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| Disease found: | Zollinger-Ellison Syndrome (ZES) |
| Current as of: | October 8, 2024 |
| Disease Overview: | Rare disorder affecting 1-3 per million yearly; gastric hypersecretion from gastrinomas, often MEN1 gene linked, causing peptic ulcers. [more info] |
| Signs and Symptoms: | Abdominal pain, Diarrhea, Steatorrhea, Gastric hypersecretion, Hypergastrinemia, Esophagitis, Pyloric stenosis |
| Diagnosis: | Lab studies (FSG & Gastric pH) to determine presence of gastrinoma, Secretin Stimulation Test, Tumor Localization using EGD, Other Imaging (CT/MRI with contrast, gallium PET/CT or Somatostatin Receptor Scintigraphy, Endoscopic Ultrasonography if PET/CT unavailable, tumors typically in duodenum (70%) [reference values] |
| Treatment: | First Line: Proton Pump Inhibitors (eg, omeprazole), H2R Blockers (eg, famotidine), Surgical Resection of Gastrinoma for localized sporadic gastrinoma and gastrinoma with MEN 1 if tumor size > 2 cm |
| Clinical Management: | Systemic chemotherapy (e.g., streptozocin, 5-fluorouracil, doxorubicin), Radiation (e.g., peptide receptor-targeted radiotherapy), Liver-directed therapy (e.g., chemoembolization) in patients with metastases primarily in the liver, Targeted Therapy (e.g., sunitinib, everolimus), Symptomatic Treatment, Regular Monitoring and Supportive Care. |
| Referral: | Gastroenterologist, endocrinologist, surgeon (general or oncological), oncologist, radiologist, pathologist [Specialists near you]. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | Many are completed, but none actively recruiting. |