VON HIPPEL-LINDAU DISEASE PRESENTING AS PANCREATIC NEUROENDOCRINE TUMOR

A 21-year-old woman with a family history of von Hippel-Lindau disease presented with a mass in the head of the pancreas. Light microscopic features of the tumour suggested neuroendocrine differentiation and al though it displayed positive immunostaining for the antigens expected in a neuroendocrine neoplasm, S-100 staining was also present. This un usual feature prompted further evaluation by routine and post-embeddin g protein-A gold immunoelectron microscopy, which demonstrated the pre sence of neuroendocrine granules. Tumour cell DNA content was normal b y flow cytometry. Although this patient exhibited no other signs of vo n Hippel-Lindau disease, the presence of a pancreatic tumour with neur oendocrine differentiation demonstrated that she was affected. Future surveillance and genetic counselling will be influenced by this diagno sis.