What is the management of Bollinger Ellison syndrome?

Management of Zollinger-Ellison Syndrome

The management of Zollinger-Ellison syndrome (ZES) requires high-dose proton pump inhibitors as first-line therapy to control gastric acid hypersecretion, followed by tumor localization studies and surgical resection of gastrinomas when feasible to improve survival outcomes.

Diagnosis

Zollinger-Ellison syndrome should be suspected in patients with:

• Severe erosive or ulcerative esophagitis
• Multiple peptic ulcers or ulcers in unusual locations (e.g., jejunum)
• Refractory or complicated peptic ulcers
• Peptic ulcers associated with diarrhea
• Family history of multiple endocrine neoplasia type 1 (MEN-1)

Diagnostic workup includes:

1. Fasting serum gastrin level (>100 pg/ml) after discontinuation of antisecretory medications
2. Assessment of gastric acidity through pH or gastric analysis (basal acid output >15 mEq/h)
3. Secretin stimulation test (best test to distinguish ZES from other conditions with elevated gastrin)

Medical Management

Acid Suppression Therapy

• First-line treatment: High-dose proton pump inhibitors (PPIs) 1
  • Controls acid hypersecretion in virtually all patients
  • Has made total gastrectomy unnecessary 2
• H2-receptor antagonists may be used but often require higher doses 3
• For patients requiring parenteral therapy, intermittent bolus injection of pantoprazole is recommended 1

Management of Associated Conditions

• Evaluate for MEN-1 syndrome, which is present in 20-25% of ZES patients 4
• Test for other potential underlying causes of gastrinoma

Tumor Localization

Recommended imaging studies include:

1. Somatostatin receptor scintigraphy (SRS) - initial localization study of choice 1
2. Computed tomography (CT) and magnetic resonance imaging (MRI) 2
3. Endoscopic ultrasound (EUS) - similar sensitivity to SRS for identifying primary tumors 1
4. Selective arterial secretin injection test 2

Note: Despite comprehensive imaging, approximately 50% of gastrinomas may not be evident on preoperative studies 2.

Surgical Management

For Sporadic Gastrinoma (non-MEN-1)

• All patients without unresectable metastatic disease should undergo exploratory laparotomy for potential curative resection 2
• With increased awareness of duodenal tumors, gastrinomas can be found in 80-90% of patients 2
• Surgery may be effective for metastatic gastrinoma if most or all tumor can be resected 2

For MEN-1 Associated Gastrinoma

• Management remains controversial 2
• Some clinicians advocate aggressive surgical approach, while others report limited success in achieving eugastrinemia 2

Surgical Considerations

• If future treatment with octreotide/somatostatin analogs is anticipated, prophylactic cholecystectomy should be considered due to the association with gallstones 5
• Proximal gastric vagotomy may reduce acid secretory response to endogenous hypergastrinemia and augment the effect of H2-receptor antagonists 3

Long-term Management

• Regular follow-up with serum gastrin measurements
• Endoscopic surveillance for recurrent ulcers
• Monitoring for tumor recurrence or metastasis
• Adjustment of antisecretory medication dosages as needed

Prognosis

• Prognosis for patients with completely resected gastrinomas is excellent 5
• Surgical resection with regional lymphadenectomy represents the best opportunity for disease control, even with metastatic disease 5
• With modern management approaches, morbidity and mortality associated with ZES have significantly decreased 6

Common Pitfalls and Caveats

1. Failure to suspect ZES in patients with refractory or unusual peptic ulcer disease
2. Premature discontinuation of acid suppression therapy
3. Inadequate dosing of PPIs leading to persistent symptoms
4. Overlooking the possibility of MEN-1 syndrome
5. Incomplete surgical exploration (especially duodenum) during attempted curative resection
6. Not considering prophylactic cholecystectomy when long-term somatostatin analog therapy is planned

By following this structured approach to diagnosis and management, mortality and morbidity from Zollinger-Ellison syndrome can be significantly reduced while improving quality of life for affected patients.