CLINICAL, GENETIC AND RADIOGRAPHIC ANALYSIS OF 108 PATIENTS WITH VON HIPPEL-LINDAU DISEASE (vHL) MANIFESTED BY PANCREATIC NEUROENDOCRINE TUMORS (PNETs)

BACKGROUND—vHL is an autosomal dominant syndrome associated with tumors in multiple organs including the pancreas. Here we report on the largest single center experience with vHL PNETs. METHODS—Between 12/88 and 11/06, 633 patients with vHL were evaluated and those with PNETs were enrolled on a prospective protocol. RESULTS—One hundred eight vHL patients had PNETs (17%). Nine had metastatic disease (8.3%) from their PNET. Patients with lesions >3 cm (n=25) were more likely to develop metastases than patients with lesions ≤3 cm (n=83) (p<0.0046). Thirty-nine patients underwent resection. Germ line sequencing showed that 78% (7/9) of patients with metastases had exon 3 mutations compared with 46% (32/98) of patients without metastases (p<0.01). Tumor doubling time (TDT) was calculated for the largest PNET. The group with metastases had an average TDT of 337 days (range: 180 to 463) compared with 2628 days (range: 103 to 9614) for those without metastases (p<0.0001). CONCLUSIONS—By implementing a system of selective surgical resection based on defined criteria, vHL patients with PNETs can be safely managed. For patients with small primary lesions Address correspondence and reprint requests to: Steven K. Libutti, M.D., Head, Tumor Angiogenesis Section, Surgery Branch, National Cancer Institute, NIH, 10 Center Drive, CRC 4W-5940, Bethesda, MD 20892, Telephone: 301-496-5049, libuttis@mail.nih.gov, Fax: 301-402-1788. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. HHS Public Access Author manuscript Surgery. Author manuscript; available in PMC 2019 October 01. Published in final edited form as: Surgery. 2007 December ; 142(6): 814–818.e2. doi:10.1016/j.surg.2007.09.012. A uhor M anscript

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