neurosurgery-basics

Hemangioblastoma

A hemangioblastoma (synonym: angioblastoma) is a rare, benign (WHO Grade I) tumor that is most located in the cerebellum, brainstem, or spinal cord. Typically, these tumors consist of a solid, highly vascular nodule with surrounding cystic components. Hemangioblastomas can occur sporadically but may also be associated with von Hippel-Lindau-Czermak syndrome (VHLCS).

Table Of Contents
  1. Epidemiology
  2. Clinical presentation
  3. Radiological findings
  4. Differential diagnoses
  5. Indication for surgery
  6. Outcome
  7. References

Epidemiology

Hemangioblastomas account for 7–12% of all tumors in the posterior cranial fossa and 1–2.5% of all brain tumors. In about 80% of cases, they occur infratentorially in adults. The peak incidence is between 30 and 40 years of age. Patients under 30 years with hemangioblastoma should be screened for von Hippel-Lindau-Czermak syndrome (retinal angiomas, cerebellar or spinal hemangioblastomas, renal cell carcinomas). VHLCS is present in about 5–30% of all patients with cerebellar hemangioblastomas—and in about 80% with spinal localization.

Clinical presentation

Typical symptoms include headaches, nausea, vomiting, gait and balance disturbances, double vision, and impaired fine motor skills or coordination. Accompanying polycythemia may be present due to increased erythropoietin production (in about 40% of cases).

Radiological findings

On MRI with contrast, a typical finding is an enhancing nodule with cystic components (in about 60%). The surrounding brain parenchyma is displaced. The fluid-filled cyst usually has no septa. Serpentine altered vessels around the nodule may be present (usually only visible via angiography). There is no enhancement of the cyst wall – a possible differential diagnostic feature compared to pilocytic astrocytoma. When located at the tentorium, it may resemble a tentorial meningioma but lacks a “dural tail.” Multiple hemangioblastomas usually occur only in the presence of VHLCS.

Differential diagnoses

  • Cerebral metastasis
  • Pilocytic astrocytoma
  • Medulloblastoma
  • Ependymoma
  • Choroid plexus carcinoma
  • Tentorial meningioma

Indication for surgery

The treatment of choice is microsurgical resection. The enhancing nodule must be completely resected. The cyst wall is not neoplastically altered and therefore does not need to be removed. Preoperative embolization can be considered in selected cases but is not standard. Radiotherapy can be used for inoperable or recurrent tumors.

Outcome

Surgical resection aims for a cure. The 5-year survival rate is about 85%.

References

  • S3 Guidelines of the German Cancer Society and the AWMF
Back to Database