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Annals of Gastroenterology
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Report (EN)

2007 (EN)
Somatostatinoma syndrome (EN)

Christopoulos C.,
Economopoulos P.,

SUMMARY Somatostatinomas are extremely rare functioning endocrine tumors of the gastrointestinal tract, occurring with almost equal frequency in the pancreas (mainly in the head) and duodenum (periampullary region). The latter are often associated with von Recklinghausen disease (50%). They occur sporadically (93.1%) or as part of multiple endocrine neoplasia type 1 (6.9%). The tumors are relatively large (with an average size of 5 cm for those of pancreatic and 2.5 cm for those of duodenal origin) and usually malignant (64.7%) with metastases mainly to the lymph nodes (31.2%) and liver (27.7%). Duodenal somatostatinomas are characterized by the frequent presence of psammoma bodies. Clinical manifestations are characterized by two distinct entities, one due to somatostatin hypersecretion (inhibitory syndrome) and the other due to tumor location and growth. The inhibitory syndrome includes diabetes mellitus or glucose intolerance, cholelithiasis, weight loss, diarrhea with or without steatorrhea, and hypochlorhydria or complete achlorhydria. Clinical manifestations due to tumor location and growth include mainly obstructive jaundice, duodenal obstruction, weight loss, and gastrointestinal bleeding. Mixed clinical manifestations may occur in cases of multiple hormone secretion by the tumor (e.g. Cushing’s syndrome, peptic ulcer etc.). The diagnosis is usually accidental at the time of laparotomy for cholecystectomy or during gastrointestinal imaging studies for various nonspecific complaints. For the definitive diagnosis of “somatostatinoma ” histology with immunocytochemistry is mandatory. The localization of tumor or metastases may be made by imaging methods, mainly by endoscopic U/S (87.2%), and angiography (82.1%) or by surgical exploration. Treatment of choice is the surgical removal of tumor and, when possible, of metastases, with or without adjuvant chemotherapy. Due to their slow natural course, somatostatinomas have a better prognosis than pancreatic or biliary duct cancer. (EN)




Hellenic Society of Gastroenterology (EN)

Annals of Gastroenterology; Volume 14, No 4 (2001) (EN)

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