Duodenal somatostatinoma

Abstract

Aim -Background: Somatostatinomas is a very rare neuroendocrine tumour of the gastrointestinal tract, first described in the pancreas in 1977 and in the duodenum in 1979. We present the case a 67-year-old woman with duodenal somatostatinoma that was resected with a pancreaduodenectomy procedure and also provide a brief review of the literature. Method: Databases from Pubmed and Medline were searched using a list of keywords such as somatostatinoma, duodenum, pancreas, neuroendocrine tumour and references from review articles.The study of such a case and those in the literature provides useful knowledge into the clinical management and diagnosis of these patients. Results: The search of the literature yielded 348 articles, 92 reviews, 175 case reports, 328 journal articles, 6 clinical trials, 1 editorial, 21 comparative studies and 3 multicenter studies. Forty-five articles related to type-1 neurofibromatosis were found, 5 associated with von Hippel-Lindau disease and 33 associated with multiple endocrine neoplasia type-1. Around 201 cases of somatostatinoma were reported Conclusion: Two different symptomatic presentations can be found that include the inhibitory syndrome in pancreatic somatostatinomas, whereas tumours arising from the duodenal wall are usually associated with mechanical enteric obstructive symptoms. The clinical symptoms are often variable and non-specific; many somatostatinomas are found incidentally during cholecystectomy or during gastrointestinal imaging studies. Surgery is the standard treatment and it is also preferred in metastatic disease providing diagnosis is made early. Patients with metastatic somatostatinoma display 5-year postoperative survival rates of 30-60% as opposed to those without metastasis whose 5-year survival rate approaches 100%.