“ Autosomal dominant inheritance. Rare syndrome giving rise to multiple benign and malignant tumours affecting multiple systems. Associated...
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Autosomal dominant inheritance. Rare syndrome giving rise to multiple benign and malignant tumours affecting multiple systems. Associated with renal cell carcinoma (most common cause of mortality), pancreatic cancer, pheochromocytoma, benign cysts and renal/hepatic angiomas. Haemangioblastomas are characteristic in the nervous system.
MRI
• Look for haemangioblastoma (cerebellar—70%, retinal—50%, spinal—10%), a cystic lesion with an enhancing mural nodule. They can be solid.
• These are highly vascular tumours at risk of haemorrhage.
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