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.
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.
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.