Clinical Scenario

This protocol addresses Multiple endocrine neoplasia type 1 (MEN1) presenting with a type 2 gastric neuroendocrine tumour (also referred to as an enterochromaffin-like cell carcinoid) in the setting of coexistent hypergastrinaemia. These tumours arise in a substantial proportion of MEN1 patients and are frequently identified incidentally during upper gastrointestinal endoscopy.

Key Clinical Context

Type 2 gastric neuroendocrine tumours in MEN1 develop against a background of elevated gastrin levels. Active evaluation of tumour size and growth behaviour is essential, as these factors determine the choice and intensity of intervention.

Approach Overview

For larger or actively growing tumours, management may involve endoscopic intervention or a medical approach with the goal of achieving tumour regression. In more extensive cases or when regional spread is a concern, surgical options may be considered.

The complete treatment algorithm, specific criteria, and follow-up schedule are available in the full protocol below.

References

Type 2 gastric neuroendocrine tumours (also referred to as enterochromaffin-like cell carcinoids) are observed in 15–70% of patients with MEN1 with coexistent hypergastrinaemia and are frequently detected incidentally at the time of upper gastrointestinal endoscopy.
Larger or growing gastric neuroendocrine tumours can be treated by endoscopic resection, although treatment with somatostatin analogues (octreotide or lanreotide), has also been reported to result in regression of these tumours.
Gastric surgery can be considered for more extensive tumours or those with spread to locoregional lymph nodes, as the risk of metastases has been reported to be between 10% and 30%.

DOI: 10.1016/S2213-8587(25)00119-6

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Treatment of MEN1 with Type 2 Gastric Neuroendocrine Tumour and Hypergastrinaemia

References