Article
Cumulative surgical morbidity in patients with multiple cerebellar and medullary hemangioblastomas
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Published: | June 9, 2017 |
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Objective: Patients affected with von Hippel-Lindau disease frequently develop multiple hemangioblastomas of the central nervous system, predominantly of the cerebellum, brain stem and spinal cord. Timing of surgical intervention is difficult and depends largely on the anticipated surgical morbidity. However, data regarding surgical outcome after multiple cerebellar and medullary surgeries are scarce.
Methods: To investigate cumulative surgical morbidity in patients operated on multiple hemangioblastomas of the central nervous system and to deduce recommendations for treatment, we analyzed all patients with surgical treatment of at least two cerebellar and/or medullary hemangioblastomas at the University of Freiburg between 1996 and 2013. Pre- and postoperative functional grades were determined by Modified Ranking Scale (cerebellar surgeries) or by Modified McCormick Score (medullary surgeries).
Results: A total of 36 patients underwent surgeries for at least two cerebellar hemangioblastomas (12 patients), for at least two medullary hemangioblastomas (19 patients) or for at least two hemangioblastomas in both locations (5 patients). All together 48 cerebellar and 80 medullary procedures were performed. Regarding cerebellar hemangioblastomas, postoperative functional grades determined by Modified Ranking Scale improved after 9 and remained stable after 39 out of 48 performed surgeries without one case of deterioration. Regarding medullary hemangioblastomas, postoperative functional grades determined by Modified McCormick Score improved after 3, remained stable after 54 and deteriorated after 23 out of 80 performed surgeries. The severity of deterioration did not increase with the number of performed medullary surgeries.
Conclusion: Resection even of multiple cerebellar hemangioblastomas is not associated with cumulative morbidity. Although there is a certain surgical morbidity caused by medullary surgeries, yet its extent does not increase with the number of performed surgeries. Microsurgical removal of asymptomatic tumors with radiographic progression should also be considered in patients with multiple tumors and previous surgeries.