ORGANPRESERVING AND EXTENSIVE PANCREATIC SURGERY FOR VON HIPPELLINDAU DISEASE. SIX CASES OF 45 PATIENTS UNDER SURVEILLANCE

Abstract

Full Text

Background. Pancreas is involved in 15% of patient with von Hippel-Lindau disease (VHL). Pancreatic surgery for VHL is recommended for pNENs>2 or >3 cm. Aim. To assess results of pancreatic surgery for VHL NENs. Material and Methods. Retrospective analysis of 6 pancreatic resections from 42 VHL patients under surveillance (2013-2018). Results and Discussion. First case: total duodenopancreatectomy for head and tail pNENs on the background of total pancreatic involvement by serous cystadenomas of different size was performed to the 54-year old woman, who 6 years ago was treated by right-sided nephrectomy performed for clear-cell cancer. 8 months later she had died due to dissemination of renal cancer. A 45-year old woman with multiple cerebellar and spine hemangioblastomas, pNENs in the head and body and tail, 5 years after right adrenalectomy for pheo, centre-preserving pancreatectomy and left adrenalectomy for pheo. Central pancreatic resection was performed to a 36-year old man for 2 cm NEN. A 47-year old man with multiple spine hemangioblastomas, large (5-6 cm) pNENs in the head and 10 years after bilateral adrenalectomy for pheo was successfully treated by pancreaticodudenectomy. One case of distal pancreatectomy (DP) for NENs combined with bilateral adrenalectomy and left renal resection and another case of DP combined with left adrenalectomy, triple left kidney and double right kidney resection after cranial hemanangioblastoma removal. All the patients were discharged and at the moment they are functional, working and fully compensated. Conclusions. Timely and possibly parenchyma-sparing pancreatic resections are the operations of choice for pNENs on the background of VHL.