Gastric and duodenal carcinoid tumours. Prevalence, tumour biology, diagnosis and surgical therapy
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4 Hellenic Journal of Surgery 2011; 83: 1
Gastric and Duodenal Carcinoid Tumours. Prevalence, Tumour Biology, Diagnosis and Surgical Therapy Review Article G. Papageorgiou, Ev. Lolis Received 22/10/2010 Accepted 22/11/2010
Abstract Carcinoid tumours is the most accepted term for neuroendocrine tumours, or neuroendocrine carcinomas. Gastric and duodenal carcinoid tumours, though rare, have been the focus of much scientific investigation. These neoplasms pose a diagnostic and therapeutic challenge. Despite the latest available therapies, the incidence of gastric carcinoid appears to be rising, mostly due to the increased application of gastroscopy, without a corresponding increase in survival. Subtyping of gastric and duodenal carcinoids (four types, according to their origin and histologic variations) is helpful in the prediction of malignant potential and long-term survival and as a guide to management. Advances in our understanding of the pathogenesis of gastric carcinoids and associate diseases have led to recent improvements in investigation and management. Surgery remains the main curative modality.
Keywords
Carcinoid tumours, Gastric neuroendocrine tumours, Duodenal neuroendocrine tumours, MEN 1 syndrome
Introduction Carcinoid tumours, now referred to as neuroendocrine tumours (NETs), encompass a wide range of neoplasms and clinical behaviours depending on their site of origin, hormonal production, and differentiation [1]. Though relatively rare, carcinoid tumours represent the most common gastrointestinal NETs [2-4]. Tumours arising from different segments of the embryologic gut varied in their histologic, immunohistochemical, and bioactive amine production. In 2000, a newer classification was developed by the World Health Organization which included not only the site of origin, but also histologic variations that were more predictive of biologic behaviour [5-7]. Using this system, tumours are classified into welldifferentiated NET tumours and well-differentiated 2nd Surgical department Evangelismos General Hospital, Athens, Greece e-mail: [email protected]
and poorly-differentiated NET carcinomas. Today the term carcinoid is preferably used for tumours that arise in the GI (gastrointestinal) tract that can produce serotonin or cause carcinoid syndrome. This term primarily refers to midgut small bowel carcinoids, whereas the remaining ‘‘carcinoid’’ tumours should be termed NETs, followed by their site of origin and the endocrinopathy, if present. Neuroendocrine cells occur throughout the length of the GI tract and are the largest group of hormone-producing cells in the body [8,9]. Neuroendocrine cells are derived from multipotent stem cells and not, as originally thought, from the migration of neural crest cells. There are at least 13 types of gut neuroendocrine cells which produce various bioactive peptides and amines including serotonin, gastrin, and histamine [1,6]. The density of these various neuroendocrine cells in a given anatomic segment of the gut is dictated by the physiologic needs of that si
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