Routine Lymph Node Dissection Versus Duodenal Inspection Alone for the Treatment of Multiple Endocrine Neoplasia Type 1 Patients with Hypergastrinemia

  • Paxton V. DicksonEmail author
Part of the Difficult Decisions in Surgery: An Evidence-Based Approach book series (DDSURGERY)


Gastrinomas represent the most common funtional pancreaticoduodenal neuroendocrine tumor in MEN1 patients, however, there is lack of consensus regarding appropriate timing and extent of operation for these patients. An optimal strategy has been difficult to define due to the uncommon nature of the disease, inconsistency of operative approaches, absence of controlled studies, limited follow-up, and incomplete understanding of the natural history of the disease. It has been demonstrated that the majority of MEN1-associated gastrinomas are located in the duodenum and often occur concomitantly with non-gastrin producing pancreatic neoplasms. Therefore, evaluation of the duodenum for removal of these tumors is critical to the operative strategy, regardless of the extent of pancreatectomy planned. This chapter reviews outcomes of studies focused on MEN1 patients with hypergastrinemia with particular attention to the incidence of gastrinoma-associated nodal metastases and the rationale for performance of a regional lymphadenectomy at the time of operative exploration. Although there are relatively few studies examining this issue, available data demonstrate a high occurrence of gastrinoma lymph node metastases in MEN1 patients. Furthermore, these are often micrometastases and commonly used preoperative imaging modalities have a low sensitivity for detecting this disease. Collectively, the available evidence suggest that in addition to removal of the primary tumor(s), a formal anatomically based regional lymphadenectomy may result in more durable reduction of gastrin hypersecretion and offer potential long-term oncologic benefit (GRADE B).


Multiple endocrine neoplasia type 1 Gastrinoma Hypergastrinemia Lymph node metastases 


  1. 1.
    Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955;142(4):709–28.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Yeung MJ, Pasieka JL. Gastrinomas: a historical perspective. J Surg Oncol. 2009;100(5):425–33.CrossRefPubMedGoogle Scholar
  3. 3.
    Brandi ML, Gagel RF, Anteli A, Bilezikian JP, Beck-Peccoz P, Bordi C, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86(12):5658–71.CrossRefPubMedGoogle Scholar
  4. 4.
    Carty SE, Helm AK, Amico JA, Clarke MR, Foley TP, Watson CG, Mulvihill JJ. The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1. Surgery. 1998;124(6):1106–14.CrossRefPubMedGoogle Scholar
  5. 5.
    Norton JA, Jensen RT. Resolved and unresolved controversies in the surgical management of pateints with Zollinger-Ellison syndrome. Ann Surg. 2004;240(5):757–73.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Ellison EC, Sparks J, Verducci JS, Johnson JA, Muscarella P, Bloomston M, Melvin WS. 50-year appraisal of gastrinoma: recommendations for staging and treatment. J Am Coll Surg. 2006;202(6):897–905.CrossRefPubMedGoogle Scholar
  7. 7.
    Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT. Does the use of routine duodentotomy (DUODZ) affect rate of cure, development of liver metastases, or survival in patients with Zollinger-Ellison syndrome? Ann Surg. 2004;239(5):617–26.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Thompson NW. 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, hypoglycaemia or both. J Intern Med. 1998;243(6):495–500.CrossRefPubMedGoogle Scholar
  9. 9.
    Tonelli F, Fratini G, Nesi G, Tommasi MS, Batignani G, Falchetti A, Brandi ML. Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias. Ann Surg. 2006;244(1):61–70.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Bartsch DK, Fendrich V, Langer P, Celik I, Kann PH, Rothmund M. Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg. 2005;242(6):757–66.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Norton JA, Franker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999;341(9):635–44.CrossRefPubMedGoogle Scholar
  12. 12.
    Imamura M, Komoto I, Ota S, Hiratsuka T, Kosugi S, Doi R, et al. Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients. World J Gastroenterol. 2011;17(10):1343–53.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Dickson PV, Rich TA, Xing Y, Cote GJ, Wang H, Perrier ND, et al. Achieving eugastrinemia in MEN1 patients: both duodenal inspection and formal lymph node dissection are important. Surgery. 2011;150(6):1143–52.CrossRefPubMedGoogle Scholar
  14. 14.
    Imamura M, Komoto I, Doi R, Onodera H, Kobayashi H, Kawai Y. New pancreas-preserving total duodenectomy technique. World J Surg. 2005;29(2):203–7.CrossRefPubMedGoogle Scholar
  15. 15.
    Anlauf M, Perren A, Meyer CL, Schmid S, Saramaslani P, Kruse ML, et al. Precursor lesions in patients with multiple endocrine neoplasia type 1-associated duodenal gastrinomas. Gastroenterology. 2005;128(5):1187–98.CrossRefPubMedGoogle Scholar
  16. 16.
    Stabile BE, Morrow DJ, Passaro E Jr. The gastrinoma triangle: operative implications. Am J Surg. 1984;147(1):25–31.CrossRefPubMedGoogle Scholar
  17. 17.
    Krampitz GW, Norton JA, Poultsides GA, Visser BC, Sun L, Jensen RT. Lymph nodes and survival in pancreatic neuroendocrine tumors. Arch Surg. 2012;147(9):820–7.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Surgical OncologyUniversity of Tennessee Health Science CenterMemphisUSA

Personalised recommendations