Clinical Study Outcomes of gamma knife treatment for solid intracranial hemangioblastomas Hakan Karabagli a , Ali Genc b , Pinar Karabagli c , Ufuk Abacioglu d , Askin Seker b , Turker Kilic b, * a Department of Neurosurgery, Selcuklu Medical Faculty, Selcuk University, Konya,Turkey b Department of Neurosurgery, Marmara University Institute of Neurological Sciences, Marmara University Medical Faculty, PK 53 Maltepe 34853, Istanbul,Turkey c Laboratory of Pathology, Konya Research and Training Hospital, Konya,Turkey d Department of Radiation Oncology, Marmara University Medical Faculty, Istanbul,Turkey a r t i c l e i n f o Article history: Received 29 July 2009 Accepted 21 September 2009 Keywords: Gamma knife Hemangioblastoma Radiosurgery Von Hippel–Lindau disease a b s t r a c t The aim of this study was to examine the results of gamma knife radiosurgery for 13 patients with resid- ual/recurrent or newly diagnosed solid hemangioblastomas. The 13 patients had 34 solid hemangioblas- tomas,and all patients underwent gamma knife radiosurgery. Seven patients had von Hippel–Lindau disease and six had sporadic disease. When individual lesions were considered, the overall mean dose at the tumor periphery was 15.8 Gy (range:12–25 Gy) and the average maximum tumor dose was 31.6 Gy (range: 24–50 Gy). The mean duration of follow-up with MRI was 50.2 months. At the last fol- low-up evaluation, growth control was achieved for all tumors (partial remission in three tumors [8.8%] and no change in 31 tumors [91.2%]). No radiation-related complications were encountered. Our findings reinforce the view that gamma knife radiosurgery is effective and safe for the management of solid hemangioblastomas with a diameter less than 3 cm, whether they are sporadic or associated with von Hippel–Lindau disease. The high response rate and lack of any radiation-induced side-effects con- firms the suitability of the doses used in the present study. Ó 2009 Elsevier Ltd. All rights reserved. 1. Introduction Hemangioblastomas are rare, highly vascular, histologically be- nign tumors of uncertain histogenesis,although ultrastructural studies indicate an angioblastic origin. 1,2 They can have a solid or cystic structure.Two histologicalvariants have been recognized: reticular and cellular.Cellular hemangioblastomas are less com- mon than the reticular kind, and they recur more frequently. 3 Hemangioblastomas present in both sporadic forms and familial forms associated with von Hippel–Lindau (VHL) disease. VHL dis- ease is an autosomal-dominant disorder characterized by benign and malignant tumors mostly seen in the retina, kidney,adrenal gland, pancreas, and epididymis, in addition to central nervous sys- tem hemangioblastomas. 4–6 For patients without a family history, the presence of at least two hemangioblastomas or one hemangio- blastoma and one visceral lesion is sufficient to establish a diagno- sis of VHL disease. 4,7 Hemangioblastomas generally occur in adults. Except when associated with VHL disease, the clinical manifesta- tion of hemangioblastomas is not specific. Headache and dizziness are common symptoms, and they are generally not serious. Intra- cranial hypertension and hydrocephalus may develop later in the course of the disease and cerebellar signs are often mild. 8 Cranial nerve involvement is not common, occurring in one-third of patients. 8,9 The cerebellum is the most frequent site ofhemangioblasto- mas. 10,11 Surgical excision and radiosurgery tend to be the treat- ments of choice for hemangioblastomas.Resection is the preferred treatment for a single tumor,and is usually curative. However, vascularity and critical location can make complete resection impossible,especially when there are multiple hema- ngioblastomas. 4,12,13 On MRI, hemangioblastomashave distinct margins, intense contrast enhancementand a spherical shape, which permit accurate and reliable measurement of tumor volume. These features also allow the detection of any enlargement due to tumor progression or reduction due to response to treatment. 7 Cystic hemangioblastomas are more likely to be symptomatic, and their clinical behavior is distinct from that of solid hema- ngioblastomas. 7 Therefore,solid hemangioblastomas have been investigated as a separate clinical entity in recent studies. 14 Most symptomatic hemangioblastomas seem to be of the cystic subtype. The results of several recent studies have suggested that gamma knife surgery (GKS) is effective for solid hemangioblastomas, yield- ing shrinkage or stabilization with low complication rates. 4,12,13,15 In the present study, we analyzed the outcomes of GKS for 13 patients with residual/recurrent or newly diagnosed solid hema- ngioblastomas in an attempt to define the role and appropriate strategy for gamma knife radiosurgery in the management of these lesions. 0967-5868/$ - see front matter Ó 2009 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2009.09.028 * Corresponding author.Tel.: +90 532 514 14 98; fax: +90 216 327 5249/305 7961. E-mail address: turker.kilic@tnn.net (T. Kilic). Journal of Clinical Neuroscience 17 (2010) 706–710 Contents lists available at ScienceDirect Journal of Clinical Neuroscience j o u r n a l h om e pa ge : w w w . e l s e v i e r . c o m / l o c a t e / j o c n