Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record - Zollinger-Ellison syndrome: pathophysiology, diagnosis and therapy
Edward Sito, Jan W Konturek, Sławomir Kwiecień, Krzysztof Lorens, Władysław Bielański, Piotr C Konturek, Stanisław J Konturek, Andrzej Bobrzyński, Teresa Pawlik, Danuta Karcz, Hany Areny, Tomasz Stachura
Med Sci Monit 2002; 8(6): CS43-59
BACKGROUND: Zollinger-Ellison syndrome is a very rare disease caused bytumor with gastrin producing cells accompanied by hypergastrinemia leading to gastric hypersecretionand peptic ulcers and their complications.CASE STUDY: Female case of gastrinoma (Zollinger-Ellison syndrome;Z-E) with a record of 38 yrs of survival. Acute gastro-duodenal ulcers started at 28 yr of age and Z-Ewas diagnosed by using gastrin assays. Basal and maximal acid outputs and ratio of basal/maximal outputswere away over normal limits. Because of ulcer recurrence and complications, patient was subjected toseveral gastric surgeries but refused total gastrectomy. She was also treated with many H2-receptor (R)antagonists and proton-pump inhibitors (PPI), each new drug being initially highly effective but thenshowing declining efficacy except when PPI, lansoprazole was used. The gastrin level rose in the courseof disease from initial high value of 2000 pg/mL to the extreme 4500 ng/mL at present. During the last2 yrs, metastasis mainly to liver developed and they were successfully treated by synthetic octapeptidederivative of somatostatin and, as a result, metastatis partly reduced and plasma gastrin drasticly decreased.Biopsy taken from liver metastasis showed the presence of typical gastrinoma cells with gastrin and chromogranin,while that from oxyntic mucosa revealed the ECL-cell hyperplasia with carcinoid tumors and unexpectedgastric atrophy.CONCLUSIONS: This phenomenal case described in this article might be the new proven evidenceneeded by gastroenterologists to overturn the traditional treatment using total gastrectomy as a treatmentof choice to the partial gastrectomy combined with proton pump inhibitors.
Keywords: Adult, Anti-Ulcer Agents, Carcinoid Tumor, Gastrinoma, Histamine H2 Antagonists, Stomach Neoplasms, Survivors, Zollinger-Ellison Syndrome