Extra and intradural spinal Hemangioblastoma

Hemangioblastoma espinhal extra e intradural

Hemangioblastoma espinal extra e intradural

CASE REPORT RELATO DE CASO RELATO DE CASO

Extra and intradural spinal Hemangioblastoma

Hemangioblastoma espinhal extra e intradural

Hemangioblastoma espinal extra e intradural

Marcelo Campos Moraes Amato^I; Caio César Marconato Simões Matias^II; João Alberto Assirati Junior^III; Aline Paixão Becker^IV; Carlos Gilberto Carlotti Junior^V; Benedicto Oscar Colli^VI

^INeurocirurgião. Doutorando da Faculdade de Medicina de Ribeirão Preto da USP – Ribeirão Preto, SP, Brasil

^IIMédico Residente de Neurocirurgia do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da USP - Ribeirão Preto, SP, Brasil

^IIINeurocirurgião. Doutor. Médico Assistente do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da USP - Ribeirão Preto, SP, Brasil

^IVPatologista. Doutor. Médico Assistente do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da USP - Ribeirão Preto, SP, Brasil

^VProfessor de Neurocirurgia do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da USP - Ribeirão Preto, SP, Brasil

^VIProfessor Titular de Neurocirurgia do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da USP - Ribeirão Preto, SP, Brasil

^{Correspondência}

ABSTRACT

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.

Keywords: Hemangioblastoma; Spinal cord neoplasms; von Hippel-Lindau disease; Cervical vertebrae.

RESUMO

Hemangioblastomas do sistema nervoso central são lesões de baixo grau de malignidade, altamente vascularizadas, que podem se apresentar esporadicamente ou associadas com a doença de Von Hippel-Lindau. Hemangioblastomas extradurais são incomuns e os extra e intradurais são ainda mais raros. Este estudo usa um caso ilustrativo e revisão da literatura para discutir as dificuldades de considerar o diagnóstico correto e selecionar a melhor abordagem cirúrgica. Um paciente do sexo masculino, branco, com 57 anos de idade apresentou-se com mielopatia e radiculopatia de C5 à direita. As imagens mostraram lesão extra-intradural lobulada, em forma de ampulheta, com alta impregnação após contraste, que ocupava o canal vertebral e estreitava o forame intervertebral de C4-C5 à direita. A ressecção total da lesão intradural foi alcançada através de abordagem posterior, mas a porção extradural só pôde ser parcialmente removida. Melhora total dos sintomas foi observada após quatro meses e o tumor residual tem sido seguido clínica e radiologicamente. Embora a impressão pré-operatória tenha sido de um schwannoma espinal, o exame histopatológico revelou hemangioblastoma grau I, segundo a OMS. Apesar de sua raridade, exames complementares atuais permitem o correto diagnóstico pré-operatório. Isto é essencial para melhor programação cirúrgica, tendo em vista as características particulares desta lesão.

Descritores: Hemangioblastoma; Neoplasia de medula espinal; Doença de von Hippel-Lindau; Vértebras cervicais.

RESUMEN

Hemangioblastomas del sistema nervioso central (SNC) son tumores altamente vascularizados, de grado bajo, que pueden ser esporádicos o vinculados a la enfermedad de Von Hippel-Lindau. Hemangioblastomas extradurales no son comunes, y aquellos localizados extra e intraduralmente son aún más raros. Este estudio usa un caso ilustrativo y la revisión de la literatura para analizar las dificultades cuanto a considerar el diagnóstico correcto y para seleccionar el mejor abordaje quirúrgico. Un paciente, hombre blanco de 57 años de edad, presentaba mielopatía con radiculopatía C5 derecha. Las imágenes mostraban lesión extra/intradural, muy acrecentada, con forma de ampolla y lobulada, la cual ocupaba el conducto espinal y ensanchaba el agujero intervertebral derecho C4-C5. La resección de la lesión intradural fue conseguida mediante un abordaje posterior, pero la parte extradural solamente pudo ser removida parcialmente. La mejoría completa fue observada después de cuatro meses de seguimiento y el tumor residual ha sido acompañado clínica y radiológicamente. Aunque la impresión preoperatoria era de schwannoma espinal, el examen histopatológico reveló hemangioblastoma grado I según la Organización Mundial de la Salud. A pesar de su rareza, los actuales exámenes complementarios permiten considerar, preoperativamente, el diagnóstico de hemangioblastoma. Esto es esencial para hacer un mejor planeamiento quirúrgico, teniendo en cuenta los aspectos quirúrgicos peculiares de esta lesión.

Descriptores: Hemangioblastoma; Neoplasias de la médula espinal; Enfermedad de von Hippel-Lindau; Vértebras cervicales.

INTRODUCTION

Haemangioblastomas of the central nervous system are low-grade highly vascular tumors that may be sporadic or associated with Von Hippel-Lindau disease^1-3. The most common location is the cerebellum² and only 3 to 13% of all haemangioblastomas occur in the spine where they account for 1,6 to 5.8% of all spinal cord tumors. In the spine they are usually intramedulary^3-6. Extramedullary haemangioblastomas are uncommon. These may be intradural, extradural, or extra-intradural with a dumbbell extension, arising from the filum terminale or a proximal nerve root^3,4,7-9. In 1975, Hurth et al. reported 17 extradural haemangioblastomas in a series of 138 spinal cases, of which only 3 cases were extra-intradural. Only 8 cases of these tumors were found described in the literature since computed tomography (CT) scanning and magnetic resonance imaging (MRI) have become available^1,3,10-15.

The aim of this study is to discuss the difficulties to determine the correct preoperative diagnosis of these lesions and its importance on deciding the best treatment strategy based on a case report and literature review.

ILLUSTRATIVE CASE

A 57 years-old white male presented with a 6 months history of neck pain and 1 month of increasing weakness of the superior limbs, followed by lower left limb paresis, bowel and bladder disturbance. His additional medical history was significant only for hypertension. Physical examination demonstrated myelopathy and C5 radiculopathy characterized by triparesis that was worse in the upper limbs with preserved strength in the lower right limb, increased tone and Babinski signal in his lower extremities as well as deep hyperreflexy, though right biccipital hyporeflexy. On spine CT scans (Figure 1A) a marked widening of the right C4-C5 intervertebral foramen was noted, as well as facets erosion and no hyperostosis. The MRI (Figure 1B and 2A,B,C) showed a lobulated, dumbbell shaped, extra-intradural lesion that was markedly enhanced after gadolinium injection. The lesion occupied the spinal canal and the C4-C5 intervertebral foramen compressing and displacing the spinal cord medially and posteriorly. The extra-spinal component came into contact with the vertebral artery, which was slightly displaced medially and anteriorly. Flow-void signals were retrospectively observed inside and around the tumoral mass, suggesting a high-vascularized tumor. Schwannoma and malignant tumors were considered as preoperative diagnosis.

The patient was placed in a right lateral position with the head fixed in the Mayfield and aligned with the vertebral spine. (Figure 3A,B) A straight medial occipito-cervical incision was performed followed by a C3-C4 laminectomy and right C4-C5 foraminotomy and "T" shape durotomy directed to the lesion entry point at the intradural space. The entry point was coagulated and cut to permit the total resection of the intradural lesion. The surgical finding consisted of a firm and high-vascularized extradural lesion that made the intervertebral foramen and the C4 joint surface very thin and crossed the dura at the level of C4. The intradural lesion was soft, resembling a sponge, attached to the dura and supplied by anomalous vessels. Total resection of the intradural part of the lesion could be achieved, but the extradural part was removed partially. The lateral dural opening was occluded with a muscle patch and sutured to the dura. The histopathological examination revealed an haemangioblastoma - WHO grade I (Figure 4A,B,^C,^D,^F). The patient improved completely and was already asymptomatic 4 month after surgery. Postoperative MRI showed remaining lesion extradurally, that has been followed clinically and radiologically for 1 year already. Investigation for Von Hippel-Lindau disease was performed and resulted negative. The brain and the rest of the spinal canal were scanned and no additional tumoral lesion was found.

DISCUSSION

In these rare cases of extra-intradural haemangioblastomas, enlargement of an intervertebral foramen and a dumbbell shape wrongly suggest the preoperative hypothesis of nerve root schwannoma or neurofibroma¹. Nevertheless, schwannomas that grow with an extension to the intervertebral foramen are not common¹⁶; and there are some other different kinds of tumors that may present with this location. Besides these facts, extra-intradural hemanioblastomas are extremely rare^1,9,10,13. However, with the diagnostic tools nowadays available, even the diagnosis of such an uncommon lesion is possible. On MRI sequences, solid portions of haemangioblastomas generally have high-intensity signal on T2-weighted images, intermediate or low-intensity signal on T1-weighted sequences, and marked enhancement with gadolinium^5,7,10,17-19. Features suggesting vessels, such as flow-void images, are often visible, especially in large tumors⁵. Angiography confirms the diagnosis of a highly vascularized tumor⁴, thus eliminating the possibility of an arteriovenous mal-formation¹⁷. The key to preoperative diagnosis in these tumors is their marked enhancement and associated enlarged vessels²⁰. Surgery is the treatment of choice for symptomatic haemangioblastomas^1,4,10, but cervical dumbbell lesions raise the problems of radical resection, vertebral artery control, nerve root preservation, and spine stability¹.

An anterior approach alone through the vertebral body is not adequate, because it cannot reach the extra spinal and foraminal part of the tumor. Barrey et al.¹ defend a lateral approach because it allows control of the distal nerve root and the vertebral artery before resection of the tumor and permits reaching even the intradural part as was showed in their case report. In that case the vertebral artery was firstly tested and sacrificed during surgery, as well as the nerve root, which stimulation resulted no response.

A posterior approach with laminectomy and unilateral facetectomy has been proposed for dumbbell neuromas²¹ and was chosen for the present case. The good visualization of the intradural space made it possible to resect the whole intradural part without causing additional injury to the spinal cord. However, the hard visualization of the vertebral artery and no control of the blood supplying vessels before the tumor resection made it impossible to perform a radical resection. Another disadvantage of this approach is that a joint must always be sacrificed¹. The residual lesion next to the vertebral artery may cause gradual occlusion of this vessel and the possibility of its sacrifice in a likely reoperation.

If the posterior surgical approach is chosen, preoperative embolization or even vertebral artery occlusion may be considered after balloon occlusion test in cases of haemangioblastomas to decrease tumoral blood supply and facilitate surgical resection¹⁴. In lateral approaches, the vertebral artery will be visible before reaching the tumor, and the vascular control could be performed at the surgical act¹, although balloon occlusion testing before surgery is mandatory.

CONSIDERACIONES FINALES

Despite their rarity, current complementary exams allow considering the diagnosis of haemangioblastoma preoperatively. That is essential to a better surgical setup as these lesions have a great propensity for bleeding.

Recebido em 03/05/2010, aceito em 10/11/2010.

Trabalho realizado no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP).

• 1. Barrey C, Kalamarides M, Polivka M, George B. Cervical dumbbell intra-extradural hemangioblastoma: total removal through the lateral approach: technical case report. Neurosurgery. 2005;56(3):E625.
• 2. Richard S, Campello C, Taillandier L, Parker F, Resche F. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group. J Intern Med. 1998;243(6):547-53.
• 3. Escott EJ, Kleinschmidt-DeMasters BK, Brega K, Lillehei KO. Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. Surg Neurol. 2004;61(3):262-73;
• 4. 4. Browne TR, Adams RD, Roberson GH. Hemangioblastoma of the spinal cord. Review and report of five cases. Arch Neurol. 1976;33(6):435-41.
• 5. Chu BC, Terae S, Hida K, Furukawa M, Abe S, Miyasaka K. MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol. 2001;22(1):206-17.
• 6. Murota T, Symon L. Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery. 1989;25(5):699-707.
• 7. Hermier M, Cotton F, Saint-Pierre G, Jouvet A, Ongolo-Zogo P, Fischer G, et  al. Myelopathy and sciatica induced by an extradural S1 root haemangioblastoma. Neuroradiology. 2002;44(6):494-8.
• 8. Higgins JN, Lammie GA, Savy LE, Taylor WJ, Stevens JM. Intraosseous vertebral haemangioblastoma: MRI. Neuroradiology. 1996;38(Suppl 1):S107-10.
• 9. Hurth M. [Intraspinal hemangioblastomas]. Neurochirurgie. 1975;21(Suppl 1):1-136.
• 10. Bilgen IG, Yünten N, Demirtas E, Cagli S, Zileli M. Hemangioblastoma causing cervical neural foraminal widening. J Neuroradiol. 2001;28(3):205-8.
• 11. Chazono M, Shiba R, Funasaki H, Soshi S, Hattori A, Fujii K. Hemangioblastoma of the L-5 nerve root. Case illustration. J Neurosurg. 1999;90(Suppl 1):160.
• 12. Rohde V, Voigt K, Grote EH. Intra-extradural hemangioblastoma of the cauda equina. Zentralbl Neurochir. 1995;56(2):78-82.
• 13. Tronnier V, Hartmann M, Hamer J, Kremer P, Sommer C, Jansen O, et al. Extradural spinal hemangioblastomas: report of two cases. Zentralbl Neurochir. 1999;60(2):86-92.
• 14. Sawyer B, Heng R, Ferris N. Typical magnetic resonance features of a haemangioblastoma, an uncommon nerve root lesion: case report and literature review. Australas Radiol. 2007;51:B28-30.
• 15. Sid-Ahmed S, Seizeur R, Forlodou P, Dam-Hieu P, Quintin-Roue I, Person H, et al. [Dumbell hemangioblastoma of the eighth cervical nerve root]. Neurochirurgie. 2006;52(6):533-6.
• 16. George B, Lot G. Surgical treatment of dumbbell neurinomas of the cervical spine. Crit Rev Neurosurg. 1999;9(3):156-160.
• 17. Grunberg A, Rodesch G, Hurth M, Carlier R, Doyon D. [Magnetic resonance imaging of intraspinal hemangioblastoma]. Neurochirurgie. 1994;40(3):155-64.
• 18. Lee RR. MR imaging of intradural tumors of the cervical spine. Magn Reson Imaging Clin N Am. 2000;8(3):529-40.
• 19. Sonier CB, De Kersaint-Gilly A, Resche F, Halimi P, Bouyssou A, Bricout JH. [The value of magnetic resonance imaging in the diagnosis of spinal Cord hemangioblastoma. Apropos of 12 cases]. J Neuroradiol. 1994;21(3):194-204.
• 20. Baker KB, Moran CJ, Wippold FJ 2nd, Smirniotopoulos JG, Rodriguez FJ, Meyers SP, et al. MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol. 2000;174(2):377-82.
• 21. McCormick PC. Surgical management of dumbbell tumors of the cervical spine. Neurosurgery. 1996;38(2):294-300.

Publication Dates

• Publication in this collection
  01 Nov 2012
• Date of issue
  Sept 2012

History

• Received
  03 May 2010
• Accepted
  10 Nov 2010