En Bloc Resection for Spinal Cord Hemangioblastomas: Surgical Technique and Clinical Outcomes

Author:

Chen Xiaofeng123,Guo Hua1,Zhang Jianli4,Ye Junyi5,Wang Shurong6,Jiang Haiping7,Mu Qingchun8,Wang Xiaoxiong123

Affiliation:

1. Department of Neurosurgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China

2. Institute of Brain Science, Harbin Medical University, Harbin, Heilongjiang, China

3. Institute of Neuroscience, Sino-Russian Medical Research Center, Harbin Medical University, Harbin, Heilongjiang, China

4. Department of Neurology, Xiamen Fifth Hospital, Xiamen, Fujian, China

5. Department of Neurosurgery, The Affiliated Lihuili Hospital of Ningbo University, Ningbo, Zhejiang, China

6. Department of Neurology, Hainan Medical University, Haikou, Hainan, China

7. Department of Neurosurgery, The Affiliated Hospital of Guangdong Medical University, Zhanjiang, Guangdong, China

8. Department of Neurosurgery, The People's Hospital of Gaozhou of Guangdong Medical University, Maoming, Guangdong, China

Abstract

Abstract Background Spinal cord hemangioblastomas are rare benign and highly vascular tumors that develop either sporadically or as part of von Hippel–Lindau (VHL) disease. Generally, complete resection without significant neurologic deficit remains considerably challenging due to the risk of massive bleeding. The current study therefore aimed to describe en bloc resection of spinal cord hemangioblastomas according to the typical anatomical structures of peripheral lesions and evaluate the neurofunctional prognosis of this technique. Methods A total of 39 spinal cord hemangioblastomas from a series of 19 patients who underwent en bloc resection were retrospectively analyzed. In all cases, clinical and radiologic characteristics, as well as surgical tenets, were retrospectively determined and analyzed. Short- and long-term outcomes were analyzed using the McCormick grade and Odom's criteria. Factors significantly associated with poor neurologic function after en bloc resection were also determined. Results All 39 spinal cord hemangioblastomas, including 28 intramedullary, 2 intramedullary–extramedullary, and 9 extramedullary lesions, were located dorsally or dorsolaterally (100.0%). The most common lesion location was the thoracic segment (53.8%), with most of the lesions being accompanied by syringomyelia (94.7%). Long-term follow-up (mean: 103 ± 50.4 months) for prognosis determination revealed that 88.2% (15/17) of all cases had stable or improved neurofunctional outcomes according to the McCormick grade and Odom's criteria. Only one case with VHL disease developed recurrence 4 years after surgery. Additionally, statistical analysis showed that VHL disease was an independent prognostic factor associated with deteriorating neurologic function (p = 0.015). Conclusions En bloc resection facilitated satisfactory long-term functional outcomes in patients with spinal cord hemangioblastomas. Given that VHL disease was identified as a predictor of poor long-term outcomes, regular long-term follow-up of patients with VHL-associated spinal cord hemangioblastoma seems necessary.

Publisher

Georg Thieme Verlag KG

Subject

Neurology (clinical),Surgery

Reference18 articles.

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2. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease;J E Conway;Neurosurgery,2001

3. Identification of the von Hippel-Lindau disease tumor suppressor gene;F Latif;Science,1993

4. von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease;A O Vortmeyer;Hum Pathol,1997

5. von Hippel-Lindau disease;R R Lonser;Lancet,2003

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