This tumour is associated with what genetic condition?
von Hippel Lindau disease.
What are the common sites of significant pathology in von Hippel Lindau disease, and how should they be examined?
Pheochormocytoma, renal cell cancers and haemangioblastomas of the spinal cord. As such CT of the abdomen and MRI of the whole cord should be performed when the diagnosis is established.
MICROSCOPIC DESCRIPTION: The sections show a moderately hypercellular intensely vascular tumour. Vascular channels vary from large calibre thin walled sinusoidal structures to small capillaries. The latter enclose lobules of cells with moderately pleomorphic round and oval hyperchromatic nuclei and clear cytoplasm. These are consistent with stromal cells. No mitotic figures or areas of necrosis are identified. The features are of capillary haemangioblastoma. Tumour is sharply demarcated from adjacent cerebellar parenchyma and focally is seen to be attached to overlying leptomeninges.
FINAL DIAGNOSIS: haemangioblastoma.