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

• The World Health Organization classifies neuroendocrine tumors (NETs) into two broad categories, well differentiated and poorly differentiated, and most gastrinomas are considered well-differentiated neuroendocrine tumors (NETs) on the basis of histopathological analysis.

Classiffication

• Gastrinomas are derived from the enteroendocrine cells that arise from the embryologic endoderm, and form tumors mainly in the pancreas, but also in the proximal small intestine. Because of their origin, these are generally classified under the larger umbrella term of neuroendocrine tumors (NETs).
• On the basis of histopathological analysis, the World Health Organization classifies neuroendocrine tumors (NETs) into two broad categories, well differentiated and poorly differentiated, and most gastrinomas are considered well-differentiated neuroendocrine tumors (NETs). It has been observed that most gastrinomas arise in the duodenum, with tumors located in the pancreas carrying greater malignant potential.
• Based on the mitotic rate, or Ki-67 index, the WHO (2010) classified all neuroendocrine tumors, including gastrinomas into three grades:

• (1) Low grade, well-differentiated endocrine tumors with benign or uncertain behavior at the time of diagnosis with a mitotic rate of < 2 and Ki-67 index of < 3% (10% to 30%)
• (2) well-differentiated endocrine carcinomas with low-grade malignant behavior with a mitotic rate of 2 to 20 and Ki-67 index of 3% to 20% (50% to 80%)
• (3) High grade, poorly differentiated endocrine carcinomas with high-grade malignant behavior with a mitotic rate of > 20 and Ki-67 index of > 20% (1% to 3%).

• The following table illustrates 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