Laparoscopic organ-sparing resection of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors.

Abstract

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. 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. 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. Organ-sparing laparoscopic surgery is an important option for treating VHL-associated PNETs of the pancreatic tail.