Vol 64, No 6 (2013)
Review paper
Published online: 2013-12-31

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Neuroendocrine neoplasms of the small intestine and the appendix — management guidelines (recommended by the Polish Network of Neuroendocrine Tumours)

Marek Bolanowski, Tomasz Bednarczuk, Barbara Bobek-Billewicz, Daria Handkiewicz-Junak, Arkadiusz Jeziorski, Ewa Nowakowska-Duława, Katarzyna Steinhof-Radwańska, Wojciech Zajęcki, Anna Zemczak, Beata Kos-Kudła, Other participants of the Consensus Conference (affiliations at the end of this section):, Elżbieta Andrysiak-Mamos, Jolanta Blicharz-Dorniak, Andrzej Cichocki, Jarosław B. Ćwikła, Andrzej Deptała, Wanda Foltyn, Marek Hartleb, Alicja Hubalewska-Dydejczyk, Michał Jarząb, Dariusz Kajdaniuk, Grzegorz Kamiński, Aldona Kowalska, Robert Król, Leszek Królicki, Jolanta Kunikowska, Katarzyna Kuśnierz, Paweł Lampe, Dariusz Lange, Anna Lewczuk, Magdalena Londzin-Olesik, Przemysław Majewski, Bogdan Marek, Gabriela Mełeń-Mucha, Anna Nasierowska-Guttmejer, Andrzej Nowak, Waldemar Patkowski, Joanna Pilch-Kowalczyk, Violetta Rosiek, Marek Ruchała, Sławomir Rudzki, Philippe Ruszniewski, Grażyna Rydzewska, Anna Sowa-Staszczak, Teresa Starzyńska, Janusz Strzelczyk, Piotr Zdunowski
DOI: 10.5603/EP.2013.0029
Endokrynol Pol 2013;64(6):480-493.

Abstract

We present revised Polish guidelines regarding the management of patients harbouring neuroendocrine neoplasms (NENs) of the small intestine and appendix. The small intestine, especially the ileum, is the most common origin of these neoplasms. Most of them are well differentiated with slow growth. Rarely, they are less differentiated, growing fast with a poor prognosis. Since symptoms can be atypical, the diagnosis is often accidental. Typical symptoms of carcinoid syndrome occur in less than 10% of patients. The most useful laboratory marker is chromogranin A; 5-hydroxyindoleacetic acid is helpful in the monitoring of carcinoid syndrome. Ultrasound, computed tomography, magnetic resonance imaging, colonoscopy, video capsule endoscopy, balloon enteroscopy and somatostatin receptors scintigraphy are used in the visualisation. A histological report is crucial for the proper diagnostics and therapy of NENs, and it has been extensively described. The treatment of choice is surgery, either radical or palliative. Somatostatin analogues are crucial in the pharmacological treatment of the hormonally active and non-active small intestine NENs and NENs of the appendix. Radioisotope therapy is possible in patients with a good expression of somatostatin receptors. Chemotherapy is not effective in general. Everolimus therapy can be applied in patients with generalised NENs of the small intestine in progression and where there has been a failure or an inability to use other treatment options. Finally, we make recommendations regarding the monitoring of patients with NENs of the small intestine and appendix. (Endokrynol Pol 2013; 64 (6): 480–493)