BACKGROUND: von Hippel-Lindau (vHL) disease is an autosomal dominant syndrome associated with neoplasms in multiple organs, which includes the pancreas. Here, we report the greatest single center experience in patients with vHL pancreatic endocrine neoplasm (PNETs). METHODS: Between December 1998 and November 2006, 633 patients with vHL were evaluated and those with PNETs were enrolled on a prospective protocol. RESULTS: Overall, 108 vHL patients had PNETs (17%). Nine patients had metastatic disease (8.3%) from their PNET. Patients with lesions greater than 3 cm (n = 25) were more likely to develop metastases than patients with lesions less than 3 cm (n = 83) (P < .005). Thirty-nine patients underwent resection. Germline sequencing showed that 78% of patients with metastases (7/9) had exon 3 mutations compared with 46% of patients without metastases (32/98; P < .01). Tumor doubling time was calculated for the largest PNET. The group with metastases had an average tumor doubling time of 337 days (range, 180-463 days) compared with 2630 days (range, 103-9614 days) for those without metastases (P < .0001). CONCLUSIONS: By implementing a system of selective operative resection based on defined criteria, vHL patients with PNETs can be managed safely. For patients with small primary lesions (<3 cm), without a mutation of exon 3 and slow tumor doubling time (>500 days), a nonoperative approach may be appropriate for these nonfunctional neoplasms.
BACKGROUND:von Hippel-Lindau (vHL) disease is an autosomal dominant syndrome associated with neoplasms in multiple organs, which includes the pancreas. Here, we report the greatest single center experience in patients with vHL pancreatic endocrine neoplasm (PNETs). METHODS: Between December 1998 and November 2006, 633 patients with vHL were evaluated and those with PNETs were enrolled on a prospective protocol. RESULTS: Overall, 108 vHLpatients had PNETs (17%). Nine patients had metastatic disease (8.3%) from their PNET. Patients with lesions greater than 3 cm (n = 25) were more likely to develop metastases than patients with lesions less than 3 cm (n = 83) (P < .005). Thirty-nine patients underwent resection. Germline sequencing showed that 78% of patients with metastases (7/9) had exon 3 mutations compared with 46% of patients without metastases (32/98; P < .01). Tumor doubling time was calculated for the largest PNET. The group with metastases had an average tumor doubling time of 337 days (range, 180-463 days) compared with 2630 days (range, 103-9614 days) for those without metastases (P < .0001). CONCLUSIONS: By implementing a system of selective operative resection based on defined criteria, vHLpatients with PNETs can be managed safely. For patients with small primary lesions (<3 cm), without a mutation of exon 3 and slow tumor doubling time (>500 days), a nonoperative approach may be appropriate for these nonfunctional neoplasms.
Authors: James R Howe; Nipun B Merchant; Claudius Conrad; Xavier M Keutgen; Julie Hallet; Jeffrey A Drebin; Rebecca M Minter; Terry C Lairmore; Jennifer F Tseng; Herbert J Zeh; Steven K Libutti; Gagandeep Singh; Jeffrey E Lee; Thomas A Hope; Michelle K Kim; Yusuf Menda; Thorvardur R Halfdanarson; Jennifer A Chan; Rodney F Pommier Journal: Pancreas Date: 2020-01 Impact factor: 3.327
Authors: Simon Turcotte; Baris Turkbey; Stephanie Barak; Steven K Libutti; H Richard Alexander; W Marston Linehan; Marybeth S Hughes; Naris Nilubol; Krisana Gesuwan; Corina Millo; Martha Quezado; Peter L Choyke; Electron Kebebew; Giao Q Phan Journal: Surgery Date: 2012-10-27 Impact factor: 3.982
Authors: Michael Charlesworth; Caroline S Verbeke; Gavin A Falk; Matthew Walsh; Andrew M Smith; Gareth Morris-Stiff Journal: J Gastrointest Surg Date: 2012-02-28 Impact factor: 3.452
Authors: H-C Jennifer Shen; Asha Adem; Kris Ylaya; Arianne Wilson; Mei He; Dominique Lorang; Stephen M Hewitt; Klaus Pechhold; David M Harlan; Irina A Lubensky; Laura S Schmidt; W Marston Linehan; Steven K Libutti Journal: PLoS One Date: 2009-04-02 Impact factor: 3.240