Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs)

Joseph A. Blansfield, Lynda Choyke, Shane Y. Morita, Peter L. Choyke, James F. Pingpank, H. Richard Alexander, Geoffrey Seidel, Yvonne Shutack, Nargiza Yuldasheva, Michelle Eugeni, David L. Bartlett, Gladys M. Glenn, Lindsay Middelton, W. Marston Linehan, Steven K. Libutti

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149 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)814-818.e2
JournalSurgery
Volume142
Issue number6
DOIs
StatePublished - Dec 2007

ASJC Scopus subject areas

  • Surgery

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    Blansfield, J. A., Choyke, L., Morita, S. Y., Choyke, P. L., Pingpank, J. F., Alexander, H. R., Seidel, G., Shutack, Y., Yuldasheva, N., Eugeni, M., Bartlett, D. L., Glenn, G. M., Middelton, L., Linehan, W. M., & Libutti, S. K. (2007). Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs). Surgery, 142(6), 814-818.e2. https://doi.org/10.1016/j.surg.2007.09.012