Neurological Outcome of Spinal Hemangioblastomas: An International Observational Multicenter Study About 35 Surgical Cases

Abstract

Introduction: Hemangioblastomas (HBs) are benign, highly vascular tumors that can be found intracranially or in the spinal region, representing around 2-15% of primary intramedullary tumors. They can occur sporadically or in association with Von Hipple-Lindau (VHL) disease. Despite recent of advancement of nonsurgical treatments, complete surgical resection remains the gold standard of care for the spinal HBs.

Materials and methods: We conducted an international multicenter retrospective analysis of adult patients surgically treated for spinal HBs in four European referral centers between January 2000 and September 2024, with a minimum post-operative follow-up duration of 6 months. Patients' sex and age at surgical intervention, clinical presentation, and duration symptoms prior to clinical diagnosis were identified. The pre- and post-operative neurological status at 1 and 6 months and at the last visit was assessed using the modified McCormick score (MCS). The extent of surgical resection was divided into gross total resection (GTR) and subtotal resection (STR). Finally, post-operative complications were inspected as well, namely cerebrospinal fluid leaks, infections, hemorrhages and post-operative spinal stability.

Results: A total of 35 patients were included in the cohort, with an age median of 52 years (34.5-60) and a slight male predominance (21/35, i.e., 60%). The median follow-up period was 37.5 months (12-75). More than half were located in the cervical region, making it the most common (54.3%). Syrinxes were observed in 23 cases (72%), and HBs were more commonly intramedullary (80%). GTR was achievable in around 88% of cases. Post-operative complications were observed in nine patients (25.7%). Nearly half of patients were discharged into rehabilitations centers (48.5%). Tumor recurrence was seen in 10.3% only. At the last follow-up, an excellent overall post-operative neurological status (positive ∆ McCormick) was observed in most of patients (88%) and was found to be associated with a relatively younger age group. Tumor location and presence of syrinxes did not show any statistical significance regarding clinical outcome. In patients having benefited from intra-operative monitoring, only D-wave changes showed statistical significance regarding post-operative outcome (p < 0.05).

Conclusions: A large majority of patients operated for a spinal HB demonstrated favorable outcome after surgery, with unchanged or improved neurological status. Advanced age could have an impact on the post-operative neurological outcome. Other factors such as tumor size, location, and the presence of syrinx did not seem to significantly impact the neurological outcome. Finally, the surgery of these vascular lesions with no possibility of debulking or piece-meal removal and requiring "en bloc" resection is technically demanding and should be performed by experienced teams in spine and spinal cord surgery only.

Keywords: Von Hippel–Lindau; clinical outcome; hemangioblastoma; neurological outcome; neurosurgery; spinal cord.

Figure 1

Hemangioblastoma in the cervical spine at C3–C4. A caudal satellite cyst was clearly visible with extensive intra-medullary oedema up to the medulla oblongata on sagittal T2-weighted MR sequence (A). The solid portion of the lesion was well delimited and characterized by an hyperintense enhancement on T1-weighted gadolinium MR sequence (B,C). Angiography demonstrated the hypervascularity of the lesion with tumoral blush and visualization of several feeding arteries (D). Post-operative MRI at 10 months confirmed the radical resection of the lesion with much better nice aspect of the spinal cord, no residual tumor, and absence of post-operative complication (E).

Figure 2

Intra-operative views. The tumor looked highly vascularized with abundant vascular structures on its surface (A). Coagulation of feeding vessels at the cord–tumor interface was first achieved. The resection was then conducted progressively without penetrating inside the tumor and occurred at the end of radical complete en bloc removal (B,C). The spinal cord was carefully respected during all the surgical procedure and meticulously preserved with the assistance of IONM (D). H—hemangioblastoma. SC—spinal cord.