Zollinger-Ellison syndrome classification
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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
- 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.