Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease

Diagnostic and management recommendations

S. K. Libutti, P. L. Choyke, D. L. Bartlett, H. Vargas, M. Walther, I. Lubensky, G. Glenn, W. M. Linehan, H. R. Alexander, M. J. Demeure, J. A. Norton, B. K. Kinder, O. H. Clark, G. B. Talpos

Research output: Contribution to journalArticle

150 Citations (Scopus)

Abstract

Background. von Hippel Lindau disease (VHL) is an inherited syndrome characterized by tumors of the kidney, adrenal, central nervous system, and pancreas. The incidence and natural history of pancreatic neuroendocrine tumors occurring in VHL are not known. Methods. From December 1988 through November 1997, 256 patients with VHL, were screened with imaging studies, and these data were reviewed from a prospective database. Results. Thirty (12%) of 256 patients had solid pancreatic lesions consistent with neuroendocrine tumors. Fourteen patients underwent resection, and 4 with metastases on imaging studies underwent biopsy only. Of the 14 patients who underwent resection, 11 remain free of disease, 2 have experienced recurrence, and 1 has died of unrelated causes (mean follow-up, 25 months; range, 3 to 73 months). The size of the primary tumor (median, 5 cm; range, 3 to 8 cm) in patients with liver metastases was significantly larger than the size of the primary tumor (median, 2 cm; range, 1 to 5 cm) in patients without liver metastases (P = .0013). Conclusions. Solid pancreatic lesions were detected in 12% of patients with VHL. Larger primary tumors were associated with liver metastases. Pancreatic imaging to identify neuroendocrine tumors and resection when they reach 2 to 3 cm may prevent the development of hepatic metastases.

Original languageEnglish (US)
Pages (from-to)1153-1159
Number of pages7
JournalSurgery
Volume124
Issue number6
DOIs
StatePublished - 1998
Externally publishedYes

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von Hippel-Lindau Disease
Neuroendocrine Tumors
Disease Management
Neoplasm Metastasis
Liver
Neoplasms
Pancreas
Central Nervous System
Databases
Kidney
Biopsy
Recurrence
Incidence

ASJC Scopus subject areas

  • Surgery

Cite this

Libutti, S. K., Choyke, P. L., Bartlett, D. L., Vargas, H., Walther, M., Lubensky, I., ... Talpos, G. B. (1998). Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease: Diagnostic and management recommendations. Surgery, 124(6), 1153-1159. https://doi.org/10.1067/msy.1998.91823

Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease : Diagnostic and management recommendations. / Libutti, S. K.; Choyke, P. L.; Bartlett, D. L.; Vargas, H.; Walther, M.; Lubensky, I.; Glenn, G.; Linehan, W. M.; Alexander, H. R.; Demeure, M. J.; Norton, J. A.; Kinder, B. K.; Clark, O. H.; Talpos, G. B.

In: Surgery, Vol. 124, No. 6, 1998, p. 1153-1159.

Research output: Contribution to journalArticle

Libutti, SK, Choyke, PL, Bartlett, DL, Vargas, H, Walther, M, Lubensky, I, Glenn, G, Linehan, WM, Alexander, HR, Demeure, MJ, Norton, JA, Kinder, BK, Clark, OH & Talpos, GB 1998, 'Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease: Diagnostic and management recommendations', Surgery, vol. 124, no. 6, pp. 1153-1159. https://doi.org/10.1067/msy.1998.91823
Libutti, S. K. ; Choyke, P. L. ; Bartlett, D. L. ; Vargas, H. ; Walther, M. ; Lubensky, I. ; Glenn, G. ; Linehan, W. M. ; Alexander, H. R. ; Demeure, M. J. ; Norton, J. A. ; Kinder, B. K. ; Clark, O. H. ; Talpos, G. B. / Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease : Diagnostic and management recommendations. In: Surgery. 1998 ; Vol. 124, No. 6. pp. 1153-1159.
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abstract = "Background. von Hippel Lindau disease (VHL) is an inherited syndrome characterized by tumors of the kidney, adrenal, central nervous system, and pancreas. The incidence and natural history of pancreatic neuroendocrine tumors occurring in VHL are not known. Methods. From December 1988 through November 1997, 256 patients with VHL, were screened with imaging studies, and these data were reviewed from a prospective database. Results. Thirty (12{\%}) of 256 patients had solid pancreatic lesions consistent with neuroendocrine tumors. Fourteen patients underwent resection, and 4 with metastases on imaging studies underwent biopsy only. Of the 14 patients who underwent resection, 11 remain free of disease, 2 have experienced recurrence, and 1 has died of unrelated causes (mean follow-up, 25 months; range, 3 to 73 months). The size of the primary tumor (median, 5 cm; range, 3 to 8 cm) in patients with liver metastases was significantly larger than the size of the primary tumor (median, 2 cm; range, 1 to 5 cm) in patients without liver metastases (P = .0013). Conclusions. Solid pancreatic lesions were detected in 12{\%} of patients with VHL. Larger primary tumors were associated with liver metastases. Pancreatic imaging to identify neuroendocrine tumors and resection when they reach 2 to 3 cm may prevent the development of hepatic metastases.",
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T2 - Diagnostic and management recommendations

AU - Libutti, S. K.

AU - Choyke, P. L.

AU - Bartlett, D. L.

AU - Vargas, H.

AU - Walther, M.

AU - Lubensky, I.

AU - Glenn, G.

AU - Linehan, W. M.

AU - Alexander, H. R.

AU - Demeure, M. J.

AU - Norton, J. A.

AU - Kinder, B. K.

AU - Clark, O. H.

AU - Talpos, G. B.

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N2 - Background. von Hippel Lindau disease (VHL) is an inherited syndrome characterized by tumors of the kidney, adrenal, central nervous system, and pancreas. The incidence and natural history of pancreatic neuroendocrine tumors occurring in VHL are not known. Methods. From December 1988 through November 1997, 256 patients with VHL, were screened with imaging studies, and these data were reviewed from a prospective database. Results. Thirty (12%) of 256 patients had solid pancreatic lesions consistent with neuroendocrine tumors. Fourteen patients underwent resection, and 4 with metastases on imaging studies underwent biopsy only. Of the 14 patients who underwent resection, 11 remain free of disease, 2 have experienced recurrence, and 1 has died of unrelated causes (mean follow-up, 25 months; range, 3 to 73 months). The size of the primary tumor (median, 5 cm; range, 3 to 8 cm) in patients with liver metastases was significantly larger than the size of the primary tumor (median, 2 cm; range, 1 to 5 cm) in patients without liver metastases (P = .0013). Conclusions. Solid pancreatic lesions were detected in 12% of patients with VHL. Larger primary tumors were associated with liver metastases. Pancreatic imaging to identify neuroendocrine tumors and resection when they reach 2 to 3 cm may prevent the development of hepatic metastases.

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