• Louis de Mestier, Sébastien Gaujoux, Jérôme Cros, Olivia Hentic, Marie-Pierre Vullierme, Anne Couvelard, Guillaume Cadiot, Alain Sauvanet, Philippe Ruszniewski, Stéphane Richard, and Pascal Hammel.
• *Department of Gastroenterology and Pancreatology, Beaujon Hospital, Clichy, France; †Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré Hospital, Reims-Champagne-Ardennes University, Reims, France; ‡Department of Hepato-Pancreatico-Biliary Surgery, Beaujon Hospital, Clichy, France; §Denis-Diderot University, Paris, France; Departments of ¶Pathology, and ‖Radiology, Beaujon Hospital, Clichy, France; **Department of Pathology, Bichat Hospital, Paris, France; ††Expert National Center for Rare Cancers PREDIR, INCa/APHP, Bicêtre Hospital, Le Kremlin-Bicêtre, France; and ‡‡Oncogenetics EPHE Laboratory and INSERM U753, Faculty of Medicine Paris Sud, Le Kremlin-Bicêtre, France.
• Ann. Surg. 2015 Aug 1; 262 (2): 384-8.

BackgroundManagement of pancreatic neuroendocrine tumors (PNETs) associated with von Hippel-Lindau disease (VHL) is challenging because of the malignant potential and difficulty in predicting prognosis.ObjectiveCompare the long-term outcome of resected VHL-PNET and sporadic PNET.MethodsData of all patients with VHL (n = 23) operated on for nonmetastatic PNET were reviewed. Patient characteristics and recurrence-free survival rates were compared with those in patients operated on for sporadic PNET, matched for tumor size, stage, and Ki-67 index.ResultsPatients in both groups had similar demographic characteristics, except that patients with VHL were younger (36 vs 56 years, P < 0.0001). Median tumor size was 30 mm. Median Ki-67 index was 3% and 4% in the VHL and sporadic groups (P = 0.95), respectively, and lymph node metastases were present in 43% and 30% of cases, respectively (P = 0.45). Sixteen (70%) patients with VHL had multiple PNET; lesions less than 15 mm were left in place in 11 patients. Median postoperative follow-up was 107 months (interquartile range, 57-124 months) and 71 months (interquartile range, 58-131 months) in the VHL and control groups, respectively. Median recurrence-free survival could not have been estimated in the VHL group due to the low number of events (hazard ratio, 5.6; 95% confidence interval, 1.4-22.6; P = 0.013). Five patients with VHL died (3 from VHL-related tumors including 1 from PNET), whereas only one control patient died due to unrelated causes.ConclusionsThe long-term outcome of resected VHL-PNET is better than that of sporadic PNET. PNET less than 15 mm left in place did not progress. A parenchyma-sparing surgical strategy seems appropriate in patients with VHL-PNET, who may develop more life-threatening tumors of other organs.

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