Figure 1
The patient presented with a history of headache over several years. Ataxia had developed recently.
Cerebellar haemangioblastoma
The patient presented with a history of headache over several years. Ataxia had developed recently. The diagnostic work-up, investigating a possible abnormality in the cerebellum, included CT and MRI examinations. These showed a cystic lesion with an enhancing mural nodule in the cerebellum. The findings suggested cerebellar haemangioblastoma as the most likely diagnosis.
Haemangioblastoma is a benign autosomal dominant tumour of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumours, arising most frequently in the cerebellar hemispheres and vermis. These tumours may also occur in the brainstem, spinal cord, retina, and cerebrum. Histologically, the tumours are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. They are usually solitary, but can be multiple (in 10% of cases).
Cerebellar haemangioblastomas usually present in the third decade with ataxia and discoordination, or with symptoms of increased intracranial pressure due to associated hydrocephalus. The average age of presentation in adulthood is 33 and there is a male preponderance. In childhood cerebellar haemangioblastomas are usually seen in association with von Hippel-Lindau disease and there is a female preponderance. Erythrocythemia occurs in 20% of cases.
CT may be used in the investigation of cerebellar haemangioblastoma, but MRI is the preferred imaging study. Typical findings on imaging are: a cystic mass (two-thirds of cases) or solid tumour (one-third of cases); homogenous enhancement of the mural nodule (50% of cases); and occasional enhancement of the cystic wall. The lesion almost never calcifies. Angiograghy shows dense staining of the cystic wall and nidus. Differential diagnosis includes: astrocytoma (>5cm, calcifications, no angiographic contrast blush of mural nodule, no erythrocythemia) and metastasis.
Haemangioblastoma, although can cause mass effect with obstructive hydrocehpalusand intracranial hypertension, is a treatable and curable disease. The standard treatment is surgical resection and this should be offered to the patient unless the risk of the operation outweighs its potential benefits. Other therapeutic modalities include endovascular embolisation of the solid component of the tumour, which may decrease the vascularity of the tumour and lower blood loss during its resection, and stereotactic radiosurgery of the tumour using either a linear accelerator or a gamma knife. Post-resection long-term recurrence rates seem to depend on the presence of VHL disease and multicentric lesions
References
[1]
Grainger RG, Allison DJ.
Diagnostic Imaging, Third Edition.
Churchill Livingstone, London, p 2075 (1997).
[2]
Sutton D.
Textbook of Radiology and Imaging, Sixth Edition.
Churchill Livingstone, New York, pp 1614-15 (1998).
[3]
Osborn AG
Diagnostic Neuroradiology,Mosby,London pp 606-607[1994].
Case information
| URL: | https://www.eurorad.org/case/1683 |
| DOI: | 10.1594/EURORAD/CASE.1683 |
| ISSN: | 1563-4086 |
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