Background: Hemangioblastomas (HB) are rare lesions accounting for 2% of all spinal cord tumors. They are highly vascular, benign tumors that occur either sporadically or in the presence of von Hippel-Lindau disease. Spinal cord HB are usually diagnosed in adult patients and their incidence in early infancy is an extreme rarity. Methods: We present a case of a 1-month-old male with a back deformity and left leg hypomotility. MRI of the spine revealed an intramedullary tumor extending from level T6 to T12. Results: The tumor was excised completely, using standard microsurgical techniques via a posterior approach. The histological diagnosis was spinal cord HB. Conclusion: A review of the literature revealed that this neoplasm is composed of 3 major cell types: endothelial cells, pericytes and stromal cells. Complete microsurgical removal is the treatment of choice for spinal cord HB because the tumor is benign. To the best of our knowledge, sporadic spinal cord HB at this age has not been reported so far.

Emery E, Hurth M, Lacroix-Jousselin C, David P, Richard S: Intraspinal hemangioblastoma: apropos of a recent series of 20 cases (in French). Neurochirurgie 1994;40:165–173.
Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES: Spinal cord hemangioblastoma: diagnosis and clinical outcome after surgical treatment. J Korean Neurosurg Soc 2007;42:436–440.
Sharma GK, Kucia EJ, Spetzler RF: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery 2009;65:E627–E628, discussion E628.
Roonprapunt C, Silvera VM, Setton A, Freed D, Epstein FJ, Jallo GI: Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery 2001;49:321–327, discussion 327–328.
Stern J, Gundlach P, Heller M, Rothemund E: Spinal hemangioblastoma in infants with dysraphic malformation of the CNS (in German). Helv Paediatr Acta 1983;38:523–532.
Mock A, Levi A, Drake JM: Spinal hemangioblastoma, syrinx, and hydrocephalus in a two-year-old child. Neurosurgery 1990;27:799–802.
Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P: Hemangioblastomas of the central nervous system: a 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 1989;70:24–30.
Xu QW, Bao WM, Mao RL, Yang GY: Magnetic resonance imaging and microsurgical treatment of intramedullary hemangioblastoma of the spinal cord. Neurosurgery 1994;35:671–675, discussion 675–676.
Hu Y, Pang Z, Wang Q, Yang G, Cheng N: A histopathologic and immunohistochemical study of 68 cases of hemangioblastoma (in Chinese). Hua Xi Yi Ke Da Xue Xue Bao 2000;31:380–382.
Samii M, Klekamp J: Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery 1994;35:865–873, discussion 873.
Wang C, Zhang J, Liu A, Sun B: Surgical management of medullary hemangioblastoma: report of 47 cases. Surg Neurol 2001;56:218–226, discussion 226–227.
Johnson MD, Mitchell AR, Troup EC, Bhowmick DA, Wait SD, Aulino J, Zhuang Z, Weil RJ: Congenital cystic hemangioblastomas of the cerebral hemisphere in a neonate without alteration in the VHL gene. Pediatr Neurosurg 2004;40:124–127.
Tse JY, Wong JH, Lo KW, Poon WS, Huang DP, Ng HK: Molecular genetic analysis of the von Hippel-Lindau disease tumor suppressor gene in familial and sporadic cerebellar hemangioblastomas. Am J Clin Pathol 1997;107:459–466.
Gijtenbeek JM, Jacobs B, Sprenger SH, Eleveld MJ, van Kessel AG, Kros JM, Sciot R, van Calenbergh F, Wesseling P, Jeuken JW: Analysis of von Hippel-Lindau mutations with comparative genomic hybridization in sporadic and hereditary hemangioblastomas: possible genetic heterogeneity. J Neurosurg 2002;97:977–982.
Ding XH, Zhou LF, Tan YZ, Zhao Y, Zhu JJ: Histologic and histogenetic investigations of intracranial hemangioblastomas. Surg Neurol 2007;67:239–245, discussion 245.
Park DM, Zhuang Z, Chen L, Szerlip N, Maric I, Li J, Sohn T, Kim SH, Lubensky IA, Vortmeyer AO, Rodgers GP, Oldfield EH, Lonser RR: Von Hippel-Lindau disease-associated hemangioblastomas are derived from embryologic multipotent cells. PLoS Med 2007;4:e60.
Almodovar CR, Zacchigna S, Carmeliet P: Angiogenesis in the central nervous system; in Figg WD, Folkman J (eds): Angiogenesis. An Integrative Approach from Science to Medicine. New York, Springer Science, 2008, pp 497–498.
Richard S, Martin S, David P, Decq P: Von Hippel-Lindau disease and central nervous system hemangioblastoma: progress in genetics and clinical management (in French). Neurochirurgie 1998;44:258–266.
Wanebo JE, Lonser RR, Glenn GM, Oldfield EH: The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003;98:82–94.
Wu TC, Guo WY, Lirng JF, Wong TT, Chang FC, Luo CB, Teng MM, Chang CY: Spinal cord hemangioblastoma with extensive syringomyelia. J Chin Med Assoc 2005;68:40–44.
Xu Q, Bao W, Pang L: Diagnosis and treatment of intramedullary hemangioblastoma of cervical spinal cord. Chin Med J (Engl) 2002;115:1010–1013.
van Velthoven V, Reinacher PC, Klisch J, Neumann HP, Gläsker S: Treatment of intramedullary hemangioblastomas, with special attention to von Hippel-Lindau disease. Neurosurgery 2003;53:1306–1313, discussion 1313–1314.
Maira G, Amante P, Denaro L, Mangiola A, Colosimo C: Surgical treatment of cervical intramedullary spinal cord tumors. Neurol Res 2001;23:835–842.
Lee DK, Choe WJ, Chung CK, Kim HJ: Spinal cord hemangioblastoma: surgical strategy and clinical outcome. J Neurooncol 2003;61:27–34.
Biondi A, Ricciardi GK, Faillot T, Capelle L, van Effenterre R, Chiras J: Hemangioblastomas of the lower spinal region: report of four cases with preoperative embolization and review of the literature. AJNR Am J Neuroradiol 2005;26:936–945.
Boström A, Hans FJ, Reinacher PC, Krings T, Bürgel U, Gilsbach JM, Reinges MH: Intramedullary hemangioblastomas: timing of surgery, microsurgical technique and follow-up in 23 patients. Eur Spine J 2008;17:882–886.
Lonser RR, Weil RJ, Wanebo JE, DeVroom HL, Oldfield EH: Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2003;98:106–116.
Lonser RR, Oldfield EH: Microsurgical resection of spinal cord hemangioblastomas. Neurosurgery 2005;57:372–376, discussion 372–376.
Young W: NASCIS: National Acute Spinal Cord Injury Study. J Neurotrauma 1990;7:113–114.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.