Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report

Abstract

Spinal and cerebellar hemangioblastomas are common in von Hippel-Lindau disease (vHLD) and usually treated surgically. Multifocal presence and surgically not amenable locations are issues that require a combined microsurgical and radiosurgical approach to control complex cases. We would like to present the case of a 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Combined microsurgical approaches to two spinal lesions and the cerebellar lesion followed by external beam radiotherapy of the posterior fossa and the whole spinal axis stabilized tumor growth of the asymptomatic lesions, while no recurrent tumors were detected at the site of surgery. A clinical deterioration connected to early postoperative deficits stabilized to a moderate gait ataxia. The follow-up after radiotherapy covered 60 months. A combination of microsurgery and radiosurgery for the surgically not amenable lesions is an adequate treatment regimen to stabilize tumor growth and clinical symptoms of multifocal spinal hemangioblastomas in vHLD, though the therapy should be limited to symptomatic or growing lesions.

Keywords: hemangioblastoma; holocord syrinx; microsurgery; radiotherapy; syringomyelia; von Hippel-Lindau.

Fig. 1

Magnetic resonance imaging scans of the cervical spine on first clinical onset of symptoms, T2-weighted (A), and contrast-enhanced T1-weighted (B) images showing an extended syrinx formation due to a large contrast enhancing formation at the cervicothoracic junction.

Fig. 2

Magnetic resonance imaging scans of the thoracic spine on first clinical onset of symptoms. T2-weighted ( A ) and contrast enhanced T1-weighted ( B ) images further demonstrate the extent of the syrinx down to the conus medullaris among multiple contrast enhancing lesions.

Fig. 3

Magnetic resonance imaging scans of the cervical spine 60 months after radiotherapy. T2-weighted ( A ) and contrast-enhanced T1-weighted ( B ) images demonstrate a considerable collapse of the cervical portion of the syrinx after resection of the cervicothoracic hemangioblastoma.

Fig. 4

Magnetic resonance imaging scans of the thoracic spine 60 months after radiotherapy. T2-weighted ( A ) and contrast-enhanced T1-weighted ( B ) images show partial collapse of the thoracic portion of the syrinx and stable-sized contrast enhancing lesions after radiotherapy.