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

• Considering their origin, gastrinomas are generally classified under the larger entity such as the neuroendocrine tumors (NETs).
• From the enteroendocrine cells that arise from the embryologic endoderm the gastrinomas are derived which form tumors mainly in the pancreas, and also in the proximal small intestine.
• 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). It has been observed that most gastrinomas arise in the duodenum, with tumors located in the pancreas carrying greater malignant potential.
• The WHO (2010) classified all neuroendocrine tumors, including gastrinomas into three grades based on the mitotic rate, or Ki-67 index:

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

• 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