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World J Gastroenterol. Apr 14, 2010; 16(14): 1696-1706
Published online Apr 14, 2010. doi: 10.3748/wjg.v16.i14.1696
Recent trends in the treatment of well-differentiated endocrine carcinoma of the small bowel
Gilles Poncet, Jean-Luc Faucheron, Thomas Walter
Gilles Poncet, Department of Digestive Surgery, Edouard Herriot Hospital, 69437 Lyon cedex 03, France
Jean-Luc Faucheron, Department of Colorectal Surgery, Michallon Hospital, 38043 Grenoble cedex, France
Thomas Walter, Department of Medical Oncology, Pavillon H, Edouard Herriot Hospital, 69437 Lyon Cedex 03, France
Author contributions: Poncet G, Faucheron JL and Walter T analyzed the literature and wrote the paper.
Correspondence to: Thomas Walter, MD, Department of Medical Oncology, Pavillon H, Edouard Herriot Hospital, 69437 Lyon Cedex 03, France. thomas.walter@chu-lyon.fr
Telephone: +33-4-72110094 Fax: +33-4-72119153
Received: December 28, 2009
Revised: February 4, 2010
Accepted: February 11, 2010
Published online: April 14, 2010
Abstract

Well-differentiated endocrine carcinomas of the small bowel are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that may cause carcinoid syndrome. However, many are clinically silent until late presentation with major effects. Initial treatment aims to control carcinoid syndrome with somatostatin analogs. Even if there is metastatic spread, surgical resection of the primitive tumor should be discussed in cases of retractile mesenteritis, small bowel ischemia or subocclusive syndrome in order to avoid any acute complication, in particular at the beginning of somatostatin analog treatment. The choice of treatment depends on the symptoms, general health of the patient, tumor burden, degree of uptake of radionuclide, histological features of the tumor, and tumor growth. Management strategies include surgery for cure (which is rarely achieved) or for cytoreduction, radiological interventions (transarterial embolization or radiofrequency ablation), and chemotherapy (interferon and somatostatin analogs). New biological agent and radionuclide targeted therapies are under investigation. Diffuse and non-evolving lesions should also be simply monitored. Finally, it has to be emphasized that it is of the utmost importance to enroll these patients with a rare disease in prospective clinical trials assessing new therapeutic strategies.

Keywords: Gastrointestinal neoplasms; Neuroendocrine carcinoma; Carcinoid tumor; Somatostatin analogs; Therapeutic chemoembolization; Surgery