Von Hippel-Lindau disease is a hereditary tumour syndrome associated with the earlier development of a variety of benign and malignant neoplasms, such as hemangioblastomas of the central nervous system and retina, tumours of the internal ear, renal carcinoma and cysts, pheochromocytoma, neuroendocrine tumours, pancreatic cysts, epididymal and broad ligament cystadenomas in men and women respectively. Von Hippel-Lindau disease is considered to be the most common cause of hereditary renal cancer.
Paediatric pancreatic neuroendocrine tumours in von Hippel–Lindau disease