Diagnosis and surgical treatment of brainstem hemangioblastomas

Abstract: Objective: The study aims to elucidate the advance of diagnosis and surgical treatment of brainstem hemangioblastomas (BSHs). Methods: The data of the following patients treated in one institute were retrospectively analyzed: (1) patients with a single tumor on the brainstem which was verified by surgery and pathology; (2) patients without von Hippel-Lindau disease or multiple hemangioblastomas. Results: Thirty-three patients with BSHs were identified, accounting for 15.5% of all intracranial hemangioblastomas surgically treated from August 1989 to May 2002 in Huashan Hospital. There were 17 males and 16 females. The patients were aged from 16 to 65 years with an average age of 45 years. The clinical manifestations were nonspecific. Magnetic resonance imaging and digital subtraction angiography were the major diagnostic modalities. Tumors were located on oblongata (14), ponto-oblongata (9), pons (6), and cervicomedulla (4). Tumors were solid in 29 cases, cyst in 4 cases, and had a small size in 5 (≤3 cm), large in 19 (3.1-4 cm), and giant in 9 (>4 cm). Extra-brainstem (EBS) type (including the fourth-ventricle hemangioblastomas) was seen in 25 cases, and intrabrainstem (IBS) type in 8 cases. Preoperative embolization was performed in 12 cases since 1996. Mild hypothermia with or without hypotension was done during the operation in 10 cases. Total tumor removal was achieved in 31 patients (94%), and incomplete removal in 2 cases. Two patients with EBS type and giant solid tumors died after operation. Follow-up study (range, 1-12 years; mean, 5 years) was available in 31 patients. Karnofsky performance scale scores were ≥80 in 25 patients (80.6%), 60 to 70 in 4 patients (12.9%), and 40 to 50 in 2 patients (6.5%). Conclusion: Two types of BSHs can be identified. Patients with cystic IBS type could obtain excellent outcome after operations. Patients with giant or large solid BSHs remain a challenge to neurosurgeons. A combined strategy of preoperative embolization, mild hypothermia with or without hypotension, microsurgical technique, and intensive perioperative management are mandatory for removal of these kinds of tumors with acceptable morbidity and mortality. [Copyright &y& Elsevier]