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Neuroendocrine neoplasms of the small intestine and the appendix — management guidelines (recommended by the Polish Network of Neuroendocrine Tumours)
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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)
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)
Keywords
neuroendocrine neoplasms; small intestine; appendix; carcinoid; diagnostics; therapy; guidelines
About this articleTitle
Neuroendocrine neoplasms of the small intestine and the appendix — management guidelines (recommended by the Polish Network of Neuroendocrine Tumours)
Journal
Issue
Article type
Review paper
Pages
480-493
Published online
2013-12-31
Page views
3763
Article views/downloads
4140
DOI
10.5603/EP.2013.0029
Bibliographic record
Endokrynol Pol 2013;64(6):480-493.
Keywords
neuroendocrine neoplasms
small intestine
appendix
carcinoid
diagnostics
therapy
guidelines
Authors
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
