Somatostatinoma of the Vater's papilla in a patient with von Recklinghausen's disease

Abstract

Somatostatinomas of the gastrointestinal tract secret hormon somatostatin which can cause "inhibitory syndrom" comprising diabetes mellitus, cholelithiasis and steatorrheic diarrhea. It is also secreted by the D cells of Langerhans's islands of the pancreas as well as endocrine cells of the stomack, small bowel, salivary glands and parafollicular cells of the thiroid gland. Somatostatinomas of the digestive tract appear within the pancreas and duodenum. Patients suffering from von Recklinghausens's disease are paticularly prone to the somatostatinomas of the duodenum. In this paper we presented a 51-year-old female patient with von Recklinghausen's disease in whom, during the investigation for obstructive jaundice, tumor of the Vater's papilla was found. The patient was submitted to Whipple's duodenopancreatectomy. Histology and immunohistochemistry discovered type B glandular carcinoid tumor with strong antisomatostatin and mild antigastrin immunoreactivity. The patient stayed symptom-free more than four years now. Patients with von Recklinghausen's disease should be examined for other tumors, particularly carcinoids of the duodenum and papilla, especially if the signs of cholestasis are present.