A vascular benign lesion in a perilous location: Case report

Abstract

Background: Hemangioblastomas are vascular grade 1 tumours developing sporadically or, more rarely, in the context of Von Hippel-Lindau disease. Cystic brainstem hemangioblastomas are uncommon, with only a limited number of cases documented in the literature. Even though they are benign, their surgery is challenging in view of their eloquent location and vascularity. Thorough perioperative management is required.
Case presentation: We report the case of a 47-year-old female patient with progressive weakness, difficulty in swallowing and ataxic gait. The brain MRI showed a cystic lesion located in the caudal part of the fourth ventricle and the dorsal medulla oblangata. We performed a subtotal excision of the tumour that was challenging due to its adherence to the floor of the 4th ventricle and its high vascularity. The pathology exam concluded to a hemangioblastoma. The patient presented a good evolution with no tumor progression in 6 months follow-up.
Clinical discussion: The cystic brainstem hemangioblastoma is a rare, seemingly innocuous lesio,n but highly challenging to neurosurgeons. MRI is the investigation of choice, regularly sufficient for preoperative evaluation. Still, some authors recommend the performance of a cerebral angiography or a CTA preoperatively. In literature, many strategies, such as the use of preoperative embolization or radiosurgery, were described, aiming for a better-quality surgery and prognosis.
Conclusion: Cystic brainstem hemangioblastoma is a scarce histologically benign but challenging lesion with surgical difficulties. Through our case and literature review, we concluded that surgery is the mainstay treatment for brainstem hemangioblastomas, mainly cystic ones and that a thorough study of its vascularization via angiography or CTA with a preoperative tumor embolization can help achieve a total tumor resection with good functional outcome and low mortality.