Hemangioblastoma Clinical Presentation

Updated: Sep 26, 2017
  • Author: Konstantin V Slavin, MD; Chief Editor: Brian H Kopell, MD  more...
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Presentation

Physical Examination

The clinical presentation of hemangioblastomas usually depends on the anatomic location and growth patterns. Cerebellar lesions may present with signs of cerebellar dysfunction, such as ataxia and discoordination, or with symptoms of increased intracranial pressure due to associated hydrocephalus. [33]  In general, intracranial hemangioblastomas present with a long history of minor neurologic symptoms that, in most cases, are followed by a sudden exacerbation, which may necessitate immediate neurosurgical intervention.

Patients with spinal cord lesions most frequently present with pain, followed by signs of segmental and long-track dysfunction due to progressive compression of the spinal cord. Patients with VHL disease may present with ocular or systemic symptoms due to involvement of other organs and systems.

The polycythemia that may develop in some patients with hemangioblastomas usually is clinically asymptomatic.

Spontaneous hemorrhage is possible in both intraspinal and intracranial hemangioblastomas, [34] but this risk is low, and tumors smaller than 1.5 cm carry virtually no risk of spontaneous hemorrhage.

Doyle and Fletcher described 22 cases of hemangioblastoma arising at peripheral sites. All the tumors were solitary, except 1, and arose in the spinal nerve roots (12), kidney (3), intestine (2), orbit (1), forearm (1), peritoneum (1), periadrenal soft tissue (1), and flank (1). Five patients had von Hippel-Lindau disease, and another 5 had lesions suggestive of von Hippel-Lindau disease. [4]