This protocol addresses patients with Multiple endocrine neoplasia type 1 (MEN1) who have a co-existing gastrinoma and hypergastrinaemia, and in whom proton pump inhibitor therapy has not achieved adequate control of acid-related symptoms and their sequelae.
Gastrinomas in patients with MEN1 are invariably located in the duodenum and are multiple, with a median age of onset in the third to fifth decade of life. The presence of hypergastrinaemia alongside the gastrinoma defines the specific risk profile and management approach for this sub-population.
Adequate doses of proton pump inhibitors were used to target control of acid-related symptoms and sequelae of hypergastrinaemia, including peptic ulceration and gastrointestinal bleeding. Failure to achieve these goals is the trigger for escalation to the next treatment step.
The next step involves an extensive surgical approach — including duodenal surgery — directed at the MEN1-related gastrinoma. The surgical rationale for MEN1-related gastrinomas is distinct from that for other functioning tumours in this setting. Full clinical indication, timing considerations, and procedural details are available in the complete protocol.
DOI: 10.1016/S2213-8587(25)00119-6
Gastrinomas in patients with MEN1 are invariably located in the duodenum and are multiple, with a median age of onset in the third to fifth decade of life.
However, extensive surgery that includes duodenal surgery is required for MEN1-related gastrinomas, and there is a clinical dilemma in deciding on its timing, as often patients are young and might also have concomitant pancreatic neuroendocrine tumours.
Thus, the primary goal of surgery for gastrinomas, unlike other functioning pancreatic neuroendocrine tumours associated with MEN1, is to reduce the risk of metastatic disease.
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