A total gastrectomy performed was due to severity of achalasia symptoms conditioning a high degree of malnutrition. Two years after discharge the patient underwent surgery for total dysphagia, epigastric pain and inexorable weight loss. Primary malignancies of the jejunum and ileum are extremely rare and many pathogenetic hypotheses were done but none of them received clinical or experimental demonstration. Symptoms are not specific and diagnosis is difficult and late, x-rays and endoscopy leading to it, US, CT and MNR are useful for differential diagnosis and staging. Prognosis is severe.
Surgical therapy is large resection, if possible, but often it is only palliative.
AB - Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. A large tumor in the descending duodenum had given rise to multiple lymph node metastases. An additional 31 duodenal somatostatinoma cases were also reviewed.