open access

Vol 73, No 3 (2022)
Guidelines / Expert consensus
Submitted: 2022-04-27
Accepted: 2022-04-28
Published online: 2022-06-30
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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)]

Tomasz Bednarczuk1, Anna Zemczak2, Marek Bolanowski3, Małgorzata Borowska4, Ewa Chmielik5, Jarosław B. Ćwikła6, Wanda Foltyn2, Iwona Gisterek7, Daria Handkiewicz-Junak8, Alicja Hubalewska-Dydejczyk9, Michał Jarząb10, Roman Junik11, Dariusz Kajdaniuk12, Grzegorz Kamiński13, Agnieszka Kolasińska-Ćwikła14, Karolina Kopacz-Wróbel15, Aldona Kowalska16, Leszek Królicki17, Jolanta Kunikowska17, Katarzyna Kuśnierz18, Andrzej Lewiński19, Łukasz Liszka20, Magdalena Londzin-Olesik2, Bogdan Marek12, Anna Malczewska2, Anna Nasierowska-Guttmejer21, Ewa Nowakowska-Duława22, Marianne E. Pavel23, Joanna Pilch-Kowalczyk24, Jarosław Reguła25, Violetta Rosiek2, Marek Ruchała26, Grażyna Rydzewska27, Lucyna Siemińska12, Anna Sowa-Staszczak9, Teresa Starzyńska28, Zoran Stojčev29, Janusz Strzelczyk2, Michał Studniarek30, Anhelli Syrenicz31, Marek Szczepkowski32, Ewa Wachuła33, Wojciech Zajęcki4, Wojciech Zgliczyński34, Krzysztof Zieniewicz35, Beata Kos-Kudła2
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Pubmed: 36059174
·
Endokrynol Pol 2022;73(3):549-583.
Affiliations
  1. Department of Internal Medicine and Endocrinology, Medical University of Warsaw, Warsaw, Poland
  2. Department of Endocrinology and Neuroendocrine Tumours, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland
  3. Chair and Department of Endocrinology, Diabetes, and Isotope Therapy, Medical University of Wroclaw, Wroclaw, Poland
  4. Department of Endocrinology and Neuroendocrine Tumours, Medical University of Silesia, Katowice, Poland
  5. Tumor Pathology Department, Maria Sklodowska-Curie Memorial National Research Institute of Oncology, Gliwice Branch, Gliwice, Poland
  6. Department of Cardiology and Internal Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, Poland
  7. Chair of Oncology and Radiotherapy, Medical University of Silesia, Katowice, Poland
  8. Department of Nuclear Medicine and Endocrine Oncology, Maria Sklodowska-Curie Memorial National Research Institute of Oncology, Gliwice Brench, Gliwice, Poland
  9. Chair and Department of Endocrinology, Jagiellonian University Medical College, Cracow, Poland
  10. Breast Unit, Maria Sklodowska-Curie Memorial National Research Institute of Oncology, Gliwice Branch, Gliwice, Poland
  11. Department of Endocrinology and Diabetology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Bydgoszcz, Poland
  12. Division of Pathophysiology, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland
  13. Department of Endocrinology and Radioisotope Therapy, Military Institute of Medicine, Warsaw, Poland
  14. Department of Oncology and Radiotherapy, Maria Sklodowska-Curie Memorial National Research Institute of Oncology, Warsaw, Poland
  15. Department od Radiology and Nuclear Medicine, Medical University of Silesia, Katowice, Poland
  16. Department of Endocrinology, Holycross Cancer Centre, Collegium Medicum, Jan Kochanowski University, Kielce, Poland
  17. Nuclear Medicine Department, Medical University of Warsaw, Warsaw, Poland
  18. Department of Gastrointestinal Surgery, Medical University of Silesia, Katowice, Poland
  19. Department of Endocrinology and Metabolic Diseases, Medical University of Lodz, Lodz, Poland
  20. Department of Pathomorphology and Molecular Diagnostics, Medical University of Silesia, Katowice, Poland
  21. Faculty of Medicine, Lazarski University in Warsaw, Warsaw, Poland
  22. Department of Gastroenterology and Hepatology, Medical University of Silesia, Katowice, Poland
  23. Department of Medicine 1, Endocrinology and Diabetology, Friedrich Alexander University of Erlangen-Nürnberg, Erlangen, Germany
  24. Department of Radiology and Nuclear Medicine, Medical University of Silesia, Katowice, Poland;
  25. Department of Oncological Gastroenterology, Maria Sklodowska-Curie Memorial National Research Institute of Oncology, Warsaw, Poland
  26. Department of Endocrinology, Metabolism and Internal Diseases, Medical University in Poznan, Poznan, Poland
  27. Department of Internal Medicine and Gastroenterology, Central Clinical Hospital of the Ministry of Interior and Administration, Warsaw, Poland
  28. Department of Gastroenterology, Medical Pomeranian University in Szczecin, Szczecin, Poland
  29. Department of Oncology and Breast Diseases, Centre of Postgraduate Medical Education, Warsaw, Poland
  30. Department of Radiology, Medical University of Gdansk, Gdansk, Poland
  31. Department of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University, Szczecin, Poland
  32. Clinical Department of Colorectal, General and Oncological Surgery, Centre of Postgraduate Medical Education, Warsaw, Poland
  33. Department of Clinical Oncology, Gdynia Oncology Centre of the Polish Red Cross Maritime Hospital, Gdynia, Poland
  34. Department of Endocrinology, Centre of Postgraduate Medical Education, Warsaw, Poland
  35. Chair and Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, Warsaw, Poland

open access

Vol 73, No 3 (2022)
Guidelines
Submitted: 2022-04-27
Accepted: 2022-04-28
Published online: 2022-06-30

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 here. 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. Najczęściej pierwszą manifestacją ANEN jest jego ostre zapalenie. Typical symptoms of carcinoid syndrome occur in approximately 20–30% of SINENs patients with distant metastases. In laboratory diagnostics the assessment of 5-hydroxyindoleacetic acid concentration is helpful in the diagnosis of carcinoid syndrome. The most commonly used imaging methods are ultrasound examination, computed tomography, magnetic resonance imaging, colonoscopy and somatostatin receptor imaging. 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 dissemination with progression during SSAs treatment, with high expression of somatostatin receptors, radioisotope therapy should be considered first followed by targeted therapies — everolimus. After the exhaustion of the above available therapies, chemotherapy may be considered in selected cases. Recommendations for patient monitoring are also presented.

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 here. 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. Najczęściej pierwszą manifestacją ANEN jest jego ostre zapalenie. Typical symptoms of carcinoid syndrome occur in approximately 20–30% of SINENs patients with distant metastases. In laboratory diagnostics the assessment of 5-hydroxyindoleacetic acid concentration is helpful in the diagnosis of carcinoid syndrome. The most commonly used imaging methods are ultrasound examination, computed tomography, magnetic resonance imaging, colonoscopy and somatostatin receptor imaging. 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 dissemination with progression during SSAs treatment, with high expression of somatostatin receptors, radioisotope therapy should be considered first followed by targeted therapies — everolimus. After the exhaustion of the above available therapies, chemotherapy may be considered in selected cases. Recommendations for patient monitoring are also presented.

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Keywords

neuroendocrine neoplasms; neuroendocrine tumours; recommendations; small intestine; appendix; carcinoid syndrome; somatostatin analogues; radioligand therapy; targeted therapies

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Title

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)]

Journal

Endokrynologia Polska

Issue

Vol 73, No 3 (2022)

Article type

Guidelines / Expert consensus

Pages

549-583

Published online

2022-06-30

Page views

5868

Article views/downloads

1921

DOI

10.5603/EP.a2022.0052

Pubmed

36059174

Bibliographic record

Endokrynol Pol 2022;73(3):549-583.

Keywords

neuroendocrine neoplasms
neuroendocrine tumours
recommendations
small intestine
appendix
carcinoid syndrome
somatostatin analogues
radioligand therapy
targeted therapies

Authors

Tomasz Bednarczuk
Anna Zemczak
Marek Bolanowski
Małgorzata Borowska
Ewa Chmielik
Jarosław B. Ćwikła
Wanda Foltyn
Iwona Gisterek
Daria Handkiewicz-Junak
Alicja Hubalewska-Dydejczyk
Michał Jarząb
Roman Junik
Dariusz Kajdaniuk
Grzegorz Kamiński
Agnieszka Kolasińska-Ćwikła
Karolina Kopacz-Wróbel
Aldona Kowalska
Leszek Królicki
Jolanta Kunikowska
Katarzyna Kuśnierz
Andrzej Lewiński
Łukasz Liszka
Magdalena Londzin-Olesik
Bogdan Marek
Anna Malczewska
Anna Nasierowska-Guttmejer
Ewa Nowakowska-Duława
Marianne E. Pavel
Joanna Pilch-Kowalczyk
Jarosław Reguła
Violetta Rosiek
Marek Ruchała
Grażyna Rydzewska
Lucyna Siemińska
Anna Sowa-Staszczak
Teresa Starzyńska
Zoran Stojčev
Janusz Strzelczyk
Michał Studniarek
Anhelli Syrenicz
Marek Szczepkowski
Ewa Wachuła
Wojciech Zajęcki
Wojciech Zgliczyński
Krzysztof Zieniewicz
Beata Kos-Kudła

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