BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are a characteristic 
feature of the tumor syndromes multiple endocrine neoplasia type 1 (MEN-1) and 
von Hippel-Lindau disease (VHL). With VHL, about 10% of the patients exhibit 
PNETs by age 40 years. Metastatic potential is high if the tumors have grown to 
>3 cm in diameter. Optimal surgical treatment is still a challenge.
METHODS: We report three cases, all women, ages 22, 30, and 39 years, 
respectively, who had known VHL, confirmed by classic organ manifestations and 
germline mutations of the VHL gene. All were diagnosed, in an asymptomatic 
stage, with solid tumors of the pancreatic tail or tail/corpus area measuring 
2.9-5.6 cm diameter. All accepted the offer of laparoscopic organ-sparing 
removal of the tumors.
RESULTS: In all three cases, the tumor was entirely removed. In two cases, 
resection of the spleen was also necessary as dissection of the tumor from the 
major splenic vessels was impossible. Operating time was 215-365 min, and blood 
loss was 200-700 ml. Histolopathology revealed benign PNETs in two cases, but 
the third patient had regional lymph node metastases. There were no 
complications, and the hospital stay was 4-7 days.
CONCLUSIONS: Organ-sparing laparoscopic surgery is an important option for 
treating VHL-associated PNETs of the pancreatic tail.