Zollinger-Ellison Syndrome Treatment & Management

Updated: Jan 04, 2019
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: Philip O Katz, MD, FACP, FACG  more...
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Treatment

Medical Care

The goals of treatment in patients with Zollinger-Ellison syndrome (ZES) are medical control of gastric acid hypersecretion and surgical resection of the tumor. Inpatient care is aimed at first controlling the gastric acid hypersecretion. Once gastric acid hypersecretion is controlled, imaging studies should be obtained to localize the tumor and determine tumor extent.

If the patient is acutely ill, immediate control of gastric acid hypersecretion can be achieved with intravenous proton pump inhibitors. Previously, this was accomplished with histamine 2 (H2) receptor blockers. Intravenous pantoprazole was approved recently by the US Food and Drug Administration. Proton pump inhibitors are superior to H2 blockers for the control of gastric acid hypersecretion.

Patients who are candidates for surgical resection should be referred for resection of the tumor.

For patients with metastatic disease, chemotherapy, interferon, and octreotide may be helpful. The response to these agents in most studies has been low. Liver transplantation for hepatic metastasis also has been reported. For patients with a single confined liver metastatic lesion, surgical resection may be attempted.

Consultations

Consider consultation with a gastroenterologist, surgeon, oncologist, and/or an endocrinologist.

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Surgical Care

All patients with sporadic Zollinger-Ellison syndrome (ZES) without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients.

The role and timing of surgical resection in patients with multiple endocrine neoplasia-type 1 (MEN 1) is less clear. An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. Cure is rarely achieved by surgical resection in patients with MEN 1; however, it may reduce the risk of subsequent metastatic disease.

In a single-institution retrospective study with a median follow-up of 18 years from the time of the diagnosis of ZES, Mortellaro et al examined the long-term outcomes in 12 patients with MEN 1 and ZES from 1970 to the present. [4] The pancreas (n = 10), duodenum (n = 4), lymph nodes (n = 3), and liver (n = 1) were the most commonly identified gastrinoma sites. A total of 15 celiotomies were performed, and surgeries included 4 each of distal pancreatectomies and acid-reducing procedures, 3 each of enucleation of pancreatic gastrinoma and duodenal resection, 1 pancreaticoduodenectomy, and 7 noted as other. [4] There was 1 each of a patient with transient (3 y) biochemical postsurgical cure and liver metastasis of gastrinoma (but no deaths from metastatic gastrinoma). [4]

Deaths included causes such as respiratory arrest (n = 1), possibly due to aspiration or pulmonary embolus, and nondisease related (n = 3). At the last follow-up, 7 patients were alive. The investigators observed patients with MEN 1 and ZES rarely achieve biochemical cures with surgery; however, extended surgical resection was not only not needed in resection of localized gastrinomas, but it was also associated with excellent long-term outcomes. [4]

There is a case report of a young adolescent male with primary lymph node gastrinoma and liver metastasis that caused ZES. This patient was successfully managed with preoperative cytoreduction followed by surgical resection of the residual mass, which had a 100% response and 4-year followup. [5]

Because gastrinoma is a rare tumor, surgical resection should be attempted only at centers with personnel experienced in treating affected patients.

Outpatient postoperative care

After surgical resection of a gastrinoma, patients should be assessed for evidence of recurrence with serum fasting gastrin levels, a secretin test, and SRS. The first evaluation should be performed at 3-6 months postresection and then, optimally, yearly thereafter.

Proton pump inhibitors can be continued with the goal of maintaining the basal acid output (BAO) below 10 mEq/h before the next dose of the proton pump inhibitors.

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