Assessment of Cure and Followup

Several reports suggest that at least one half of all Z-E syndrome patients explored, with the expectation to extirpate tumor and cure, continue to have hypergastrinemia postoperatively.84'96 Typically, a small duodenal gastrinoma or lymph node containing tumor has been excised, but an elevated serum gastrin level indicates that more tumor remains. A follow-up plan is needed for such patients. Should such a patient be re-explored or monitored? Should re-exploration be done only when localizing studies indicate the site of tumor? A prospective study by the NIH group to assess and predict long-term cure in Z-E syndrome patients provides insight into these questions.112 Eighty-one consecutive Z-E syndrome patients who had undergone surgical exploration for gastrinoma resection were studied. Fasting gastrin and secretin provocative tests were the first to become positive in patients with recurrence, whereas the calcium provocative test and imaging studies were less sensitive. Fifty-two percent of the patients were disease free immediately after surgery, 44% at 3 to 6 months, 42% at 1 year, and the number of "cured" patients was down to 35% by 5 years. This careful study indicates that in some Z-E syndrome patients who have a normal serum gastrin level immediately postoperatively and who appear to have been cured, hypergastrinemia recurs with time, indicating recurrent tumor.

After removal of a gastrinoma, I recommend a serum gastrin measurement before the patient is discharged from the hospital and then at 3-month intervals for the first year. Hypergastrinemia indicates residual gastrinoma tissue. A normal gastrin level may indicate a surgical cure, but a positive secretin provocative test for gastrin response unmasks some patients who still harbor gastrinomas. A secretin provocative test should be performed 3 months postoperatively and then annually for all normogastrinemic patients who have undergone "curative" gastrinoma resection.

Patients previously operated on for gastrinoma but with progression of hypergastrinemia should also be evaluated periodically to search for a gastrinoma that might be amenable to curative excision because these tumors can grow and cause death. A CT scan and selective abdominal angiography might be done at 1- to 2-year intervals in selected patients. I have successfully excised a large extra-pancreatic gastrinoma from a Z-E syndrome patient who presented with a new abdominal mass and a serum gastrin level of 1.5 million pg/mL 20 years after a TG. At the first operation, a duodenal wall gastrinoma was removed along with a TG, which was done to control bleeding ulcers. The patient was in good health during the 20 years since her TG until the discovery of an abdominal mass. After successful removal of the gastrinoma, the patient's serum gastrin and secretin provocative tests have been normal for 5 years. This case illustrates that some Z-E syndrome patients may be cured by an operation, many years after incomplete tumor excision. Lifelong follow-up is required.

Follow-up of Zollinger-Ellison Syndrome Patients with Total Gastrectomy

Although TG is now done infrequently, there are numerous living patients who have had TG. Some may have had gastrinomas removed at the time of TG, but many still have hypergastrinemia and residual tumor. Although these patients are no longer at risk for complications of excess gastric acid, they still need periodic evaluation to assess for tumor progression as well as nutrition-related problems. Evaluation of patients is recommended at 6-month intervals. Vitamin B12 injections are given monthly, and most patients can administer their Bu injections at home. Folic acid, calcium, and iron supplements are advisable. Many Z-E syndrome patients are noncompliant, particularly when there is a history of alcohol abuse.48 TG also places the patient at risk for cholelithiasis. Nearly all Z-E syndrome patients who have undergone TG acquire gallstones if they live long enough.113 I recommend that cholecystectomy be done at the time of TG.33

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