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]

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.
• 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).

• The WHO (2010) classified all neuroendocrine tumors, including gastrinomas into three grades based on the mitotic rate, or Ki-67 index:

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:

Sporadic and MEN-1-associated ZES
Factors	Sopradic ZES	MEN-1 ZES
Prevalence Family history Other endocrinopathies Gastrinoma Size Number of tumors Most common tumor location Lymph node primary Surgical cure rate Malignant potential	80% No No >2cm Single Pancreas 10% 60% High	20% Yes Yes <2cm Multiple Duodenum No Rare Low