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Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: institutional experience on their similarities and differences

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Abstract

Introduction

Hemangioblastomas are uncommon tumors of the central nervous system that can be seen in Von Hippel-Lindau (VHL) disease. Despite their benign histology, hemangioblastomas can cause substantial morbidity due to involvement of critical structures. In order to better understand the clinical behavior of spinal cord hemangioblastomas, we have analyzed the clinical, pathologic, radiologic characteristics and management of sporadic and VHL-associated cases at our institution.

Methods

We performed a database search to identify all spinal hemangioblastomas at our institution between 1997 and 2016. Tumor characteristics were analyzed for sporadic and VHL-associated tumors separately in order to understand the differences in groups.

Results

We included 20 patients with VHL-associated spinal hemangioblastomas, and 22 patients with sporadic spinal hemangioblastomas. VHL-associated patients were significantly younger at time of presentation compared to sporadic patients (p < 0.0025). Thirty-two patients (76.2%) presented with focal weakness, 34 (81.0%) with sensory loss, and 22 (52.4%) with pain. VHL patients were more likely to present with multiple symptoms (p < 0.001). Median follow-up time was 20.9 months, during which 17 tumors recurred. The median recurrence free interval was 44 months. There were no differences in gross total resection rates between sporadic and VHL-associated cases (p = 0.197). VHL-associated cases had a higher rate of repeat surgery for recurrence (14 patients-73.6%) compared to sporadic cases (3 patients-13.6%; p < 0.001).

Conclusion

VHL-associated spinal hemangioblastomas differ from sporadic tumors in terms of age, presenting symptoms, multifocality, and rate of recurrence. Recurrences seem to be unrelated to the extent of resection, indicating the need for life-long follow up for VHL patients.

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Authors and Affiliations

  1. Department of Neurological Surgery, UCSF School of Medicine, 505 Parnassus Avenue, San Francisco, CA, 94143, USA

    Andrew Yousef & Martin J. Rutkowski

  2. Division of Neuropathology, UCSF School of Medicine, 505 Parnassus Avenue, Room M551, San Francisco, CA, 94143, USA

    Can Ege Yalcin, Ozgur Can Eren, Ilay Caliskan & Tarik Tihan

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  1. Andrew Yousef
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  2. Martin J. Rutkowski
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  3. Can Ege Yalcin
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Correspondence to Andrew Yousef.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Yousef, A., Rutkowski, M.J., Yalcin, C.E. et al. Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: institutional experience on their similarities and differences. J Neurooncol 143, 547–552 (2019). https://doi.org/10.1007/s11060-019-03189-w

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  • DOI: https://doi.org/10.1007/s11060-019-03189-w

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