Surgical precision highlighted by endoscopic transillumination during gastrinoma removal.

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

Surgical precision highlighted by endoscopic transillumination during gastrinoma removal.

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.

Endoscopic ultrasonography and duodenoscopy identified at least three distinct duodenal gastrinomas. Pathological examination graded one as G1, and multiple NETs were found in the pancreatic head. The largest of these, though infracentimetric, was closely positioned to the main pancreatic duct. Preoperative assessments could not determine whether this lesion was functional.

Here's a breakdown of the surgical steps:
  • 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.
Postoperatively, the patient's gastrin levels decreased from 612 to 220 ng/L (normal range < 115 ng/L), while still on proton pump inhibitors. This detailed surgical approach underscores critical steps in managing multifocal and metastatic duodenal gastrinomas. These steps include endoscopic duodenal transillumination, intraoperative pancreatic ultrasound, and duodenotomy, all aimed at precise lesion identification and resection.

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.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.1016/j.jviscsurg.2018.09.005, Alternate LINK

Title: Surgical Procedure For Multifocal Duodenopancreatic Gastrinomas In A Men-1 Patient (With Video)

Subject: General Medicine

Journal: Journal of Visceral Surgery

Publisher: Elsevier BV

Authors: M. Flacs, F. Prunel, L. Groussin, B. Dousset, S. Gaujoux

Published: 2019-02-01

Everything You Need To Know

1

What are gastrinomas, and why are they important in the context of MEN1?

Gastrinomas are neuroendocrine tumors (NETs) primarily found in the duodenum or pancreas. They lead to hypergastrinemia and Zollinger-Ellison Syndrome (ZES). In the context described, they are a key concern, especially in patients with Multiple Endocrine Neoplasia type 1 (MEN1), where they often appear in multiple locations. These tumors can cause significant health issues. The presence of these tumors can necessitate surgical intervention when tumors exceed a certain size, if there's localized metastatic disease, or if the gastrinomas are classified as aggressive. The surgical approach detailed in the text aims to manage these tumors effectively.

2

What is the significance of Multiple Endocrine Neoplasia type 1 (MEN1) in relation to the development of gastrinomas?

Multiple Endocrine Neoplasia type 1 (MEN1) is a genetic disorder where patients develop tumors in multiple endocrine glands. In this context, patients with MEN1 are prone to developing multifocal gastrinomas in the duodenum and pancreas. This is significant because it complicates the management of these tumors, as they are often widespread and require a more complex surgical approach compared to single, localized gastrinomas. The presence of MEN1 affects how these tumors are managed, particularly regarding the need for a thorough surgical approach and the assessment of the risk of metastasis and disease aggressiveness.

3

When is surgical intervention typically considered for gastrinomas, and what does the surgical procedure entail?

Surgical intervention for gastrinomas in patients with MEN1 is considered when tumors are larger than 2 cm, in cases of localized metastatic disease, or when they are classified as aggressive. The surgical procedure involves several steps. These include an initial abdominal exploration, exposure through a Kocher maneuver, intraoperative ultrasound to identify tumors, enucleation and lymphadenectomy to remove tumors and affected lymph nodes, and duodenal assessment using endoscopic transillumination to identify and remove tumors. This approach is meticulously designed to remove gastrinomas effectively and prevent complications. These specific steps, such as endoscopic transillumination and intraoperative ultrasound, improve the precision of lesion detection and resection.

4

What are endoscopic transillumination and intraoperative ultrasound, and why are they important in the surgical management of gastrinomas?

Endoscopic transillumination is a technique used during surgery to help identify and locate gastrinomas within the duodenum. This technique involves shining a light through the duodenal wall, which helps visualize the tumors. Intraoperative ultrasound is used during surgery to provide real-time imaging of the pancreas, aiding in the detection and assessment of pancreatic gastrinomas. Both techniques are important because they enhance the accuracy of tumor localization and resection, especially when dealing with multiple, small tumors, as is common in MEN1 patients. Accurate tumor identification is crucial for ensuring complete tumor removal and improving patient outcomes.

5

What is involved in the postoperative care of a patient who has undergone surgery for gastrinomas?

Postoperative care in managing gastrinomas includes monitoring of gastrin levels and the continued use of medications like proton pump inhibitors. The case study mentioned that the patient’s gastrin levels decreased after surgery, even while using proton pump inhibitors. This demonstrates the effectiveness of surgical intervention in managing the hormone excess caused by the tumors. Follow-up care focuses on monitoring for recurrence, managing symptoms, and assessing the long-term impact on the patient’s health and quality of life. The goal is to ensure that the patient's symptoms are controlled, and the risk of tumor recurrence and metastasis is minimized.

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