Gastric carcinoid tumors in multiple endocrine neoplasia-1 patients with Zollinger-Ellison syndrome can be symptomatic, demonstrate aggressive growth, and require surgical treatment.
BACKGROUND
Gastric carcinoid tumors occur in 15% to 50% of patients with multiple endocrine neoplasia-1/Zollinger-Ellison syndrome (MEN-1/ZES) but are thought to be benign. We report 5 patients with MEN-1/ZES with symptomatic, aggressive gastric carcinoid tumors that required surgical procedures.


METHODS
This was a retrospective chart review.


RESULTS
Each patient had MEN-1/ZES. Each patient had innumerable gastric carcinoid tumors with symptoms. The fasting gastrin level was 47,000 pg/mL (normal, <200 pg/mL); the basal acid output was 79 mEq/hr (n = 3), and the age at surgical exploration was 47 +/- 6 years, with a duration of MEN-1 of 21 +/- 3 years and of ZES of 15 +/- 2 years. All patients had elevated 5-HIAA or serotonin levels. Somatostatin receptor scintigraphy showed increased stomach uptake in 4 patients (80%). Four patients had a total gastrectomy; 4 patients had lymph node metastases removed, and 3 patients had liver metastases resected. One patient who did not have a total gastrectomy had liver carcinoid metastases.


CONCLUSIONS
These results demonstrate that gastric carcinoid tumors in patients with longstanding MEN-1/ZES may be symptomatic, aggressive, and metastasize to the liver. With increased long-term medical treatment and life expectancy, these tumors will become an important determinant of survival.
Norton
Jeffrey A
JA
Department of Surgery, Stanford University Medical Center, 300 Pasteur Drive, Stanford, CA 94305-5651, USA.
Melcher
Marc L
ML
Gibril
Fathia
F
Jensen
Robert T
RT
D000328:Adult; D002276:Carcinoid Tumor / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D009361:Neoplasm Invasiveness; D012189:Retrospective Studies; D013274:Stomach Neoplasms / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D015043:Zollinger-Ellison Syndrome / Q000150:complications / Q000503:physiopathology* / Q000601:surgery