Surgical treatment of endocrine pancreatic lesions in MEN-1.
The surgical treatment of endocrine pancreatic lesion in the multiple endocrine neoplasia syndrome type 1 (MEN-1) has remained a controversial issue. Histologic studies have revealed that the pancreatic lesions generally consist of numerous microadenomas, spread throughout the pancreas, together with occasional larger tumors. The patients may also harbor multiple, mainly gastrin secreting, duodenal microadenomas. A total pancreatectomy/duodenectomy should theoretically be needed for cure, but has not been recommended due to the associated mortality and morbidity, considering also the favorable prognosis of most MEN-1 patients. When pancreatic involvement in MEN-1 is biochemically diagnosed efforts should be made to localize the lesions by computed tomography, angiography and, in some patients, also transhepatic portal vein catheterization and venous sampling (PTP). Hypergastrinemia and the Zollinger-Ellison syndrome (ZES) generally constitute two-thirds of the clinically detected pancreatic lesions in MEN-1. Surgery may be undertaken in ZES-MEN-1 patients with focal lesions visualized by radiology or PTP in order to minimize the risk of malignant development in a gross tumor. Patients with insulin excess and hypoglucemia as well as the rare vipoma patient may, even in the absence of radiologically visualized tumors, be subjected to exploration, and these patients are usually found to harbor one or several gross tumors. The more frequent clinically silent, mainly PP-producing tumors should be removed when visualized by radiology. However, indications for surgery also have to emphasize an unusually malignant behavior in certain kindreds and patients may thus have to be explored when only biochemical data indicate the presence of pancreatic lesions. Pancreatic operations in MEN-1 should generally include a corpus and tail resection, together with enucleation of lesions in the pancreatic head, and in addition to that a careful duodenal exploration. Intraoperative ultrasound examination appears to be of considerable value by its ability to reveal also smaller lesions which may escape palpation.
Department of Surgery, University Hospital, Uppsala, Sweden.
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Akerström
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Johansson
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Grama
D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000000981:diagnostic imaging / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography