title: Neuroendocrine neoplasms of the small intestine and the appendix - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours).
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.
authors.forename: Marek
authors.initials: M
authors.lastname: Bolanowski
authors.forename: Tomasz
authors.initials: T
authors.lastname: Bednarczuk
authors.forename: Barbara
authors.initials: B
authors.lastname: Bobek-Billewicz
authors.forename: Daria
authors.initials: D
authors.lastname: Handkiewicz-Junak
authors.forename: Arkadiusz
authors.initials: A
authors.lastname: Jeziorski
authors.forename: Ewa
authors.initials: E
authors.lastname: Nowakowska-Duława
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Steinhof-Radwańska
authors.forename: Wojciech
authors.initials: W
authors.lastname: Zajęcki
authors.forename: Anna
authors.initials: A
authors.lastname: Zemczak
authors.affiliation: Division of Endocrinology, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland. endoklin@sum.edu.pl.
authors.forename: Beata
authors.initials: B
authors.lastname: Kos-Kudła
mesh_terms: D001063:Appendiceal Neoplasms / Q000175:diagnosis / Q000628:therapy; D002983:Clinical Competence; D004704:Endocrinology / Q000592:standards; D006801:Humans; D007414:Intestinal Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D008495:Medical Oncology / Q000592:standards; D018358:Neuroendocrine Tumors / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D010808:Physical Examination; D011044:Poland; D011785:Quality Assurance, Health Care / Q000592:standards*