Zollinger-Ellison syndrome classification: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Zollinger-Ellison syndrome}} | {{Zollinger-Ellison syndrome}} | ||
{{CMG}}; {{AE}} {{ARK}} | {{CMG}}; {{AE}} {{ARK}} {{S.M}} | ||
==Overview== | ==Overview== | ||
[[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. | |||
== | ==Classification== | ||
*Gastrinomas are | *[[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 [[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|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> | |||
{| style="border: 0px; font-size: | *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" | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Grade | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diffrentiation | |||
! 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" + |Behavior | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |WHO Category | |||
|- | |||
! style="background:#DCDCDC;" align="center" + |G1 | |||
| style="background:#F5F5F5;" + | Low grade well-differentiated | |||
| style="background:#F5F5F5;" + |< 2 | |||
| style="background:#F5F5F5;" + |< 3% (10% to 30%) | |||
| style="background:#F5F5F5;" + |Uncertain | |||
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]] | |||
|- | |||
! style="background:#DCDCDC;" align="center" + |G2 | |||
| style="background:#F5F5F5;" + |Intermediate grade, well-differentiated | |||
| style="background:#F5F5F5;" + |2 to 20 | |||
| style="background:#F5F5F5;" + |3% to 20% (50% to 80%) | |||
| style="background:#F5F5F5;" + |Low-grade malignant | |||
| style="background:#F5F5F5;" + |[[Neuroendocrine tumor]] | |||
|- | |||
! style="background:#DCDCDC;" align="center" + |G3 | |||
|High grade, poorly differentiated | |||
| style="background:#F5F5F5;" + |> 20 | |||
| style="background:#F5F5F5;" + |> 20% (1% to 3%) | |||
| style="background:#F5F5F5;" + |High-grade malignant | |||
| style="background:#F5F5F5;" + |[[Neuroendocrine]] [[carcinoma]] | |||
|} | |||
*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;" | |||
| colspan="3" style="background: #4479BA; text-align: center;" | {{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" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|Factors}} | ||
! style="background: #4479BA; padding: 5px 5px;" | ! rowspan="1" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|Sopradic ZES}} | ||
! style="background: #4479BA; padding: 5px 5px;" | ! colspan="1" style="background: #4479BA; padding: 5px 5px;" | {{fontcolor|#FFFFFF|MEN-1 ZES}} | ||
|- | |||
! style="background:#DCDCDC;" align="center" + |[[Prevalence]] | |||
| style="background:#F5F5F5;" + |80% | |||
| style="background:#F5F5F5;" + |20% | |||
|- | |||
! 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=" | |||
| style=" | |||
|- | |- | ||
! 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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Zollinger-Ellison syndrome Microchapters |
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Differentiating Zollinger-Ellison syndrome from other Diseases |
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Zollinger-Ellison syndrome classification On the Web |
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Risk calculators and risk factors for Zollinger-Ellison syndrome classification |
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
- Gastrinomas are generally classified under the larger entity, "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.[1]
- 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 gastrinomas are considered well-differentiated neuroendocrine tumors (NETs).[2]
- The WHO (2010) classified all neuroendocrine tumors, including gastrinomas into three grades based on the mitotic rate, or Ki-67 index:[3][4]
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
- ↑ Norton JA (1994). "Neuroendocrine tumors of the pancreas and duodenum". Curr Probl Surg. 31 (2): 77–156. PMID 7904550.
- ↑ 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.
- ↑ "Gastrinoma - StatPearls - NCBI Bookshelf".
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.