Clinical Presentation

Certain clinical scenarios should arouse the suspicion of gastrinoma. They include multiple peptic ulcers, persistence or recurrence of ulcer disease despite adequate medical or surgical treatment, ulcers in unusual locations (such as beyond the first portion of the duodenum), and younger age at presentation. Patients with persistent diarrhea and ulcer disease, spontaneous jejunal perforation, or severe esophagitis, also need to be investigated to rule out gastrinoma. Patients with multiple endocrine neoplasia can pre sent with ulcer disease in conjunction with hypercalcemia or nephrolithiasis. One-third of patients with MEN I-related gastrinoma may have no evidence of other endocrinopathy. Thus, even patients with "sporadic" gastrinoma should undergo periodic screening to rule out MEN I, and be considered for genetic testing.

Duodenal gastrinomas usually tend to be smaller than pancreatic gastrinomas; 80% of duodenal gastrinomas are < 1 cm in size compared to pancreatic gastrinomas, which are usually > 3 cm in size. Gastrinomas frequently metastasize to regional lymph nodes and the liver. Lymph node metastases are seen in > 50 % of patients in both groups, whereas liver metastases are more frequently seen in pancreatic (32 to 54%) than in duodenal gastrinomas (5 to 14%). Pancreatic gastrinomas tend to be more aggressive and are associated with a worse prognosis than duodenal gastrinomas (10 year survival of 50% versus 94%). Metastasis to the liver seems to be related to the size of the primary lesion. Lesions located to the left side of the superior mesenteric artery in the pancreas tend to be larger in size.

It appears that there may be two forms of gastrinoma, aggressive and nonaggressive types. The aggressive type is usually present in females, comprises 25% of the cases, is associated with a short disease duration, larger tumors, pancreatic location, a higher incidence of liver metastasis, and a poorer prognosis.

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