 8. Burger P C, Scheithauer BW. Tumor of the central nervous system atlas of tumor pathology, Fasc 10 Washington: AFIP, 1993:239-243.
• 9. Raghavan R, Krumerman J, Rushing E J, et al. Recurrent (non-familial) hemangioblastomas involving spinal nerve roots: case report. Neurosurgery 2000;47:1443-1452.
• 10. Yasargil MG, Antic J, Laciga R, de Preux J, Fideler RW, Boone SC. The microsurgical removal of intramedullary spinal hemangioblastomas: report of twelve cases and a review of the literature. Surg Neurol 1976;6:141-148.

Publication Dates

• Publication in this collection
  28 July 2003
• Date of issue
  June 2003

History

• Accepted
  06 Jan 2003
• Received
  13 Dec 2002