CLINICAL STUDY - PATIENT STUDY Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups Fabrice Parker Æ Nozar Aghakhani Æ Luis Gustavo Ducati Æ Adriano Yacubian-Fernandes Æ Mateus Violin Silva Æ Phillipe David Æ Stephane Richard Æ Marc Tadie Received: 7 October 2008 / Accepted: 16 March 2009 Ó Springer Science+Business Media, LLC. 2009 Abstract Purpose To analyze the surgical outcome of a consecutive and single center series of medulla oblongata (MO) and spinal cord hemangioblastomas (HB). Patient and methods We retrospectively reviewed the medical charts of all MO and spinal HB patients operated on in our institution between 1985 and 2002. All patients had pre- and at least one post-operative MRI. McCormick classifi- cation was used to assess neurological status and functional outcome. Results Forty surgical procedures have been performed on 34 patients (19 females and 15 males, mean age of 41 years). Twenty-five (73%) patients had Von Hippel Lindau (VHL) disease confirmed by genetic screening, and nine patients had sporadic disease. Com- plete clinical, radiological, and genetic studies were done in all cases. The most frequent clinical symptom was pain (28 patient, 85%) followed by motor (42%) and sensitive deficits (42%). Fourteen lesions (19%) were located in the MO, 28 (38%) in the cervical spine, 25 (34%) in the tho- racic spine, 4 (5%) in the lumbar spine and 3 (4%) in the Cauda Equina. In the VHL group, 15 patients (60%) pre- sented multiple lesions and 10 a single neurological lesion (40%). A cyst was present in 23% of VHL patients and in 55% of the non-VHL group. A complete removal was achieved in 85% of all cases. No deaths related to surgery occurred. At the end of the follow-up period (mean 60 months) 50% of patients were stabilized, while the condition of 32.35% was improved and of 17.65% wors- ened. Comparing the clinical evolution considering the presence or not of VHL we have seen that there are no differences in terms of functional outcome between VHL and non-VHL groups. Conclusion Our results confirmed that surgery remains a safe and effective treatment for medulla oblongata and spinal hemangioblastoma. Only symptomatic lesion required surgical treatment. In other cases, especially in VHL patients, a close and regular fol- low-up (clinical and MRI) is necessary. Keywords Hemangioblastoma Á Medulla oblangata Á Spinal cord Á Von Hippel Lindau Introduction Spinal cord hemangioblastomas correspond to 5% of intramedullary tumors [1–4]. They are associated with Von Hippel Lindau disease in 20–30% of the cases [5–10]. Pain syndrome, motor and sensitive deficits are the clinical symptoms usually presented. When located in the medulla oblongata, neck pain and cranial nerves palsy can be present [11–13]. Magnetic resonance imaging (MRI) sug- gests the diagnosis when the lesion has contrast enhancing associated or not to abnormal vessels in the subarachnoid space and intramedullary cysts [14, 15]. Complete micro- surgical removal is the treatment of choice for these tumors [16–22]. The objectives of the present study were to ana- lyze the results of the microsurgical treatment for the spinal cord and medulla oblongata hemangioblastomas and better understand the clinical and radiological presentation of these tumors. The early and late post-operative follow-ups were analyzed considering the occurrence of VHL. F. Parker Á N. Aghakhani (&) Á L. G. Ducati Á A. Yacubian-Fernandes Á M. V. Silva Á P. David Á M. Tadie Department of Neurosurgery, Bice ˆtre University Hospital, 78 rue du general Leclerc, 94275 Le Kremlin-Bice ˆtre Cedex, France e-mail: Nozar.aghakhani@bct.aphp.fr S. Richard Laboratoire de ge ´ne ´tique oncologique EPHE and CNRS FRE 2939, Bicetre University Hospital, University Paris XI, Bicetre, France 123 J Neurooncol (2009) 93:133–137 DOI 10.1007/s11060-009-9861-0