Introduction: Hemangioblastomas in the spinal cord may occur sporadically or may be associated with von Hippel-Lindau syndrome (vHL). Clinical characteristics and the management of hemangioblastomas associated with vHL have been extensively covered in the literature. This report aims to analyze the characteristics and surgical treatment results of sporadic spinal hemangioblastomas (SSHB).
Methods: This is a retrospective analysis of 14 SSHB patients (8 males and 6 females) operated on over a 23-year period. The median age was 41.5 (24-70). vHL was excluded by clinical and imaging evaluations in all patients. This was further confirmed by genetic testing in 57.1% of the patients. All patients underwent preoperative and early postoperative MRI. All tumors were approached posteriorly with a laminectomy. After dural opening, exophytic lesions were directly approached, and intramedullary tumors were resected with a posterior midline myelotomy. Intraoperative ultrasound was used in 6 of the 8 dorsal intramedullary tumors. No adjuvant treatment was administered. The median follow-up time was 4 years (1-23). We also conducted a meta-analysis of all 271 SSHB cases reported in the English-language literature from 1967 to 2011.
Results: All lesions were solitary. Nine (64.3%) lesions were cervical, 3 (28.5%) were thoracic, and 1 (7.1%) was lumbar. Eight (57.1%) tumors were dorsal intramedullary, 4 (28.6%) were exophytic, 1 (7.1%) was intradural extramedullary and 1 (7.1%) was completely extradural. Diffuse segmental cord enlargement was present in 7 (50%) and a cyst/syrinx was present in 7 (50%) cases. The 14 patients underwent 15 operations, and gross-total resection was achieved in all operations. There was no mortality. Symptoms improved after 8 (53.3%) of 15 operations, remained the same after 5 (33.3%) and worsened after 2 (13.3%). The mean Karnofsky Performance Score improved from 79.3 (±17.5) to 87.3 (±12.2). There was one recurrence 15 years after MRI had confirmed total resection, and this patient underwent a repeat total resection with improvement in neurological status.
Conclusions: SSHB's occur most commonly as solitary lesions in the upper spinal cord. Excellent surgical results can be achieved with microsurgery alone. The long-term outcome of microsurgery treatment is good with only rare recurrences.
Patient Care: Treatment modalities of the spinal hemangioblastomas can be reviwed by this article.
Learning Objectives: Preoperative embolisation for the treatment of spinal hemangioblastoma can be used in the clinics. We aimed to show our cases to do this surgery without embolisation.