Symptoms And Signs

The mean age at diagnosis of ZES is 50 years, and the male-to-female ratio is approximately 2:1. In patients with MEN type I, ZES is usually diagnosed in the third decade of life.4 Clinical manifestations are related to the excessive secretion of gastric acid. The most common symptoms are epigastric pain, diarrhea, heartburn, and dysphagia. Almost all patients with ZES will be found to have peptic ulcers, with the proximal duodenum as the most commonly involved site. A minority of patients will have multiple ulcers or ulcers in unusual locations such as the distal duodenum or jejunum.15 In 7 to 10% of patients, a perforated peptic ulcer may be the initial sign of the disease. Gastric acid hypersecretion also leads to secretory diarrhea, which occurs in up to 40% of patients with ZES and may be the sole presenting complaint in 20% of individuals.16 In those with diarrhea, malabsorption the hepatic vein. A substantial increase in hepatic vein gastrin levels localizes the gastrinoma to the area supplied by the injected artery.

Intraoperative studies have been used as a way to localize tumors, particularly those not imaged pre-operatively, or to confirm preoperative findings. In this regard, intraoperative ultrasonography (IOUS) and intraoperative endoscopy (IOE) with and without transillumination have proven utility.13 IOUS images gastrinomas within the pancreas as sonolu-cent masses (Figure 14-12) and facilitates removal of these tumors by showing the relationship of the tumor to the pancreatic duct and other structures. IOUS has been ineffective at imaging duodenal gastrinomas such that IOE with duodenal transillumination was developed. Using endoscopy, the operator

Figure 14-10. A unique situation in which selective angiography images both the primary gastrinoma (white arrows) and a metastatic lesion within the liver (dark arrows).

A Portal Venous Sampling for Gastrin

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