Aetiology
Neuroendocrine neoplasms of the ampulla of Vater are rare tumors that form in the intestinal tract, with an estimated incidence of 1–2 cases per million inhabitants per year. They arise from the enteroendocrine cells that line the ampulla of Vater.
Diagnosis
Diagnosis of Neuroendocrine neoplasms of the ampulla of Vater is based on clinical presentation, imaging studies (eg, endoscopic ultrasound, CT scan, MRI), laboratory tests (eg, carcinoembryonic antigen, chromogranin A, urinary 5-hydroxyindoleacetic acid) and histological evaluation.
Differential diagnosis
The differential diagnosis of Neuroendocrine neoplasms of the ampulla of Vater includes adenocarcinoma, pancreatic ductal adenocarcinoma, duodenal adenocarcinoma, and other gastrointestinal tumors.
Treatment
Treatment of Neuroendocrine neoplasms of the ampulla of Vater depends on the size and stage of the tumor. Treatment options include surgical resection, chemotherapy, and radiation therapy.
Prognosis
The prognosis for Neuroendocrine neoplasms of the ampulla of Vater depends on the stage of the tumor at the time of diagnosis, but the overall 5-year survival rate is approximately 50%. Patients with localized tumors have a better prognosis than those with advanced tumors.