Clinical Management of Central Nervous System Hemangioblastomas

Central nervous system hemangioblastomas are rare, benign vascular tumours predominantly found in the cerebellum, brainstem and spinal cord. They may arise sporadically or in the setting of von Hippel–Lindau (VHL) disease, an autosomal dominant syndrome marked by multisystem tumours and cysts. Clinical management hinges on accurate diagnosis through contrast-enhanced MRI, vigilant surveillance of asymptomatic lesions and timely intervention for symptomatic or rapidly growing tumours. Surgical resection remains the mainstay of treatment, aiming for gross total removal with minimal morbidity. In selected cases—particularly for deep-seated or multiple lesions—stereotactic radiosurgery (SRS) and fractionated radiotherapy offer non-invasive alternatives. Long-term follow-up is essential, as growth patterns are often unpredictable and recurrences may occur years after initial treatment. Recent advances in microsurgical techniques, neuronavigation, intraoperative angiography and endovascular embolisation have enhanced surgical safety. Molecular insights into angiogenic pathways promise targeted therapeutics in the future, while multidisciplinary care pathways optimise neurological outcomes and quality of life.

Research from Nature Portfolio

A large-scale analysis of national cancer registry data evaluated survival outcomes across treatment modalities. It confirmed that gross total resection confers the greatest overall survival advantage, whereas stereotactic radiosurgery achieves local control comparable to subtotal resection. External-beam radiotherapy was associated with inferior survival outcomes compared to surgery or SRS. Age over 40, comorbidities and brainstem location emerged as independent predictors of poorer prognosis. These findings reinforce the primacy of complete surgical excision where feasible, support the role of radiosurgical techniques for selected patients and underline the need for age- and location-adapted treatment algorithms.

Clinical Management of Central Nervous System Hemangioblastomas publication trend

The graph below shows the total number of articles in clinical management of central nervous system hemangioblastomas across all publications each year (not limited to Nature Index journals).

Technical terms

Central nervous system (CNS): The brain and spinal cord, protected by the skull and vertebral column.

Hemangioblastoma: A benign, highly vascular tumour composed of capillary networks and stromal cells.

von Hippel–Lindau (VHL) disease: A hereditary syndrome characterised by VHL gene mutations and predisposition to multiple tumours.

Gross total resection (GTR): Surgical removal of all visible tumour tissue.

Stereotactic radiosurgery (SRS): A non-invasive, high-precision radiation technique targeting intracranial lesions.

Progression-free survival (PFS): The length of time during and after treatment that a patient lives without tumour growth.

References

1. Predictors of Progression in a Series of 81 Adult Patients Surgically Managed for an Intracranial Hemangioblastoma: Implications for the Postoperative Follow-Up. Cancers (2024).
2. Radiosurgically Treated Recurrent Cerebellar Hemangioblastoma: A Case Report and Literature Review. Current Oncology (2024).
3. Management Strategies and Outcomes for VHL-related Craniospinal Hemangioblastomas. Journal of Kidney Cancer (2017).
4. Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment. Child's Nervous System (2020).
5. The clinical outcome of intracranial hemangioblastomas treated with linac-based stereotactic radiosurgery and radiotherapy. Journal of Radiation Research (2014).
6. Assessment of care pattern and outcome in hemangioblastoma. Scientific Reports (2018).