MEN1-Associated Gastrinomas: A Surgical Solution
"Navigating the complexities of multifocal duodenopancreatic gastrinomas in MEN1 patients through advanced surgical techniques."
Gastrinomas, neuroendocrine tumors (NETs) typically found in the duodenum or pancreas, are responsible for hypergastrinemia and Zollinger-Ellison Syndrome (ZES). Gastric acid hypersecretion, which can be managed with proton pump inhibitors, often makes surgery unnecessary to address the hormone excess itself.
However, in patients with Multiple Endocrine Neoplasia type 1 (MEN1), gastrinomas frequently appear in multiple locations within the duodenum and pancreas. This presents a complex clinical challenge, especially when balancing the risk of metastasis.
Surgical intervention may be considered for MEN1 patients when tumors exceed 2 cm in size, in cases of localized metastatic disease, or if the gastrinomas are classified as aggressive. This article explores a detailed surgical procedure employed to manage multifocal duodenopancreatic gastrinomas in a patient with MEN1, offering insights into the techniques and considerations involved.
Surgery Versus Long-Term Medication
Pharmacologic acid-suppressive therapy is the standard of care for most patients with gastrinoma and Zollinger-Ellison syndrome associated with MEN1. However, a 2022 review discusses concerns about the serious side effects and tumorigenic effects of prolonged proton pump inhibitor use. That review reports curative surgery in 20 patients with MEN1 over 30 years and concludes that surgery should be considered the first-line treatment, although the surgical approach remains controversial because MEN1 gastrinomas are often multiple, difficult to localize before surgery, and unpredictable in their course.
Case Study: Surgical Management of Multifocal Gastrinomas
This case involves a 62-year-old woman diagnosed with MEN1 and multifocal duodenal gastrinomas, along with a metastatic lymph node. The patient's symptoms were effectively managed with proton pump inhibitors. Diagnostic imaging, including CT scans, MRI with diffusion-weighted sequences, and PET/CT scans using 68Ga-DOTA-TOC, revealed significant uptake in the duodenopancreatic area near the superior genu.
- Initial Exploration: A bi-subcostal laparotomy was performed, beginning with a comprehensive abdominal exploration.
- Exposure: A wide detachment of the colo-epiploic area and a Kocher maneuver provided optimal access to the duodenum and pancreas.
- Intraoperative Ultrasound: This revealed an 8 mm NET in the head of the pancreas, in direct contact with the main pancreatic duct.
- Enucleation and Lymphadenectomy: Partial enucleation of the NET was performed, and immunohistochemistry later confirmed its nonfunctional status. A regional lymphadenectomy of the hepatic pedicle and right gastro-epiploic pedicle was also conducted.
- Duodenal Assessment: Endoscopic transillumination identified a lesion in the second part of the duodenum (D2).
- Gastrinoma Removal: A longitudinal duodenotomy on D2 allowed for the identification and palpation of three gastrinomas, which were then removed.
- Closure: The duodenotomy was closed using two hemi-continuous sutures, reinforced with an epiplooplasty.
Recent Surgical Evidence
A 2019 report describes a surgical procedure for multifocal duodenopancreatic gastrinomas in a patient with MEN1. More recent results presented in 2025 report that gastrinoma is often diagnosed late because duodenal tumors are small and that MEN1 has a low surgical cure rate. The same 2025 report states that nearly a 50% cure rate lasting more than 10 years was achieved after surgery during the last 25 years, with reduced mortality.
Limits of Current Management
Research on duodenal neuroendocrine neoplasms describes rapid progress in endoscopic treatment for early disease and systematic treatment for advanced disease. However, the source also reports that many clinicians still lack a comprehensive understanding of the diagnosis and treatment of these tumors. This gap can limit consistent application of emerging management strategies.
Conclusion: Optimizing Surgical Strategies for MEN1 Gastrinomas
In summary, surgical intervention for multifocal duodenopancreatic gastrinomas in MEN1 patients requires a meticulous and multifaceted approach. The integration of advanced imaging techniques, such as intraoperative ultrasound and endoscopic transillumination, enhances the precision of lesion detection and resection. While surgery is not always necessary due to effective medical management of hypergastrinemia, it remains a critical option for managing tumor size, metastasis risk, and aggressive disease patterns, ultimately improving patient outcomes and quality of life.
Prognostic Guidance for Surgery
Life expectancy is reduced in patients with MEN1 gastrinoma. Fasting serum gastrin levels and pancreatic neuroendocrine tumors measuring at least 2 cm are reported as prognostic factors. Fasting serum gastrin levels may help guide surveillance intensity, prompt additional diagnostic evaluation, and support selection of patients who might benefit from surgery.
Risk-Stratified Surveillance
Future MEN1 gastrinoma management may increasingly use fasting serum gastrin levels to individualize surveillance. The cited study identifies fasting serum gastrin levels and pancreatic neuroendocrine tumors measuring at least 2 cm as prognostic factors. It suggests that these measures could help determine when to intensify surveillance, pursue additional diagnostics, or consider surgery.
Personalized Localization and Treatment
Accurate localization of gastrinomas and their metastases is important for developing precise treatment strategies. A recent report describes three cases of metastatic gastrinomas evaluated with 68Ga-exendin-4 and 68Ga-DOTANOC PET/CT. The report notes that most gastrinomas highly express the relevant target, while a separate MEN1 case report illustrates the continuing clinical controversy surrounding surgery and the rare use of total pancreatectomy.