Pancreatic neuroendocrine tumors associated with von Hippel Lindau disease: Diagnostic and management recommendations

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• 作者：Steven K. Libutti ; Peter L. Choyke ; David L. Bartlett ; Hernan Vargas ; McClellan Walther ; Irina Lubensky ; Gladys Glenn ; W.Marston Linehan ; H.Richard Alex ; er
• 刊名：Surgery
• 出版年：1998
• 出版时间：December 1998
• 年：1998
• 卷：124
• 期：6
• 页码：1153-1159
• 全文大小：135 K

文摘

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. (Surgery 1998;124:1153-9.)