Zollinger-Ellison syndrome classification
|
Zollinger-Ellison syndrome Microchapters |
|
Differentiating Zollinger-Ellison syndrome from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Zollinger-Ellison syndrome classification On the Web |
|
American Roentgen Ray Society Images of Zollinger-Ellison syndrome classification |
|
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]
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 gastrinomas are considered well-differentiated neuroendocrine tumors (NETs).
Classiffication
- 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 MEN-1-associated ZES:[5][6][7][8][9][10]
| Sporadic and MEN-1-associated ZES | ||
| Factors | Sopradic ZES | MEN-1 ZES |
|---|---|---|
| Prevalence | 80% | 20% |
| Family history | No | Yes |
| Associated endocrinopathies | No | Yes |
| Gastrinoma size | >2 cm | <2 cm |
| Tumors number | Single | Multiple |
| Most common tumor location | Pancreas | Duodenum |
| Lymph node primary | 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.