Zollinger-Ellison syndrome surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2]; Mohamad Alkateb, MBBCh [3]

Overview

The feasibility of surgery depends on the stage of gastrinoma causing Zollinger-Ellison syndrome at the time of diagnosis. However, all patients diagnosed with Zollinger-Ellison syndrome with no metastasis should be offered surgical exploration and resection.

Surgery

  • The highly effective medical therapy which is inexpensive, easy to administer, and well-tolerated pharmacologically has replaced acid-reducing surgical procedures. [1]
  • The surgical management of ZES has progressed to the eradication of the primary tumor along with control and prevention of its metastatic spread. [1]
  • In sporadic ZES and ZES associated with MEN-1 surgical approach to gastrinoma is quite different. Even though many gastrinomas are well-differentiated, over 50% carry a malignant potential and their mortality results from metastatic disease. [2]
  • If the size of the lesion is less than 2 cm, presence of metastatic disease worsens the prognosis and decreases survival the survival rate even though the gastrinomas are slow-growing tumors, and the metastatic potential is low. Hence, in patients with ZES, it is advisable to perform early surgical exploration and excision of primary lesions in order to prevent distant spread. In less than 50% of patients with sporadic ZES, complete surgical resection is possible and it isnt possible in patients who also have MEN-1. [3]
  • The “gastrinoma triangle,” encompasses the porta hepatis, duodenal sweep, and the pancreatic head wherein the vast majority of gastrinomas are present. Localization of the primary lesion is often difficult but necessary as surgery is the treatment of choice. [4]
  • While diagnosis is being established, depending on the stage of gastrinoma causing Zollinger-Ellison syndrome, the feasibility of surgery is assessed. And, in patients with ZES and no metastasis, surgical exploration and resection is the treatment of choice. [5]

Gastrinoma Triangle

  • Gastrinoma triangle borders are defined by the merging of the cystic and common bile duct superiorly, the second and third portions of the duodenum inferiorly, and the neck and body of the pancreas medially, both dorsally and ventrally. [6]

References

  1. 1.0 1.1 Norton JA, Fraker DL, Alexander HR, Gibril F, Liewehr DJ, Venzon DJ; et al. (2006). "Surgery increases survival in patients with gastrinoma". Ann Surg. 244 (3): 410–9. doi:10.1097/01.sla.0000234802.44320.a5. PMC 1856542. PMID 16926567.
  2. Norton JA, Jensen RT (2003). "Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1)". Surg Oncol. 12 (2): 145–51. PMID 12946485.
  3. Norton JA (2005). "Surgical treatment and prognosis of gastrinoma". Best Pract Res Clin Gastroenterol. 19 (5): 799–805. doi:10.1016/j.bpg.2005.05.003. PMID 16253901.
  4. Yang RH, Chu YK (2015). "Zollinger-Ellison syndrome: Revelation of the gastrinoma triangle". Radiol Case Rep. 10 (1): 827. doi:10.2484/rcr.v10i1.827. PMC 4921170. PMID 27408649.
  5. Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J; et al. (1999). "Surgery to cure the Zollinger-Ellison syndrome". N Engl J Med. 341 (9): 635–44. doi:10.1056/NEJM199908263410902. PMID 10460814.
  6. Epelboym I, Mazeh H (2014). "Zollinger-Ellison syndrome: classical considerations and current controversies". Oncologist. 19 (1): 44–50. doi:10.1634/theoncologist.2013-0369. PMC 3903066. PMID 24319020.

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