Tumors associated with Von Hippel-Lindau disease
Cerebellar hemangioblastoma
see Cerebellar hemangioblastoma associated with Von Hippel-Lindau disease.
Spinal cord hemangioblastomas
see Spinal cord hemangioblastoma.
Brainstem hemangioblastomas
see Brainstem hemangioblastoma.
Pheochromocytomas
see Pheochromocytoma
Endolymphatic sac tumors
Retinal hemangioblastomas
Renal cell carcinoma
see Renal cell carcinoma associated with Von Hippel-Lindau disease.
Renal cysts
a) 50–70% of Von Hippel-Lindau disease patients have bilateral and multiple renal cysts
b) rarely cause profound renal impairment
c) chronic renal failure or renal hypertension not as common as with polycystic kidney disease
Epididymal cystadenomas
a) benign lesions that arise from the epididymal duct
b) found in 10–60% of male VHL patients
c) typically appear in the teenage years
d) may cause infertility if bilateral
e) may be multiple
Broad ligament cystadenomas
a) arise from the embryonic mesonephric duct
b) true incidence unknown
c) rarely reported and usually not recognized in women with VHL
Pancreatic neuroendocrine tumors and cysts
a) 35 to 70% of patients with VHL develop an endocrine tumor or cyst
b) pancreatic cysts are generally asymptomatic and often multiple
c) pancreatic neuroendocrine tumors are usually non-functional and 8% of them are malignant
d) differential diagnosis: pancreatic islet cell tumors, MEN2
Sinonasal renal cell-like adenocarcinoma
Sinonasal renal cell-like adenocarcinoma is an emerging tumor associated with VHL syndrome and it is hoped that future studies shed light on the underlying biology of this unique tumor 1).