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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2013 January-February;172(1-2):109-16

language: English, Italian

Anasarca as onset of an occult neuroendocrine tumor: a case report

Zagari D. 1, Zinnarello C. 2, Di Bartolo C. E. 3, Puntorieri E. 4, Leonardi V. 5, Mileto G. 3

1 Unit of Coronary Intensive Therapy, Department of Internal Medicine and, Clinical Pharmacology, Policlinico Universitario G. Martino, Messina, Italy;
2 Unit of Respiratory Diseases, Department of Cardiovascular and Thoracic Diseases, Policlinico Universitario G. Martino, Messina, Italy;
3 Unit of Internal and Gastrointestinal Medicine, Department of Internal Medicine, Policlinico Universitario G. Martino, Messina, Italy;
4 Unit of Nephrology and Dialysis, Department of Internal Medicine, Policlinico Universitario G. Martino, Messina, Italy;
5 Unit of Psychiatry, Department of Neurosciences, Psychiatric and Anesthesiological Sciences, Policlinico Universitario G. Martino, Messina, Italy


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The anasarca is characterized by generalized edema often associated with effusions in the large serous cavities. The pathogenesis is due to an alteration of the pathophysiological mechanisms (Hydrostatic pressure/Oncotic pressure) that regulate the intra- and extravascular compartmentalization of body fluids. An increase in hydrostatic pressure (cardiopatic or nephrological pathologies) or a decreasing of the oncotic pressure, owing to hypoproteinemia deriving from lack of protein synthesis or from protidodispersion leads to onset of edema and anasarca. A severe protidodispersion can occur in course of protein-losing enteropathy (PLE), characterized by an excessive loss of plasma proteins from the gastrointestinal tract: it should be suspected in every case of severe hypoproteinemia not justified by malnutrition, burns, nephropathies or hepatopathies, etc. The diagnosis is based on the clearance of faecal α1-antitrypsin (α1-AT) and endoscopic, histologic, radiologic, radioisotopic investigations. The PLE can be primary or secondary to Right Heart Failure or to Neuroendocrine Tumor (NET), a very heterogeneous group of ubiquitarious tumors, slow-growing, often occult, secreting or not, characterized by the ability to sythesize precursors or active fragments or inactive of amines or peptide hormones. These substances, numerous and often undetectable and/or not easily identifiable, can induce both retroperitoneal and intestinal lymphatic vessels fibrosis and then PLE, but can also induce endocardial and right heart valves fibrosis and right heart failure, which is the main cause of death in patients with a NET-induced cardiopathy. The onset of PLE caused by a NET may be a consequence of tumoral secretion of hormones acting on the intestinal lymphatic vessels or of the right heart failure induced by NET. We report a case of an occult NET. This case report finds its originality in the way of presentation of an occult primary NET begun with a severe anasarca.

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