Zusammenfassung
Da die präoperative bildgebende Lokalisation von Gastrinomen nur selten gelingt, sollte nach biochemischer Sicherung eines Zollinger-Ellison-Syndroms (ZES) und Ausschluss einer diffusen Fernmetastasierung beim sporadischen und MEN1-Gastrinom die Indikation zur Operation gestellt werden, um das Auftreten von Lebermetastasen als entscheidende prognostische Determinante zu verhindern. Der erfahrene Chirurg findet mit Hilfe der subtilen Pankreasexploration, des intraoperativen Ultraschalls (IOUS) und der Duodenotomie mehr als 90% der Primärtumoren.
Beim sporadischen Gastrinom erfolgt die Resektion der pankreatischen Gastrinome durch Enukleation bzw. partielle Pankreasresektion mit Lymphadenektomie. Duodenalwandgastrinome werden exzidiert. Die vollständige Resektion dieser Tumoren ist der einzige kurative Ansatz für die betroffenen Patienten. Bei MEN1-Gastrinomen sollte entweder eine milzerhaltende Pankreaslinksresektion mit Enukleation von Tumoren aus dem Pankreaskopf sowie eine Duodenotomie und die Exzision von Duodenalwandgastrinomen oder eine pyloruserhaltende Pankreaskopfresektion erfolgen, wenn die Gastrinquelle durch präoperative SASI-Angiographie in der Pankreaskopfregion regionalisiert wurde.
Abstract
The preoperative localization of gastrinomas often fails despite all modern imaging methods. Therefore, after biochemical confirmation of the diagnosis and exclusion of diffuse metastases, a meticulous surgical exploration including intraoperative ultrasound (IOUS) and duodenal exploration after duodenotomy should be performed. The experienced surgeon will be able to identify more than 90% of the primary tumors. Depending on the localization, excision of the tumor in the duodenal wall or enucleation from the pancreatic head should be performed. If the tumor is localized in the tail of the pancreas, distal pancreatectomy is the treatment of choice. Complete resection of the tumor is the only curative approach for the patients. For MEN-1 gastrinomas a spleen-preserving distal pancreatectomy with enucleation of tumors of the pancreatic head and duodenotomy with excision of duodenal gastrinomas should be performed. If the source of gastrin secretion can be regionalized to the pancreatic head by a preoperative SASI angiography, a pylorus-preserving partial pancreaticoduodenectomy might be the treatment of choice.
Literatur
Anderson MA, Carpenter S, Thompson NW et al. (2000) Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of the pancreas. Am J Gastroenterol 95:2271
Arnold R, Simon B (2000) Duodenale und pankreatische Gastrinome—Klinik und Diagnostik. In: Siewert JR, Harder F, Rothmund M (Hrsg) Praxis der Viszeralchirurgie—Endokrine Chirurgie. 1. Aufl. Springer, Berlin Heidelberg New York, S. 492
Arnold WS, Fraker DL, Alexander HR et al. (1994) Apparent lymph node primary gastrinoma. Surgery 116:1123
Bartsch DK, Langer P, Wild A et al. (2000) Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: surgery or surveillance? Surgery 128:958
Berger AC, Gibril F, Venzon DJ et al. (2001) Prognostic value of initial fasting serum gastrin levels in patients with Zollinger-Ellison syndrome. J Clin Oncol 19:3051
Chandrasekharappa SC, Guru SC, Manickam P et al. (1997) Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science 276:404
Cadiot G, Vuagnat A, Doukhan I et al. (1999). Prognostic factors in patients with Zollinger-Ellison Syndrome and multiple endocrine neoplasia type 1. Gastroenterology 116:286–93
Creutzfeldt W, Arnold R, Creutzfeldt C, Track NS (1975) Pathomorphologic, biochemical and diagnostic aspects of gastrinomas (Zollinger-Ellison syndrome). Hum Pathol 6:47
Delcore R, Friesen SR (1995) Role of pancreaticoduodenectomy in the management of primary duodenal wall gastrinomas in patients with Zollinger-Ellison syndrome. Surgery 112:1016
Fendrich V, Langer P, Bartsch DK, Zielke A, Rothmund M (2004) Diagnostik und operative Therapie beim Insulinom—Erfahrungen bei 40 Patienten. Dtsch Med Wochenschr 129:941
Fraker DL, Norton JA, Alexander R, Venzon DJ, Jensen RT (1994) Surgery for Zollinger-Ellison syndrome alters the natural history of gastrinoma. Ann Surg 220:320
Gagner M, Pomp A, Herrera MF (1996) Early experience with laparoscopic resections of islet cell tumors. Surgery 120:1051
Gibril F, Reynolds JC, Doppmann JL et al. (1996) Somatostatin receptor scintigraphy: its sensitivity compared with that of other imaging methods in detecting primary and metastatic gastrinomas. A prospective study. Ann Intern Med 125:26
Imamura M, Takahashi K, Adachi H et al. (1987) Usefulness of selective arterial secretin injection test for localization of gastrinoma in the Zollinger-Ellison syndrome. Ann Surg 205:230
Jensen RT, Gardner JD (1993) Gastrinoma. In: Go VLW, Di Magno EP, Gardner JD (eds) The Pancreas: Biology, Pathobiology and Disease, 2nd ed. Raven Press, New York, pp 931
Kisker O, Bartsch D, Weinel RJ et al. (1997) The value of Somatostatin Receptor Scintigraphy in newly diagnosed endocrine gastroenteropancreatic tumors. J Am Coll Surg 184:487
Kisker O, Bastian D, Bartsch D et al. (1998) Localization, malignant potential and surgical management of gastrinomas. World J Surg 22:651
Lairmore TC, Chen VY, DeBenedetti MK et al. (2000) Duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg 231:909
Langer P, Wild A, Nies C, Rothmund M, Bartsch DK (2001) Variable expression of multiple endocrine neoplasia type 1- implications for screening strategies. Int J Surg Invest 3:473
Langer P, Wild A, Hall A, Celik I, Rothmund M, Bartsch DK (2003) Prevalence of multiple endocrine neoplasia type 1 in young patients with apparently sporadic primary hyperparathyroidism or pancreaticoduodenal endocrine tumours. Br J Surg 90:1599
Langer P, Kann PH, Fendrich V et al. (2004) Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. World J Surg 28:1317–1322
Mignon M, Ruszniewski P, Podevin P et al. (1993) Current approach to the management of a gastrinoma and insulinoma in adults with multiple endocrine neoplasia I. World J Surg 17:489
Norton JA, Doppman JL, Gardner Jl et al. (1986) Aggressive resection of metastatic disease in selected patients with malignant gastrinomas. Ann Surg 203:352
Norton JA, Cornelius MJ, Doppman JL, Maton PN, Gardner JD, Jensen RT (1987) Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome, and multiple endocrine neoplasia type I: a prospective study. Surgery 102:958
Norton JA, Cromack DT, Shawker TH et al. (1988) Intraoperative ultrasonographic localization of islet cell tumors. Ann Surg 207:160
Norton JA, Fraker DL, Alexander HR et al. (1999) Surgery to cure the zollinger-ellison syndrome. N Engl J Med 341:635
Norton JA, Alexander HR, Fraker DL et al. (2004) Does the use of routine duodenotomy (DUODX) affect rate of cure, development of liver metastases, or survival in patients with Zollinger-Ellison syndrome? Ann Surg 239:617
Norton JA, Jensen RT (2004) Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome. Ann Surg 240:757
Pipeleers-Marichal M, Somers G, Willems G et al. (1990) Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. N Engl J Med 322:723
Rothmund M (1994) Localization of endocrine pancreatic tumors. Br J Surg 81:164
Rothmund M (2000) Duodenale und pankreatische Gastrinome. Operative Therapie. In: Rothmund M, Siewert JW, Harder F (Hrsg) Praxis der Viszeralchirurgie: Endokrine Chirurgie. 1. Aufl. Springer, Berlin Heidelberg New York, S. 497
Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A et al. (2000) Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine 79:379
Skogseid B, Oberg K, Akerstrom G et al. (1998) Limited tumor involvement found at multiple endocrine neoplasia type I pancreatic exploration: can it be predicted by preoperative tumor localization? World J Surg 22:673
Stabile BE, Morrow DJ, Passaro E (1987) The gastrinoma triangle: Operative implications. Am J Surg 209:550
Stadil F (1995) Treatment of gastrinomas with pancreatoduodenectomy In: Mignon M, Jensen RT (eds) Endocrine tumors of the pancreas: recent advances in research and management. Vol. 23 of Frontiers of gastrointestinal research. Karger, Basel, p 333
Sugg SL, Norton JA, Fraker DL et al. (1993) A prospective study of intraoperative methods to find and resect duodenal gastrinomas. Ann Surg 218:138
Thompson NW, Bondeson AG, Bondeson L, Vinik A (1989) The surgical treatment of gastrinoma in MEN I syndrome patients. Surgery 106:1081
Thompson NW (1998) Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycemia or both. J Intern Med 243:495
Vinik AL, Moattari AR, Cho K, Thompson N (1990) Transhepatic portal vein catheterization for localization of sporadic and MEN gastrinomas: a ten-year experience. Surgery 107:246
Weber HC, Venzon DJ, Lin TJ et al. (1995) Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: A prospective long-term study. Gastroenterology 108:1637
Yu F, Venzon DJ, Serrano J et al. (1999) Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome. J Clin Oncol 17:615
Zimmer T, Scherübl H, Faiss S et al. (2000) Endoscopic ultrasonography of neuroendocrine tumors. Digestion 62 [Suppl1]:45
Zogakis TG, Gibril F, Libutti SK et al. (2003) Management and outcome of patients with sporadic gastrinoma arising in the duodenum. Ann Surg 238:42
Zollinger RM, Ellison EH (1955) Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 142:709
Zollinger RM, Coleman D (1974) The Influences of pancreatic tumors on the stomach. Charles C. Thomas, Springfield Illinois
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Fendrich, V., Bartsch, D.K., Langer, P. et al. Zollinger-Ellison-Syndrom. Chirurg 76, 217–226 (2005). https://doi.org/10.1007/s00104-004-0995-5
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DOI: https://doi.org/10.1007/s00104-004-0995-5