Presentation
Transferred for neurosurgery.
Patient Data
Right paramedian cystic cerebellar mass.
3 cm cystic mass in the right paramedian posterior fossa compressing the 4th ventricle with focal nodular wall enhancement
lobulated cystic mass extending inferiorly through the foramen magnum compressing the medulla and spinal cord
symmetrical differential function.
minor distension R upper pole calices.
multiple solid renal tumors and hyper-attenuating cysts.
expected post-op findings
surgical clips indicate the multiple parenchyma-preserving partial nephrectomies
pancreas enlarged by multiple simple cysts
V-P shunt
post surgical changes
CSF tumor seeding over the cerebellar folia
two enhancing intramedullary tumors, each associated with a syrinx
enlarged spinal arteries associated with the thoracic lesion
numerous enhancing foci over the pial surface of the spinal cord indicating seeding
multiple areas of leptomeningeal thickening and enhancement
Case Discussion
histology confirmed posterior fossa and spinal hemangioblastomas
5 renal tumors excised with clear cell RCCs histology, (4 papillary)
Hemangioblastoma is a common presentation of Von Hippel-Lindau disease and RCC is a common cause of death. This man also has a retinal angioma.