Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record -- Zollinger-Ellison syndrome: pathophysiology, diagnosis and therapy.
BACKGROUND
Zollinger-Ellison syndrome is a very rare disease caused by tumor with gastrin producing cells accompanied by hypergastrinemia leading to gastric hypersecretion and peptic ulcers and their complications.


METHODS
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-E was diagnosed by using gastrin assays. Basal and maximal acid outputs and ratio of basal/maximal outputs were away over normal limits. Because of ulcer recurrence and complications, patient was subjected to several 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 then showing declining efficacy except when PPI, lansoprazole was used. The gastrin level rose in the course of disease from initial high value of 2000 pg/mL to the extreme 4500 ng/mL at present. During the last 2 yrs, metastasis mainly to liver developed and they were successfully treated by synthetic octapeptide derivative 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 unexpected gastric atrophy.


CONCLUSIONS
This phenomenal case described in this article might be the new proven evidence needed by gastroenterologists to overturn the traditional treatment using total gastrectomy as a treatment of choice to the partial gastrectomy combined with proton pump inhibitors.
Konturek
Stanisław J
SJ
Department of Clinical Physiology, College of Medicine, Jagiellonian University School of Medicine, Krakow, Poland. mpkontur@cyf-kr.edu.pl
Konturek
Piotr C
PC
Bielański
Władysław
W
Lorens
Krzysztof
K
Sito
Edward
E
Konturek
Jan W
JW
Kwiecień
Sławomir
S
Bobrzyński
Andrzej
A
Pawlik
Teresa
T
Karcz
Danuta
D
Areny
Hany
H
Stachura
Tomasz
T
D000328:Adult; D000897:Anti-Ulcer Agents / Q000627:therapeutic use; D002276:Carcinoid Tumor / Q000150:complications*; D005260:Female; D015408:Gastrinoma / Q000150:complications*; D006635:Histamine H2 Antagonists / Q000494:pharmacology; D006801:Humans; D013274:Stomach Neoplasms / Q000150:complications*; D017741:Survivors; D015043:Zollinger-Ellison Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery
D000897:Anti-Ulcer Agents; D006635:Histamine H2 Antagonists