Rapid Worsening of Symptoms and High Cell Proliferative Activity in Intra- and Extramedullary Spinal Hemangioblastoma: A Need for Earlier Surgery

Abstract

Study design: A retrospective analysis of a prospective database.

Objective: To compare preoperative symptoms, ambulatory ability, intraoperative spinal cord monitoring, and pathologic cell proliferation activity between intramedullary only and intramedullary plus extramedullary hemangioblastomas, with the goal of determining the optimal timing for surgery.

Methods: The subjects were 28 patients (intramedullary only in 23 cases [group I] and intramedullary plus extramedullary in 5 cases [group IE]) who underwent surgery for spinal hemangioblastoma. Preoperative symptoms, ambulatory ability on the McCormick scale, intraoperative spinal cord monitoring, and pathologic findings using Ki67 were compared between the groups.

Results: In group IE, preoperative motor paralysis was significantly higher (100 versus 26%, p < 0.005), the mean period from initial symptoms to motor paralysis was significantly shorter (3.5 versus 11.9 months, p < 0.05), and intraoperative spinal cord monitoring aggravation was higher (65 versus 6%, p < 0.05). All 5 patients without total resection in group I underwent reoperation. Ki67 activity was higher in group IE (15% versus 1%, p < 0.05). Preoperative ambulatory ability was significantly poorer in group IE (p < 0.05), but all cases in this group improved after surgery, and postoperative ambulatory ability did not differ significantly between the two groups.

Conclusions: Intramedullary plus extramedullary spinal hemangioblastoma is characterized by rapid preoperative progression of symptoms over a short period, severe spinal cord damage including preoperative motor paralysis, and poor gait ability compared with an intramedullary tumor only. Earlier surgery with intraoperative spinal cord monitoring is recommended for total resection and good surgical outcome especially for an IE tumor compared with an intramedullary tumor.

Keywords: ambulatory ability; early surgery; preoperative motor paralysis; progress of preoperative symptom; spinal hemangioblastoma in intramedullary and extramedullary location.

Fig. 1

(A) Characteristic magnetic resonance imaging findings for intramedullary hemangioblastoma at C5 include a wide high-intensity area on the craniocaudal sides of the tumor on T2-weighted imaging. (B) Syrinxes naturally disappear postoperatively after total tumor resection, and shunting is not needed. (C) A clearly and densely contrasted tumor on contrast T1-weighted imagining (T1WI) as a “focal sign” is seen in intramedullary location only. (D) An intramedullary plus extramedullary hemangioblastoma showing a characteristic “snowman sign” on contrast T1WI. An extramedullary tumor occupied all the spinal canal in all cases in the intramedullary plus extramedullary hemangioblastoma group, and the spinal cord was severely compressed from the intramedullary and extramedullary sides.

Fig. 2

The percentage of cases with stable independent ambulation (McCormick classes I and II) preoperatively was significantly lower in the intramedullary plus extramedullary hemangioblastoma group (group IE) than in the intramedullary group (group I).

Fig. 3

Better preoperative ambulatory ability was significantly associated with good postoperative ambulation, with probabilities of achieving good postoperative results for patients in preoperative classes I and II of 79% in all patients, 78% in the intramedullary only group (group I), and 100% in the intramedullary plus extramedullary group (group IE). In contrast, improvement of postoperative independent ambulatory ability was difficult for cases with severe preoperative symptoms, with probabilities for patients in preoperative classes III, IV, and V of 22% in all patients, 20% in group I, and 25% in group IE.

Fig. 4

The percentage of cases with stable independent ambulation (McCormick classes I and II) postoperatively did not differ significantly between groups I and IE, although there was a significant difference preoperatively. Abbreviation: group I, intramedullary only; group IE, intramedullary plus extramedullary; NS, not significant.