OBJECTIVE Patients affected with von Hippel-Lindau disease often develop multiple hemangioblastomas in the cerebellum 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. Our objective was to evaluate cumulative surgical morbidity in patients operated on multiple cerebellar and medullary hemangioblastomas and to deduce recommendations for treatment. METHODS We performed a retrospective analysis for a consecutive cohort of von Hippel-Lindau patients with surgical treatment of at least two cerebellar and/or medullary hemangioblastomas. Pre- and postoperative functional grades were reviewed in patients' files and compared by Modified Ranking Scale (cerebellar surgeries) or by Modified McCormick Score (medullary surgeries). RESULTS Thirty-six patients were surgically treated for at least two cerebellar hemangioblastomas (12 patients), at least two medullary hemangioblastomas (19 patients) or at least two hemangioblastomas in both locations (5 patients). Fourthy-eight cerebellar and 80 medullary procedures were performed in total. On average, multiple cerebellar surgeries caused no clinical deterioration, whereas multiple medullary surgeries led to a slight cumulative deterioration of postoperative functional grades. The severity of this deterioration did not correlate to the number of performed medullary surgeries. CONCLUSION Resection of multiple cerebellar hemangioblastomas is not associated with cumulative morbidity. Although there is a certain cumulative surgical morbidity caused by medullary surgeries, its extent does not increase with the number of performed surgeries. Microsurgical removal of asymptomatic tumors with radiographic progression can also be considered for patients with multiple tumors and previous surgeries.

中文翻译：

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多发性小脑和髓质血管母细胞瘤患者的累积手术发病率

目的 受 von Hippel-Lindau 病影响的患者通常会在小脑和脊髓中发展为多发性血管母细胞瘤。手术干预的时机很困难，很大程度上取决于预期的手术发病率。然而，关于多次小脑和延髓手术后手术结果的数据很少。我们的目标是评估多发性小脑和髓质血管母细胞瘤手术患者的累积手术发病率，并推导出治疗建议。方法 我们对连续队列的 von Hippel-Lindau 患者进行了回顾性分析，这些患者接受了至少两个小脑和/或髓质血管母细胞瘤的手术治疗。对患者的术前和术后功能等级进行审查 文件，并通过改良等级量表（小脑手术）或改良 McCormick 评分（髓质手术）进行比较。结果 36 名患者接受了至少两个小脑血管母细胞瘤（12 名患者）、至少两个髓质血管母细胞瘤（19 名患者）或两个位置至少两个血管母细胞瘤（5 名患者）的手术治疗。共进行了 48 次小脑手术和 80 次延髓手术。平均而言，多次小脑手术不会导致临床恶化，而多次延髓手术导致术后功能等级的轻微累积恶化。这种恶化的严重程度与进行髓质手术的次数无关。结论 多发性小脑血管母细胞瘤的切除与累积发病率无关。髓质手术虽然有一定的累积手术发病率，但其程度不会随着手术次数的增加而增加。对于多发肿瘤和既往手术的患者，也可以考虑显微手术切除放射学进展的无症状肿瘤。