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Neuroendocrine neoplasms of the small intestine and the appendix - Update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

Bednarczuk T, Zemczak A, Bolanowski M, Borowska M, Chmielik E, Cwikla JB, Foltyn W, Gisterek I, Handkiewicz-Junak D, Hubalewska-Dydejczyk A, Jarzab M, Junik R, Kajdaniuk D, Kaminski G, Kolasinska-Cwikla A, Kopacz-Wróbel K, Kowalska A, Królicki L, Kunikowska J, Kusnierz K, Lewinski A, Liszka L, Londzin-Olesik M, Marek B, Malczewska A, Nasierowska-Guttmejer A, Nowakowska-Dulawa E, Pavel ME, Pilch-Kowalczyk J, Regula J, Rosiek V, Ruchala M, Rydzewska G, Sieminska L, Sowa-Staszczak A, Starzynska T, Stojcev Z, Strzelczyk J, Studniarek M, Syrenicz A, Szczepkowski M, Wachula E, Zajecki W, Zgliczynski W, Zieniewicz K, Kos-Kudla B (2022)

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Publication Type: Journal article

Publication year: 2022

Journal

Book Volume: 73

Pages Range: 549-583

Journal Issue: 3

DOI: 10.5603/EP.a2022.0052

Abstract

Updated Polish recommendations for the management of patients with neuroendocrine neoplasms (NENs) of the small intestine (SINENs) and of the appendix (ANENs) are presented herein. The small intestine, and especially the ileum, is one of the most common locations for these neoplasms. Most of them are well-differentiated and slow-growing tumours; uncommonly - neuroendocrine carcinomas. Their symptoms may be untypical, and their diagnosis may be delayed or accidental. Most often, the first manifestation of ANEN is their acute inflammation. Typical symptoms of carcinoid syndrome occur in approximately 20-30% of SINENs patients with distant metastases. In laboratory diagnostics, it is most useful to determine the concentration of chromogranin A (CgA) and assessment of urinary or plasma the 5-hydroxyindoleacetic acid concentration is helpful in the diagnosis of carcinoid syndrome. The most commonly used imaging methods are ultrasound (US) examination, computed tomography (CT), magnetic resonance imaging (MRI), colonoscopy, and somatostatin receptor imaging (SRI). Histopathological examination is crucial for the proper diagnosis and treatment of patients with SINENs and ANENs. The treatment of choice is a surgical procedure, either radical or palliative. Long-acting somatostatin analogues (SSAs) are essential in the medical treatment of functional and non-functional SINENs. In patients with SINENs, at the stage of dissemination with progression during SSAs treatment, with high expression of somatostatin receptors on functional imaging, radioligand therapy should be considered first followed by targeted therapies - everolimus. Recommendations for patient monitoring are also presented.

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How to cite

APA:

Bednarczuk, T., Zemczak, A., Bolanowski, M., Borowska, M., Chmielik, E., Cwikla, J.B.,... Kos-Kudla, B. (2022). Neuroendocrine neoplasms of the small intestine and the appendix - Update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych). Endokrynologia Polska, 73(3), 549-583. https://doi.org/10.5603/EP.a2022.0052

MLA:

Bednarczuk, Tomasz, et al. "Neuroendocrine neoplasms of the small intestine and the appendix - Update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)." Endokrynologia Polska 73.3 (2022): 549-583.

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