Neuroendocrine tumors of the small intestine and the appendix - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)

Marek Bolanowski; Barbara Jarząb; Daria Handkiewicz-Junak; Arkadiusz Jeziorski; Beata Kos-Kudła; Wojciech Zajęcki oraz pozostali uczestni Stołu

Vol 59, No 1 (2008)

Review paper

Submitted: 2013-02-15

Published online: 2008-01-21

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Neuroendocrine tumors of the small intestine and the appendix - management guidelines (recommended by The Polish Network of Neuroendocrine Tumors)

Marek Bolanowski, Barbara Jarząb, Daria Handkiewicz-Junak, Arkadiusz Jeziorski, Beata Kos-Kudła, Wojciech Zajęcki oraz pozostali uczestni Stołu

Endokrynol Pol 2008;59(1):87-96.

Vol 59, No 1 (2008)

Reviews — Postgraduate Education

Submitted: 2013-02-15

Published online: 2008-01-21

Abstract

Polish recommendations regarding management of patients suffering from neuroendocrine tumors of small intestine and appendix are presented. Small intestine, especially ileum represent most common origin of these tumors. Majority of them are well differentiated and grow slowly. Rarely, they are less differentiated with fast growth and poor prognosis. Symptoms are atypical, diagnosis could be often accidental. In 4-10% of patients typical symptoms of carcinoid syndrome are present. Chromogranin A is useful in the laboratory diagnostics, and urinary excretion of 5-hydroxyindoloacetic acid is helpuf for the diagnostics and monitoring of the disease. Histopathological diagnostics was extensively described. Ultrasound, colonoscopy, capsule endoscopy, baloon enteroscopy, computed tomography, magnetic resonance and somatostatin analogs scintigraphy could be used for the visualization. The treatment of choice in the neuroendocrine tumors of small intestine and appendix is radical or palliative surgery, if possible using endoscopy. Pharmacotherapy consists of biotherapy and chemotherapy. The crucial in biotherapy is somatostatin analogs application, possible in symptomatic treatment of hormonally functioning tumors. This is treatment of choice in carcinoid crisis. Interferon alfa could be applied because of the same indications as somatostatin analogs, except for carcinoid crisis. Chemotherapy is less successful in disseminated or locally advanced intestinal neuroendocrine tumors, so radioisotope therapy should be considered in each case of unresectable tumor. Pol J Endocrinol 2008; 59 (1): 87-96