Zollinger-Ellison syndrome classification: Difference between revisions

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__NOTOC__
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{{Zollinger-Ellison syndrome}}
{{Zollinger-Ellison syndrome}}
{{CMG}}; {{AE}} {{ARK}}
{{CMG}}; {{AE}} {{ARK}} {{S.M}}


==Overview==
==Overview==
*According to the World Health Organization, [[neuroendocrine tumors]] (NETs) are classified into two broad categories namely, well differentiated and poorly differentiated. On the basis of [[histopathological]] analysis, most [[Gastrinoma|gastrinomas]] are considered well-differentiated [[neuroendocrine tumors]] (NETs).
[[World Health Organization]] has [[Classification|classified]] [[neuroendocrine tumors|neuroendocrine tumors (NETs)]] into two broad categories, namely well-differentiated and poorly-differentiated. On the basis of [[histopathological]] [[analysis]], most [[Gastrinoma|gastrinomas]] are considered well-differentiated [[neuroendocrine tumors|neuroendocrine tumors (NETs)]]. There is also another [[classification]] for differentiating sporadic Zollinger-Ellison from [[MEN 1 syndrome|MEN 1]] Zollinger-Ellison syndrome which is based on [[familial history]], associated [[Endocrinopathy|endocrinopathies]], [[gastrinoma]] size, number of [[tumors]], [[tumor]] location, and [[lymph node]] involvement.


==Classiffication==
==Classification==
*Gastrinomas are generally classified under the larger entity, "[[neuroendocrine tumors]]" (NETs).
*[[Gastrinoma|Gastrinomas]] are generally classified under the larger entity, "[[neuroendocrine tumors|neuroendocrine tumors" (NETs)]].
*Among the [[enteroendocrine cells]] that arise from the [[embryologic]] [[endoderm]], the gastrinomas are derived mainly from the [[pancreas]], and also from the proximal [[small intestine]]. <ref name="pmid7904550">{{cite journal |vauthors=Norton JA |title=Neuroendocrine tumors of the pancreas and duodenum |journal=Curr Probl Surg |volume=31 |issue=2 |pages=77–156 |year=1994 |pmid=7904550 |doi= |url=}}</ref>
*Among the [[enteroendocrine cells]] that arise from the [[embryologic]] [[endoderm]], the [[Gastrinoma|gastrinomas]] are derived mainly from the [[pancreas]], and also from the [[Anatomical terms of location|proximal]] [[small intestine]].<ref name="pmid7904550">{{cite journal |vauthors=Norton JA |title=Neuroendocrine tumors of the pancreas and duodenum |journal=Curr Probl Surg |volume=31 |issue=2 |pages=77–156 |year=1994 |pmid=7904550 |doi= |url=}}</ref>
*According to the [[World Health Organization]], [[neuroendocrine tumors]] (NETs) are classified into two broad categories; well differentiated, and poorly differentiated gastrinomas. On the basis of [[histopathological]] analysis, most of the [[Gastrinoma|gastrinomas]] are considered well-differentiated [[neuroendocrine tumors]] (NETs). <ref name="pmid23582915">{{cite journal| author=O'Toole D, Delle Fave G, Jensen RT| title=Gastric and duodenal neuroendocrine tumours. | journal=Best Pract Res Clin Gastroenterol | year= 2012 | volume= 26 | issue= 6 | pages= 719-35 | pmid=23582915 | doi=10.1016/j.bpg.2013.01.002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23582915  }} </ref>
*According to the [[World Health Organization]], [[neuroendocrine tumors|neuroendocrine tumors (NETs)]] are [[Classification|classified]] into two broad categories: well differentiated, and poorly differentiated [[Gastrinoma|gastrinomas]].  
*On the basis of [[histopathological]] [[analysis]], most of the [[Gastrinoma|gastrinomas]] are considered well-differentiated [[neuroendocrine tumors|neuroendocrine tumors (NETs)]].<ref name="pmid23582915">{{cite journal| author=O'Toole D, Delle Fave G, Jensen RT| title=Gastric and duodenal neuroendocrine tumours. | journal=Best Pract Res Clin Gastroenterol | year= 2012 | volume= 26 | issue= 6 | pages= 719-35 | pmid=23582915 | doi=10.1016/j.bpg.2013.01.002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23582915  }} </ref>


*The WHO (2010) classified all neuroendocrine tumors, including [[Gastrinoma|gastrinomas]] into three grades based on the mitotic rate, or Ki-67 index: <ref name="urlGastrinoma - StatPearls - NCBI Bookshelf">{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK441842/ |title=Gastrinoma - StatPearls - NCBI Bookshelf |format= |work= |accessdate=}}</ref><ref name="pmid27259015">{{cite journal| author=Tang LH, Basturk O, Sue JJ, Klimstra DS| title=A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas. | journal=Am J Surg Pathol | year= 2016 | volume= 40 | issue= 9 | pages= 1192-202 | pmid=27259015 | doi=10.1097/PAS.0000000000000662 | pmc=4988129 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27259015  }} </ref>
*The [[WHO]] (2010) [[Classification|classified]] all [[neuroendocrine tumors]], including [[Gastrinoma|gastrinomas]] into three [[Grading (tumors)|grades]] based on the [[Mitosis|mitotic rate]], or Ki-67 index:<ref name="urlGastrinoma - StatPearls - NCBI Bookshelf">{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK441842/ |title=Gastrinoma - StatPearls - NCBI Bookshelf |format= |work= |accessdate=}}</ref><ref name="pmid27259015">{{cite journal| author=Tang LH, Basturk O, Sue JJ, Klimstra DS| title=A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas. | journal=Am J Surg Pathol | year= 2016 | volume= 40 | issue= 9 | pages= 1192-202 | pmid=27259015 | doi=10.1097/PAS.0000000000000662 | pmc=4988129 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27259015  }} </ref>
:{| class="wikitable"
:{| class="wikitable"
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Grade
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Grade
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diffrentiation
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diffrentiation
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Mitotic range
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Mitotic Range
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Ki-67 index
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Ki-67 index
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Behavior
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Behavior
! style="background:#4479BA; color: #FFFFFF;" align="center" + |WHO category
! style="background:#4479BA; color: #FFFFFF;" align="center" + |WHO Category
|-
|-
| style="background:#DCDCDC;" align="center" + |G1
! style="background:#DCDCDC;" align="center" + |G1
| style="background:#F5F5F5;" + | Low grade well-differentiated
| style="background:#F5F5F5;" + | Low grade well-differentiated
| style="background:#F5F5F5;" + |< 2
| style="background:#F5F5F5;" + |< 2
Line 27: Line 28:
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]]
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]]
|-
|-
| style="background:#DCDCDC;" align="center" + |G2
! style="background:#DCDCDC;" align="center" + |G2
| style="background:#F5F5F5;" + |Intermediate grade, well-differentiated
| style="background:#F5F5F5;" + |Intermediate grade, well-differentiated
| style="background:#F5F5F5;" + |2 to 20
| style="background:#F5F5F5;" + |2 to 20
Line 34: Line 35:
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]]
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]]
|-
|-
| style="background:#DCDCDC;" align="center" + |G3
! style="background:#DCDCDC;" align="center" + |G3
|High grade, poorly differentiated
|High grade, poorly differentiated
| style="background:#F5F5F5;" + |> 20
| style="background:#F5F5F5;" + |> 20
| style="background:#F5F5F5;" + |> 20% (1% to 3%)
| style="background:#F5F5F5;" + |> 20% (1% to 3%)
| style="background:#F5F5F5;" + |High-grade malignant
| style="background:#F5F5F5;" + |High-grade malignant
| style="background:#F5F5F5;" + |[[Neuroendocrine]] carcinoma
| style="background:#F5F5F5;" + |[[Neuroendocrine]] [[carcinoma]]
|}
|}
*The following table illustrates the factors associated and the differences between sporadic and [[MEN-1]]-associated ZES: <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>
*The following table illustrates the factors associated and the differences between sporadic and [[MEN1]] associated Zollinger-Ellison syndrome (ZES):<ref name="pmid19059523">{{cite journal| author=Ellison EC, Johnson JA| title=The Zollinger-Ellison syndrome: a comprehensive review of historical, scientific, and clinical considerations. | journal=Curr Probl Surg | year= 2009 | volume= 46 | issue= 1 | pages= 13-106 | pmid=19059523 | doi=10.1067/j.cpsurg.2008.09.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19059523  }} </ref><ref name="pmid17312377">{{cite journal| author=Jensen RT, Niederle B, Mitry E, Ramage JK, Steinmuller T, Lewington V et al.| title=Gastrinoma (duodenal and pancreatic). | journal=Neuroendocrinology | year= 2006 | volume= 84 | issue= 3 | pages= 173-82 | pmid=17312377 | doi=10.1159/000098009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17312377  }} </ref><ref name="pmid15802099">{{cite journal| author=Gibril F, Jensen RT| title=Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome. | journal=Curr Gastroenterol Rep | year= 2005 | volume= 7 | issue= 2 | pages= 114-21 | pmid=15802099 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15802099  }} </ref><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><ref name="pmid11573043">{{cite journal| author=Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT| title=Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome. | journal=Ann Surg | year= 2001 | volume= 234 | issue= 4 | pages= 495-505; discussion 505-6 | pmid=11573043 | doi= | pmc=1422073 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11573043  }} </ref><ref name="pmid24319020">{{cite journal| author=Epelboym I, Mazeh H| title=Zollinger-Ellison syndrome: classical considerations and current controversies. | journal=Oncologist | year= 2014 | volume= 19 | issue= 1 | pages= 44-50 | pmid=24319020 | doi=10.1634/theoncologist.2013-0369 | pmc=3903066 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24319020  }}</ref>


{| align="center" style="border: 0px; font-size: 110%; margin: 3px;"
{| align="center" style="border: 0px; font-size: 110%; margin: 3px;"
| style="background: #4479BA; text-align: center;" colspan="3" | {{fontcolor|#FFF|'''Sporadic and MEN-1-associated ZES'''}}
| colspan="3" style="background: #4479BA; text-align: center;" | {{fontcolor|#FFF|'''Sporadic and MEN-1-associated ZES'''}}
|+  
|+  
! style="background: #4479BA; padding: 5px 5px;" rowspan="1" | {{fontcolor|#FFFFFF|Factors}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|Factors}}
! style="background: #4479BA; padding: 5px 5px;" rowspan="1" | {{fontcolor|#FFFFFF|Sopradic ZES}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|Sopradic ZES}}
! style="background: #4479BA; padding: 5px 5px;" colspan="1" | {{fontcolor|#FFFFFF|MEN-1 ZES}}
! colspan="1" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|MEN-1 ZES}}
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="2" |
! style="background:#DCDCDC;" align="center" + |[[Prevalence]]
*Prevalence
| style="background:#F5F5F5;" + |80%
*Family history
| style="background:#F5F5F5;" + |20%
*Other endocrinopathies
*[[Gastrinoma]] Size
*Number of tumors
*Most common tumor location
*[[Lymph node]] primary
*Surgical cure rate
*Malignant potential
| style="padding: 5px 5px; background: #F5F5F5;" |
*80%
*No
*No
*>2cm
*Single
*[[Pancreas]]
*10%
*60%
*High
| style="padding: 5px 5px; background: #F5F5F5;" |
*20%
*Yes
*Yes
*<2cm
*Multiple
*[[Duodenum]]
*No
*Rare
*Low
|-
|-
! style="background:#DCDCDC;" align="center" + |[[Familial history]]
| style="background:#F5F5F5;" + |No
| style="background:#F5F5F5;" + |Yes
|-
! style="background:#DCDCDC;" align="center" + |Associated endocrinopathies
| style="background:#F5F5F5;" + |No
| style="background:#F5F5F5;" + |Yes
|-
! style="background:#DCDCDC;" align="center" + |[[Gastrinoma]] size
| style="background:#F5F5F5;" + |> 2 cm
| style="background:#F5F5F5;" + |< 2 cm
|-
! style="background:#DCDCDC;" align="center" + |[[Tumors]] number
| style="background:#F5F5F5;" + |Single
| style="background:#F5F5F5;" + |Multiple
|-
! style="background:#DCDCDC;" align="center" + |[[Tumor]] location
| style="background:#F5F5F5;" + |[[Pancreas]]
| style="background:#F5F5F5;" + |[[Duodenum]]
|-
| style="background:#DCDCDC;" align="center" + |[[Lymph node]] involvement
| style="background:#F5F5F5;" + |10%
| style="background:#F5F5F5;" + |No
|-
! style="background:#DCDCDC;" align="center" + |[[Surgical]] [[cure]] [[rate]]
| style="background:#F5F5F5;" + |60%
| style="background:#F5F5F5;" + |Rare
|-
! style="background:#DCDCDC;" align="center" + |[[Malignancy]] [[rate]]
| style="background:#F5F5F5;" + |High
| style="background:#F5F5F5;" + |Low
|}
|}



Latest revision as of 21:41, 11 September 2019

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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] Shadan Mehraban, M.D.[3]

Overview

World Health Organization has classified neuroendocrine tumors (NETs) into two broad categories, namely well-differentiated and poorly-differentiated. On the basis of histopathological analysis, most gastrinomas are considered well-differentiated neuroendocrine tumors (NETs). There is also another classification for differentiating sporadic Zollinger-Ellison from MEN 1 Zollinger-Ellison syndrome which is based on familial history, associated endocrinopathies, gastrinoma size, number of tumors, tumor location, and lymph node involvement.

Classification

Grade Diffrentiation Mitotic Range Ki-67 index Behavior WHO Category
G1 Low grade well-differentiated < 2 < 3% (10% to 30%) Uncertain Neuroendocrine tumor
G2 Intermediate grade, well-differentiated 2 to 20 3% to 20% (50% to 80%) Low-grade malignant Neuroendocrine tumor
G3 High grade, poorly differentiated > 20 > 20% (1% to 3%) High-grade malignant Neuroendocrine carcinoma
  • The following table illustrates the factors associated and the differences between sporadic and MEN1 associated Zollinger-Ellison syndrome (ZES):[5][6][7][8][9][10]
Sporadic and MEN-1-associated ZES
Factors Sopradic ZES MEN-1 ZES
Prevalence 80% 20%
Familial history No Yes
Associated endocrinopathies No Yes
Gastrinoma size > 2 cm < 2 cm
Tumors number Single Multiple
Tumor location Pancreas Duodenum
Lymph node involvement 10% No
Surgical cure rate 60% Rare
Malignancy rate High Low

References

  1. Norton JA (1994). "Neuroendocrine tumors of the pancreas and duodenum". Curr Probl Surg. 31 (2): 77–156. PMID 7904550.
  2. O'Toole D, Delle Fave G, Jensen RT (2012). "Gastric and duodenal neuroendocrine tumours". Best Pract Res Clin Gastroenterol. 26 (6): 719–35. doi:10.1016/j.bpg.2013.01.002. PMID 23582915.
  3. "Gastrinoma - StatPearls - NCBI Bookshelf".
  4. Tang LH, Basturk O, Sue JJ, Klimstra DS (2016). "A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas". Am J Surg Pathol. 40 (9): 1192–202. doi:10.1097/PAS.0000000000000662. PMC 4988129. PMID 27259015.
  5. Ellison EC, Johnson JA (2009). "The Zollinger-Ellison syndrome: a comprehensive review of historical, scientific, and clinical considerations". Curr Probl Surg. 46 (1): 13–106. doi:10.1067/j.cpsurg.2008.09.001. PMID 19059523.
  6. Jensen RT, Niederle B, Mitry E, Ramage JK, Steinmuller T, Lewington V; et al. (2006). "Gastrinoma (duodenal and pancreatic)". Neuroendocrinology. 84 (3): 173–82. doi:10.1159/000098009. PMID 17312377.
  7. Gibril F, Jensen RT (2005). "Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome". Curr Gastroenterol Rep. 7 (2): 114–21. PMID 15802099.
  8. 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.
  9. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT (2001). "Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome". Ann Surg. 234 (4): 495–505, discussion 505-6. PMC 1422073. PMID 11573043.
  10. 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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