Introduction.

Gangliocytic paraganglioma is a rare histological entity, with the duodenum being its main extra-nodal location.

Clinical case.

This is a 54-year-old man who presented with abdominal pain and upper gastrointestinal bleeding. A suspicious ampuloma lesion was diagnosed, for which a pancreaticoduodenectomy was performed. In the histological analysis, the diagnosis of metastatic gangliocytic paraganglioma was confirmed by the pathognomonic presence of three cell lines (epithelial, ganglionic, and Schwann-like). Given his good prognosis associated with low chemosensitivity, he did not receive adjuvant treatment.

Results.

During follow-up, the patient did not present late complications or signs of recurrence one year after the intervention.

Conclusion.

Gangliocytic paraganglioma is a potentially malignant entity that requires a correct extension study and close long-term follow-up.

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