P05.17 Dorsal solid cervicomedullary hemangioblastomas: surgical results in seven patients

Abstract

BACKGROUND: Hemangioblastomas are highly vascular, but rare tumors, which account for only 1.5-2.5% of intracranial neoplasms. They are predominantly found in posterior fossa and spinal cord. Cerebellar hemangioblastomas usually have 'cyst with mural nodule' appearance and dorsal cervicomedullary solid tumors are occasionally found. Total resection of tumor may lead to cure of the lesion, but the surgical resection of large solid hemangioblasoma is challenge for neurosurgeons due to their association with critical neurovascular structures and rich vascularity. We present surgical experience of dorsal solid cervicomedullary hemangioblastomas. MATERIAL AND METHODS: Among 32 patients with surgically resected infratentorial hemangioblastomas between 1998 and 2017, seven cases (5 males and 2 females) presented dorsal solid cervicomedullary hemangioblastomas. The clinical data were analyzed retrospectively. The follow-up ranged from 2 to 149 months with an average of 40.1 months. RESULTS: Mean age was 39.0 years (range, 30-50). The common presenting symptoms were headache in 4 patients, posterior neck pain and arm weakness in 3 patients respectively. Two cases presented with an acute intracranial hemorrhage and the other two patients had von Hippel-Lindau disease. Mean maximal diameter of solid nodule was 3.5 cm and 4 patients showed a large-sized mass over 4.0 cm. Ventricular enlargement and syrinx formation in upper cervical cord were noted in 5 and 3 cases respectively. Preoperative embolization of tumor was performed in four cases. All hemangioblastomas were radically removed without significant morbidity except one patient who showed a prominent peri-tumoral edema on preoperative MR imaging and insufficient preoperative tumor embolization. The syrinx disappeared immediately after operation on imaging and two patients received radiation as an adjuvant therapy during follow-up period. No patients required the permanent CSF shunt procedure. CONCLUSION: The dorsal solid cervicomedullary hemangioblastomas can be resected with good surgical outcome in most cases. Better understanding of the vascular pattern of tumor, careful preoperative embolization and en bloc excision should be required for total tumor resection without morbidity especially in the cases of large-sized cervicomedullary hemangioblastoma.