Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Abstract

The clinical differences were evaluated in spinal hemangioblastoma between patients with sporadic disease and patients with von Hippel-Lindau (VHL) syndrome. The distribution of hemangioblastomas in the central nervous system was investigated in 56 patients treated between 1988 and 2008 at the University of Tokyo Hospital. The characteristics of spinal hemangioblastomas were compared in 35 patients including 17 with sporadic disease and 18 with VHL syndrome treated between 1988 and 2008 at our hospital and three affiliated institutions. Spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%, p º 0.001). Spinal hemangioblastomas associated with VHL syndrome were diagnosed a decade earlier (p ＝ 0.007) and were associated with less severe neurological symptoms than sporadic lesions (p ＝ 0.004). Most patients with sporadic disease had a single lesion at the cervical or thoracic level, whereas patients with VHL syndrome had multiple lesions at all spinal levels (p ＝ 0.04). Patients with sporadic disease exhibited significant improvement in postoperative neurological status (p ＝ 0.02), whereas patients with VHL syndrome had no change in status (p ＝ 1.00). Number of removed lesions (p ＝ 0.03) and lower spinal cord lesions (p ＝ 0.05) were associated with poor surgical outcome. Tumor recurrence was correlated with partial removal of lesions (p ＝ 0.05). One third of patients with VHL syndrome developed new lesions every 2 years. The major finding of this study is that the incidence of spinal hemangioblastoma, distributed through all spinal levels, may be as high as 88% in patients with VHL syndrome, which is much greater than previously reported.

Intra-extradural Dumbbell-Shaped Hemangioblastoma Manifesting as Subarachnoid Hemorrhage in the Cauda Equina

“…25) Furthermore, one third of patients with VHL develop new lesions every 2 years. 25) Accordingly, we should monitor all VHL patients closely for the de novo appearance of spinal lesions.…”

“…De novo tumors in VHL are more frequently observed in lower spinal locations, such as the cauda equina and conus medullaris as in our case, and these lesions are associated with poor surgical outcome. 25) We should remove these de novo tumors very carefully after becoming symptomatic and focus on total tumor removal to prevent tumor recurrence as far as possible.…”

Multifocal Spinal Hemangioblastoma in von Hippel-Lindau Syndrome: A Case Report and Literature Review

“…1F) (1, 5, 6). However, more recent studies have shown different results (7,8). Conway et al (8) found that the incidences of spinal hemangioblastomas were 47% in patients with VHL syndrome and 12% in patients with sporadic disease.…”

“…According to Takai et al (7), spinal lesions were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%). These results are based on the availability of high resolution imaging with high detectability of spinal lesions (7).…”

“…Other lesions include retinal angiomas, pheochromocytomas, pancreatic cysts, and epididymal cystadenomas (4). Patients with VHL syndrome-associated spinal hemangioblastomas tend to present with neurological symptoms and signs at a younger age, and have multiple small lesions (5)(6)(7). When VHL syndrome-associated spinal hemangioblastomas are represented by multiple, tiny to small lesions, they can mim- …”