Patients with Zollinger-Ellison Syndrome suffer from ulcers of the upper gastrointestinal
tract, higher than normal levels of gastric acid, and tumors of the pancreas known as
non-beta islet cell tumors.
Prior to the use of drugs to cure the ulcers, patients typically died due to severe ulcers.
Because of such effective drugs to treat the ulcers it is more common to see patients dying
due to the pancreatic tumors.
The study will observe patients suffering from Zollinger-Ellison Syndrome and non-beta islet
cell tumors and determine the effectiveness of combined chemotherapy with streptozotocin,
5-fluorouracil, and doxorubicin.
Heretofore morbidity and risk of death in Zollinger-Ellison syndrome were caused by severe
ulcer disease. The advent of specific drugs to cure ulcer disease now extends life until
metastases from the non-beta-islet cell tumor cause death. The present study proposes to
continue to test the effect of combined chemotherapy with streptozotocin, 5-fluorouracil and
doxorubicin in biopsy-proven cases of metastatic non-beta-islet cell tumor in patients with
Zollinger-Ellison syndrome. We published our initial experience with this protocol in 1988.
An objective response was observed in 40% of patients.
Subjects selected for this study will be patients with Zollinger-Ellison syndrome who are
being evaluated under the protocol entitled "Diagnostic Evaluation of Patients with
Suspected Abnormalities of Gastric Secretion" (80-DK-0123).
Histologically proven gastrinoma;
Evidence of metastatic disease or locally invasive tumor by angiography, ultrasound,
computerized axial tomography, MRI scan or bone scan;
Progression of the tumor during the preceding 6 months.
The following pre-existing conditions will exclude patients from the study:
Congestive heart failure;
Proteinuria greater than 1 gram/day;
Serum creatinine greater than 1.5 mg%;
Platelet count less than 100,000/mm3;
White blood count less than 2500/mm3;