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Review
. 2023 Dec;53(12):1325-1334.
doi: 10.1007/s00595-022-02627-z. Epub 2022 Dec 7.

How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?

Masayuki Imamura  1   2 , Izumi Komoto  3 , Yoshiro Taki  3
Affiliations
Review

How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?

Masayuki Imamura et al. Surg Today. 2023 Dec.

Abstract

In patients with multiple endocrine neoplasia type 1 syndrome (MEN 1) and Zollinger-Ellison syndrome (ZES), gastrinomas arise from the duodenum, about 60% are multiple, and about 15% of patients have coexisting pancreatic gastrinomas, which can be localized by the selective arterial secretagogue injection test (SASI test). The guidelines (GLs) by the Japanese Neuroendocrine Tumor Society (JNETS) recommend surgical resection for functioning duodenopancreatic neuroendocrine tumors (NETs), including gastrinomas, in patients with MEN1 (Grade A, 100% agreement among members). Conversely, the GLs of the National Comprehensive Cancer Network (NCCN) in the USA recommend observation and treatment with proton pump inhibitors (PPIs) or exploratory surgery for occult gastrinomas. An international Consensus Statement (ICS) from the European Union (EU) also does not recommend resection of gastrinomas in patients with MEN1, despite some surgeons having reported surgery being curative for gastrinomas in MEN1 patients. In this review, we discuss the serious side effects and tumorigenic effects of the prolonged use of PPIs and the safety and curability of surgery, supported by our results of curative surgery for gastrinomas in 20 patients with MEN1 over 30 years. We conclude that surgery should be the first-line treatment for gastrinomas in MEN1 patients.

Keywords: Gastrinomas; MEN1; Pancreas preserving total duodenectomy (PPTD); Proton pump inhibitors (PPIs); Selective arterial secretagogue injection test (SASI test).

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Conflict of interest statement

Masayuki Imamura and his co-authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Patient with ZE syndrome in whom gastrinomas could not be found during surgery
Fig. 2
Fig. 2
Overall survival (OS) and progression-free survival (PFS) of 20 patients with MEN1/gastrinomas who underwent surgery for gastrinomas in our center
See this image and copyright information in PMC

References

    1. Imamura M, Komoto I, Ota S, Hiratsuka T, Kosugi S, Doi R, et al. Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients. World J Gastroenterol. 2011;17(10):1343–1353. doi: 10.3748/wjg.v17.i10.1343. - DOI - PMC - PubMed
    1. Pipeleers-Marichal M, Somers G, Willems G, Foulis A, Imrie C, Bishop AE, et al. Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. N Engl J Med. 1990;322:723–727. doi: 10.1056/NEJM199003153221103. - DOI - PubMed
    1. Anlauf M, Garbrecht N, Henopp T, Schmitt A, Schlenger R, Raffel A, et al. Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. World J Gastroenterol. 2006;12:5440–5446. doi: 10.3748/wjg.v12.i34.5440. - DOI - PMC - PubMed
    1. Imamura M, Takahashi K, Isobe Y, Hattori Y, Satomura K, Tobe T. Curative resection of multiple gastrinomas aided by selective arterial secretin injection test and intraoperative secretin test. Ann Surg. 1989;210(6):710–718. doi: 10.1097/00000658-198912000-00004. - DOI - PMC - PubMed
    1. Thompson NW. Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the 40 patients with Zollinger-Ellison syndrome, hypoglycemia or both. J Intern Med. 1998;243:495–500. doi: 10.1046/j.1365-2796.1998.00307.x. - DOI - PubMed

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