Multiple gastric endocrine tumours and gastrinomas of the duodenum in a patient with ZES MEN 1

Abstract

An asymptomatic 39-year-old man, coming from a family aving a genetic nonsense mutation (type Arg415Stop, exon ) for multiple endocrine neoplasia type 1 (MEN 1), was een in our Unit to undergo screening tests, because he was carrier of the same mutation. Laboratory evaluation revealed high levels of gasrin (41,400 pg/ml, nv 20–100 pg/ml), chromogranin (17,000 U/l, nv <17 U/l), calcium (12.9 mg/dl, nv –11.5 mg/dl) and parathyroid hormone (325 pg/ml, nv 4–72 pg/ml). The patient underwent upper-gastrointestinal endoscopy hich revealed, in the gastric body and fundus (Fig. 1), ultiple polyps which involved almost the entire mucosa. e did not find any lesions in the antrum. The gastric olyps varied from 1–2 mm to 3 cm. In addition, in the econd portion of the duodenum, there were sessile polyps hich varied from 1 cm to 3 cm (Fig. 2). The gastric pH as 1.5. Pathological examination of multiple biopsy pecimens showed a well-differentiated endocrine tumour gastric carcinoid type II: chromogranin positive and gastrin egative) in the stomach and a well-differentiated gastrinroducing endocrine tumour of the duodenum (gastrinoma). his diffuse gastric carcinoidosis is the consequence f the trophic effect on the entero-chromaffin-like cells ECL) of the gastric body and fundus induced by gastrin ecreted by the duodenal gastrinomas in a patient with ZES EN 1.