Zollinger-Ellison syndrome surgery: Difference between revisions

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==Overview==
==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.<ref name="pmid10460814">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J et al.| title=Surgery to cure the Zollinger-Ellison syndrome. | journal=N Engl J Med | year= 1999 | volume= 341 | issue= 9 | pages= 635-44 | pmid=10460814 | doi=10.1056/NEJM199908263410902 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10460814  }} </ref>
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==
==Surgery==


*With the advent of inexpensive, easy to administer, and well-tolerated pharmacologic options, acid-reducing surgical procedures largely fell by the wayside, instead replaced by the highly effective medical therapy. <ref name="pmid16926567">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Gibril F, Liewehr DJ, Venzon DJ et al.| title=Surgery increases survival in patients with gastrinoma. | journal=Ann Surg | year= 2006 | volume= 244 | issue= 3 | pages= 410-9 | pmid=16926567 | doi=10.1097/01.sla.0000234802.44320.a5 | pmc=1856542 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16926567  }} </ref>
*The highly effective medical therapy which is inexpensive, easy to administer, and well-tolerated pharmacologically has replaced acid-reducing surgical procedures. <ref name="pmid16926567">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Gibril F, Liewehr DJ, Venzon DJ et al.| title=Surgery increases survival in patients with gastrinoma. | journal=Ann Surg | year= 2006 | volume= 244 | issue= 3 | pages= 410-9 | pmid=16926567 | doi=10.1097/01.sla.0000234802.44320.a5 | pmc=1856542 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16926567  }} </ref>
*The role of surgical management of ZES has instead shifted to eradication of primary tumor and control/prevention of metastatic spread. <ref name="pmid16926567">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Gibril F, Liewehr DJ, Venzon DJ et al.| title=Surgery increases survival in patients with gastrinoma. | journal=Ann Surg | year= 2006 | volume= 244 | issue= 3 | pages= 410-9 | pmid=16926567 | doi=10.1097/01.sla.0000234802.44320.a5 | pmc=1856542 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16926567  }} </ref>
*The surgical management of ZES has progressed to the eradication of the primary [[tumor]] along with control and prevention of its [[metastatic]] spread. <ref name="pmid16926567">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Gibril F, Liewehr DJ, Venzon DJ et al.| title=Surgery increases survival in patients with gastrinoma. | journal=Ann Surg | year= 2006 | volume= 244 | issue= 3 | pages= 410-9 | pmid=16926567 | doi=10.1097/01.sla.0000234802.44320.a5 | pmc=1856542 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16926567  }} </ref>
*Surgical approach to gastrinoma differs in sporadic ZES and ZES associated with MEN-1. As already mentioned, even although many gastrinomas are well-differentiated, over half do carry malignant potential and mortality results from metastatic disease. <ref name="pmid12946485">{{cite journal| author=Norton JA, Jensen RT| title=Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1). | journal=Surg Oncol | year= 2003 | volume= 12 | issue= 2 | pages= 145-51 | pmid=12946485 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12946485  }} </ref>
*In [[sporadic]] ZES and ZES associated with [[MEN]]-1 surgical approach to [[gastrinoma]]  is quite different. Even though many [[gastrinoma]]<nowiki/>s are well-differentiated, over 50%  carry a [[malignant]] potential and their [[mortality]] results from [[metastatic]] disease. <ref name="pmid12946485">{{cite journal| author=Norton JA, Jensen RT| title=Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1). | journal=Surg Oncol | year= 2003 | volume= 12 | issue= 2 | pages= 145-51 | pmid=12946485 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12946485  }} </ref>
*Even though for the most part gastrinomas are slow-growing tumors, and their metastatic propensity is low if the lesion is less than 2 cm in size, presence of metastatic disease dramatically worsens prognosis and decreases survival. Because of this, early surgical exploration and excision of primary lesions is done in patients with ZES to prevent distant spread. Unfortunately, complete surgical resection is possible in less than half of patients with sporadic ZES and not at all in patients who also have MEN-1. <ref name="pmid16253901">{{cite journal| author=Norton JA| title=Surgical treatment and prognosis of gastrinoma. | journal=Best Pract Res Clin Gastroenterol | year= 2005 | volume= 19 | issue= 5 | pages= 799-805 | pmid=16253901 | doi=10.1016/j.bpg.2005.05.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16253901  }} </ref>
*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. <ref name="pmid16253901">{{cite journal| author=Norton JA| title=Surgical treatment and prognosis of gastrinoma. | journal=Best Pract Res Clin Gastroenterol | year= 2005 | volume= 19 | issue= 5 | pages= 799-805 | pmid=16253901 | doi=10.1016/j.bpg.2005.05.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16253901  }} </ref>
*The vast majority of gastrinomas are present within the “gastrinoma triangle,” which is composed of the porta hepatis, duodenal sweep, and pancreatic head. As surgery remains the treatment of choice, localization of the primary lesion is often challenging but essential. <ref name="pmid27408649">{{cite journal |vauthors=Yang RH, Chu YK |title=Zollinger-Ellison syndrome: Revelation of the gastrinoma triangle |journal=Radiol Case Rep |volume=10 |issue=1 |pages=827 |year=2015 |pmid=27408649 |pmc=4921170 |doi=10.2484/rcr.v10i1.827 |url=}}</ref>
*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. <ref name="pmid27408649">{{cite journal |vauthors=Yang RH, Chu YK |title=Zollinger-Ellison syndrome: Revelation of the gastrinoma triangle |journal=Radiol Case Rep |volume=10 |issue=1 |pages=827 |year=2015 |pmid=27408649 |pmc=4921170 |doi=10.2484/rcr.v10i1.827 |url=}}</ref>
*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.<ref name="pmid10460814">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J et al.| title=Surgery to cure the Zollinger-Ellison syndrome. | journal=N Engl J Med | year= 1999 | volume= 341 | issue= 9 | pages= 635-44 | pmid=10460814 | doi=10.1056/NEJM199908263410902 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10460814  }} </ref>
*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. <ref name="pmid10460814">{{cite journal| author=Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J et al.| title=Surgery to cure the Zollinger-Ellison syndrome. | journal=N Engl J Med | year= 1999 | volume= 341 | issue= 9 | pages= 635-44 | pmid=10460814 | doi=10.1056/NEJM199908263410902 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10460814  }} </ref>


===Gastrinoma Triangle===
===Gastrinoma Triangle===
*Gastrinoma triangle borders are defined by confluence 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. <ref name="pmid24319020">{{cite journal |vauthors=Epelboym I, Mazeh H |title=Zollinger-Ellison syndrome: classical considerations and current controversies |journal=Oncologist |volume=19 |issue=1 |pages=44–50 |year=2014 |pmid=24319020 |pmc=3903066 |doi=10.1634/theoncologist.2013-0369 |url=}}</ref>
*Gastrinoma triangle borders are defined by the merging of the [[Cystic duct|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. <ref name="pmid24319020">{{cite journal |vauthors=Epelboym I, Mazeh H |title=Zollinger-Ellison syndrome: classical considerations and current controversies |journal=Oncologist |volume=19 |issue=1 |pages=44–50 |year=2014 |pmid=24319020 |pmc=3903066 |doi=10.1634/theoncologist.2013-0369 |url=}}</ref>
 
[[Image:ZES_-_GASTRINOMA_TRIANGLE.jpg|center|400px]].


==References==
==References==

Latest revision as of 14:39, 29 September 2017

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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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