Abstract
Cerebellar hemangioblastomas remain surgically challenging because of the narrow, deep surgical corridors and tumor hypervascularity. Various surgical approaches are used according to the location, but optimal approaches have not been established. We propose a system of surgical approaches based on the venous drainage systems to facilitate surgical planning and achieve acceptable neurological outcomes. Cerebellar hemangioblastomas were divided into five types based on the main drainage systems: suboccipital hemangioblastomas draining to the transverse sinus (TS) or torcula, tentorial hemangioblastomas draining to the tentorialsinus orstraight sinus, petrosal hemangioblastomas draining to the superior petrosal sinus (SPS), quadrigeminal hemangioblastomas draining to the galenic system, and tonsillar hemangioblastomas draining to the TS or torcula in conjunction with jugular bulb or SPS. Microsurgical approaches and patient outcome were retrospectively reviewed according to this classification. This study included 17 patients who underwent 21 operations for resection of 19 cerebellar hemangioblastomas, classified into 9 suboccipital, 4 tentorial, 2 petrosal, 2 quadrigeminal, and 2 tonsillar. Standard suboccipital craniotomies were utilized for suboccipital hemangioblastomas, the occipital transtentorial approach (OTA), and supracerebellar infratentorial approach for tentorial hemangioblastomas, the retrosigmoid approach for petrosal hemangioblastomas, OTA for quadrigeminal hemangioblastomas, and midline suboccipital approach for tonsillar hemangioblastomas. Gross total resection was achieved in all patients except one. Two patients with large hemangioblastomas (tonsillar and quadrigeminal) required second-stage operation which finally achieved gross total removal. No single approach had a significantly higher incidence of postoperative neurological deficits. Selection of the optimum surgical approach for cerebellar hemangioblastomas was successful based on the main drainage systems. Understanding of tumor growth and extension with respect to the venous drainage system is critical to select the appropriate surgical approach.
Highlights
Central nervous system (CNS) hemangioblastoma is a relatively rare intracranial neoplasm accounting for 1.5-2.5% of CNS tumors, and can occur as a sporadic entity as well as in patients with von HippelLindau (VHL) disease, an autosomal dominant inherited disorder.[10,14] CNS hemangioblastomas are benign, highly vascularized tumors classified as grade I according to the World Health Organization classification
Three recurrent hemangioblastomas in 2 patients were excluded because the drainage veins could not be determined due to the previous surgery, so 17 patients who underwent a total of 21 operations for 19 cerebellar hemangioblastomas were evaluated
This study included 17 patients who underwent a total of 21 operations for resection of 19 cerebellar hemangioblastomas at our institution, 10 men and 7 women aged 23-86 years at initial surgery
Summary
Central nervous system (CNS) hemangioblastoma is a relatively rare intracranial neoplasm accounting for 1.5-2.5% of CNS tumors, and can occur as a sporadic entity as well as in patients with von HippelLindau (VHL) disease, an autosomal dominant inherited disorder.[10,14] CNS hemangioblastomas are benign, highly vascularized tumors classified as grade I according to the World Health Organization classification. CNS hemangioblastomas occur most frequently in the cerebellum followed by other specific CNS locations such as the retina, spinal cord, and brainstem.[6,13,34] Various surgical approaches for cerebellar hemangioblastoma are adopted according to tumor location.[1,7,8,9,10,11,15,20,21,33] Cerebellar hemangioblastomas present unique operative challenges, despite current advances in microneurosurgical techniques, such as the narrow and deep surgical corridor in the posterior fossa, inability to debulk the tumor mass due to hypervascularity, difficulty in early access to the feeding artery behind the tumor, and interference by large tortuous draining veins during the resection.[8,9,10,19,21] selection of the optimal surgical approach and operative planning is quite important to access the pial surface and achieve panoramic exposure of the circumferential entire margin of the lesion for early management of the cortical feeding artery and identification of the main draining system. The optimal surgical approach to cerebellar hemangioblastomas has not been systematically established and comprehensively specified because of the rarity of these lesions
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