open access

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

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
·
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.

Get Citation

Keywords

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

Supp./Additional Files (2)
Supplementary File [Materiały dodatkowe] — Tab. S1, S2
Download
80KB
Conflict of interests [Konflikt interesów]
Download
429KB
About this article
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

5726

Article views/downloads

1786

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

References (126)
  1. Auernhammer C, Spitzweg C, Angele M, et al. Advanced neuroendocrine tumours of the small intestine and pancreas: clinical developments, controversies, and future strategies. Lancet Diabetes Endocrinol. 2018; 6(5): 404–415.
  2. Clift AK, Kidd M, Bodei L, et al. Neuroendocrine Neoplasms of the Small Bowel and Pancreas. Neuroendocrinology. 2020; 110(6): 444–476.
  3. Niederle B, Pape UF, Costa F, et al. Vienna Consensus Conference participants. ENETS Consensus Guidelines Update for Neuroendocrine Neoplasms of the Jejunum and Ileum. Neuroendocrinology. 2016; 103(2): 125–138.
  4. Pape UF, Niederle B, Costa F, et al. Vienna Consensus Conference participants. ENETS Consensus Guidelines for Neuroendocrine Neoplasms of the Appendix (Excluding Goblet Cell Carcinomas). Neuroendocrinology. 2016; 103(2): 144–152.
  5. Ito T, Lee L, Jensen RT. Carcinoid-syndrome: recent advances, current status and controversies. Curr Opin Endocrinol Diabetes Obes. 2018; 25(1): 22–35.
  6. Bednarczuk T, Bolanowski M, Zemczak A, et al. Neuroendocrine neoplasms of the small intestine and appendix — management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017; 68(2): 223–236.
  7. Dasari A, Shen C, Halperin D, et al. Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncol. 2017; 3(10): 1335–1342.
  8. Genus TSE, Bouvier C, Wong KF, et al. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015. Br J Cancer. 2019; 121(11): 966–972.
  9. Lewkowicz E, Trofimiuk-Müldner M, Wysocka K, et al. Gastroenteropancreatic neuroendocrine neoplasms: a 10-year experience of a single center. Pol Arch Med Wewn. 2015; 125(5): 337–346.
  10. Abou Saleh M, Mansoor E, Anindo M, et al. Prevalence of Small Intestine Carcinoid Tumors: A US Population-Based Study 2012-2017. Dig Dis Sci. 2019; 64(5): 1328–1334.
  11. Xu Z, Wang Li, Dai S, et al. Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States. JAMA Netw Open. 2021; 4(9): e2124750.
  12. Levy S, van Veenendaal LM, Korse CM, et al. Survival in Patients with Neuroendocrine Tumours of the Small Intestine: Nomogram Validation and Predictors of Survival. J Clin Med. 2020; 9(8).
  13. Clift AK, Faiz O, Goldin R, et al. Predicting the survival of patients with small bowel neuroendocrine tumours: comparison of 3 systems. Endocr Connect. 2017; 6(2): 71–81.
  14. Tsilimigras DI, Hyer JM, Paredes AZ, et al. Resection of Primary Gastrointestinal Neuroendocrine Tumor Among Patients with Non-Resected Metastases Is Associated with Improved Survival: A SEER-Medicare Analysis. J Gastrointest Surg. 2021; 25(9): 2368–2376.
  15. Kaczmarska-Turek D, Pryć M, Kuś A, et al. Pitfalls in the diagnosis of carcinoid syndrome. Fam Med Prim Care Rev. 2016; 2: 109–113.
  16. Ten Cate EM, Wong LA, Groff WL, et al. Post-surgical surveillance of locally advanced ileal carcinoids found by routine ileal intubation during screening colonoscopy: a case series. J Med Case Rep. 2014; 8: 444.
  17. Halperin D, Shen C, Dasari A, et al. Frequency of carcinoid syndrome at neuroendocrine tumour diagnosis: a population-based study. The Lancet Oncology. 2017; 18(4): 525–534.
  18. Clement D, Ramage J, Srirajaskanthan R. Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome. J Oncol. 2020; 2020: 8341426.
  19. Khan MS, Walter T, Buchanan-Hughes A, et al. Differential diagnosis of diarrhoea in patients with neuroendocrine tumours: A systematic review. World J Gastroenterol. 2020; 26(30): 4537–4556.
  20. Eads JR, Reidy-Lagunes D, Soares HP, et al. from the Carcinoid Syndrome Control Collaborative. Differential Diagnosis of Diarrhea in Patients With Neuroendocrine Tumors. Pancreas. 2020; 49(9): 1123–1130.
  21. Oleinikov K, Korach A, Planer D, et al. Update in carcinoid heart disease - the heart of the matter. Rev Endocr Metab Disord. 2021; 22(3): 553–561.
  22. Bober B, Saracyn M, Kołodziej M, et al. Carcinoid Heart Disease: How to Diagnose and Treat in 2020? Clin Med Insights Cardiol. 2020; 14: 1179546820968101.
  23. Koumarianou A, Alexandraki KI, Wallin G, et al. Pathogenesis and Clinical Management of Mesenteric Fibrosis in Small Intestinal Neuroendocine Neoplasms: A Systematic Review. J Clin Med. 2020; 9(6).
  24. Michałowska IM, Kolasinska-Ćwikla A, Ćwikla J, et al. Right and left-sided carcinoid heart disease. Cardiol J. 2018; 25(3): 417.
  25. Trofimiuk-Müldner M, Lewkowicz E, Wysocka K, et al. Epidemiology of gastroenteropancreatic neuroendocrine neoplasms in Krakow and Krakow district in 2007–2011. Endokrynol Pol. 2017; 68(1): 42–46.
  26. Toumpanakis C, Fazio N, Tiensuu Janson E, et al. The ENETS 2016 Munich Advisory Board Participants, ENETS 2016 Munich Advisory Board Participants. Unmet Needs in Appendiceal Neuroendocrine Neoplasms. Neuroendocrinology. 2019; 108(1): 37–44.
  27. Sansone A, Lauretta R, Vottari S, et al. Specific and Non-Specific Biomarkers in Neuroendocrine Gastroenteropancreatic Tumors. Cancers (Basel). 2019; 11(8).
  28. Kos-Kudla B, Foltyn W, Malczewska A. Update of the diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2022; 73(3): 387–423.
  29. Mirakhur B, Pavel ME, Pommier RF, et al. Biochemical responses in symptomatic and asymptomatic patients with neuroendocrine tumors: Pooled analysis of 2 phase 3 trials. Endocr Pract. 2018 [Epub ahead of print].
  30. Gut P, Czarnywojtek A, Ruchała M. Chromogranin A assessment in patients with neuroendocrine neoplasm of the small bowel and carcinoid syndrome treated with somatostatin analogues. Adv Clin Exp Med. 2020; 29(11): 1319–1324.
  31. Ćwikła JB, Bodei L, Kolasinska-Ćwikła A, et al. Circulating Transcript Analysis (NETest) in GEP-NETs Treated With Somatostatin Analogs Defines Therapy. J Clin Endocrinol Metab. 2015; 100(11): E1437–E1445.
  32. Malczewska A, Oberg K, Kos-Kudla B. NETest is superior to chromogranin A in neuroendocrine neoplasia: a prospective ENETS CoE analysis. Endocr Connect. 2021; 10(1): 110–123.
  33. Gut P, Ruchała M. Evaluation of 5-hydroxyindoloacetic acid excretion in urine in patients with small intestine neuroendocrine neoplasm and carcinoid syndrome treated with somatostatin analogues. Neuro Endocrinol Lett. 2019; 40(7-8): 315–318.
  34. Pavel ME, Phan AT, Wolin EM, et al. CLARINET Study Investigators. Effect of Lanreotide Depot/Autogel on Urinary 5-Hydroxyindoleacetic Acid and Plasma Chromogranin A Biomarkers in Nonfunctional Metastatic Enteropancreatic Neuroendocrine Tumors. Oncologist. 2019; 24(4): 463–474.
  35. Soyer P, Aout M, Hoeffel C, et al. Helical CT-enteroclysis in the detection of small-bowel tumours: a meta-analysis. Eur Radiol. 2013; 23(2): 388–399.
  36. Bonekamp D, Raman SP, Horton KM, et al. Role of computed tomography angiography in detection and staging of small bowel carcinoid tumors. World J Radiol. 2015; 7(9): 220–235.
  37. Ganeshan D, Bhosale P, Yang T, et al. Imaging features of carcinoid tumors of the gastrointestinal tract. AJR Am J Roentgenol. 2013; 201(4): 773–786.
  38. Taylor SA, Avni F, Cronin CG, et al. The first joint ESGAR/ ESPR consensus statement on the technical performance of cross-sectional small bowel and colonic imaging. Eur Radiol. 2017; 27(6): 2570–2582.
  39. Faggian A, Fracella MR, D'Alesio G, et al. Small-Bowel Neoplasms: Role of MRI Enteroclysis. Gastroenterol Res Pract. 2016; 2016: 9686815.
  40. Masselli G, Casciani E, Polettini E, et al. Magnetic resonance imaging of small bowel neoplasms. Cancer Imaging. 2013; 13: 92–99.
  41. Masselli G, Di Tola M, Casciani E, et al. Diagnosis of Small-Bowel Diseases: Prospective Comparison of Multi-Detector Row CT Enterography with MR Enterography. Radiology. 2016; 279(2): 420–431.
  42. Barlow JM, Goss BC, Hansel SL, et al. CT enterography: technical and interpretive pitfalls. Abdom Imaging. 2015; 40(5): 1081–1096.
  43. Lebda-Wyborny T, Barczyk-Gutkowska A, Pilch-Kowalczyk J, et al. CT enterocysis and CT enterography — new approaches to assesing patology of the small intestine. Polish Surg. 2012; 14(1–2): 69–80.
  44. Chatterji M, Fidler JL, Taylor SA, et al. State of the Art MR Enterography Technique. Top Magn Reson Imaging. 2021; 30(1): 3–11.
  45. Smereczyński A, Starzyńska T, Kołaczyk K. Mesenteric changes in an ultrasound examination can facilitate the diagnosis of neuroendocrine tumors of the small intestine. J Ultrason. 2015; 15(62): 274–282.
  46. Leszczyński S, Pilch-Kowalczyk J. (eds). Imaging diagnostics. Digestive system. PZWL, Warszawa 2012.
  47. Iacopini F, Saito Y, Gotoda T, et al. Endoscopic submucosal dissection of a nonpolypoid superficial neoplasm of the terminal ileum. Endoscopy. 2016; 48 Suppl 1 UCTN: E57–E58.
  48. van Tuyl SAC, van Noorden JT, Timmer R, et al. Detection of small-bowel neuroendocrine tumors by video capsule endoscopy. Gastrointest Endosc. 2006; 64(1): 66–72.
  49. Rossi RE, Conte D, Elli L, et al. Endoscopic techniques to detect small-bowel neuroendocrine tumors: A literature review. United European Gastroenterol J. 2017; 5(1): 5–12.
  50. Yoon JiY, Kumta NA, Kim MK. The Role of Endoscopy in Small Bowel Neuroendocrine Tumors. Clin Endosc. 2021; 54(6): 818–824.
  51. Noujaim MG, Green J, Min M, et al. Carcinoids and Capsules: A Case Series Highlighting the Utility of Capsule Endoscopy in Patients With Small Bowel Carcinoids. Gastroenterology Res. 2017; 10(6): 347–351.
  52. Daskalakis K, Karakatsanis A, Stålberg P, et al. Clinical signs of fibrosis in small intestinal neuroendocrine tumours. Br J Surg. 2017; 104(1): 69–75.
  53. Enns RA, Hookey L, Armstrong D, et al. Clinical Practice Guidelines for the Use of Video Capsule Endoscopy. Gastroenterology. 2017; 152(3): 497–514.
  54. Yamamoto H, Kita H, Sunada K, et al. Clinical outcomes of double-balloon endoscopy for the diagnosis and treatment of small-intestinal diseases. Clin Gastroenterol Hepatol. 2004; 2(11): 1010–1016.
  55. Rondonotti E, Spada C, Adler S, et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Technical Review. Endoscopy. 2018; 50(4): 423–446.
  56. Ethun CG, Postlewait LM, Baptiste GG, et al. Small bowel neuroendocrine tumors: A critical analysis of diagnostic work-up and operative approach. J Surg Oncol. 2016; 114(6): 671–676.
  57. Sulbaran M, de Moura E, Bernardo W, et al. Overtube-assisted enteroscopy and capsule endoscopy for the diagnosis of small-bowel polyps and tumors: a systematic review and meta-analysis. Endosc Int Open. 2016; 4(2): E151–E163.
  58. Deprez PH, Moons LMG, OʼToole D, et al. Endoscopic management of subepithelial lesions including neuroendocrine neoplasms: European Society of Gastrointestinal Endoscopy (ESGE) Guideline. Endoscopy. 2022; 54(4): 412–429.
  59. Walsh J, Schaeffer D, Kirsch R, et al. Ileal “carcinoid” tumors — small size belies deadly intent: high rate of nodal metastasis in tumors ≤1 cm in size. Hum Pathol. 2016; 56: 123–127.
  60. Manguso N, Gangi A, Johnson J, et al. The role of pre-operative imaging and double balloon enteroscopy in the surgical management of small bowel neuroendocrine tumors: Is it necessary? J Surg Oncol. 2018; 117(2): 207–212.
  61. Ethun CG, Postlewait LM, Baptiste GG, et al. Small bowel neuroendocrine tumors: A critical analysis of diagnostic work-up and operative approach. J Surg Oncol. 2016; 114(6): 671–676.
  62. Howe JR, Cardona K, Fraker DL, et al. The Surgical Management of Small Bowel Neuroendocrine Tumors: Consensus Guidelines of the North American Neuroendocrine Tumor Society. Pancreas. 2017; 46(6): 715–731.
  63. Schreiter NF, Bartels AM, Froeling V, et al. Searching for primaries in patients with neuroendocrine tumors (NET) of unknown primary and clinically suspected NET: Evaluation of Ga-68 DOTATOC PET/CT and In-111 DTPA octreotide SPECT/CT. Radiol Oncol. 2014; 48(4): 339–347.
  64. Ambrosini V, Kunikowska J, Baudin E, et al. Consensus on molecular imaging and theranostics in neuroendocrine neoplasms. Eur J Cancer. 2021; 146: 56–73.
  65. Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J, et al. Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017; 68(2): 79–110.
  66. Severi S, Nanni O, Bodei L, et al. Role of 18FDG PET/CT in patients treated with 177Lu-DOTATATE for advanced differentiated neuroendocrine tumours. Eur J Nucl Med Mol Imaging. 2013; 40(6): 881–888.
  67. Perren A, Basturk O, Bellizzi AM. Small intestinal and ampullary neuroendocrine neoplasms. In: WHO Classification of Tumours. 5th ed. Digestive system tumours. International Agency for Research on Cancer, Lyon 2019: 131–134.
  68. Pavel M, Öberg K, Falconi M, et al. ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org. Gastroenteropancreatic neuroendocrine neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020; 31(7): 844–860.
  69. Woltering EA, Bergsland EK, Beyer DT. Neuroendocrine tumors of the jejunum and ileum. American Joint Committee on Cancer 2017. In: Amin MB. ed. AJCC Cancer Staging Manual.8th ed. Springer 2017: 375–387.
  70. Shi C, Adsay V, Bergsland EK, et al. Protocol for the examination of specimens from patients with neuroendocrine tumors (carcinoid tumors) of the jejunum and ileum. College of American Pathologists. 2020. version 1.0.0.2. https://www.cap.org/protocols-and-guidelines/cancer-reporting-tools/cancer-protocol-templates. (May, 18th, 2021).
  71. Blažević A, Hofland J, Hofland LJ, et al. Small intestinal neuroendocrine tumours and fibrosis: an entangled conundrum. Endocr Relat Cancer. 2018; 25(3): R115–R130.
  72. Chopin-Laly X, Walter T, Hervieu V, et al. Neuroendocrine neoplasms of the jejunum: a heterogeneous group with distinctive proximal and distal subsets. Virchows Arch. 2013; 462(5): 489–499.
  73. Yantiss RK, Odze RD, Farraye FA, et al. Solitary versus multiple carcinoid tumors of the ileum: a clinical and pathologic review of 68 cases. Am J Surg Pathol. 2003; 27(6): 811–817.
  74. Numbere N, Huber AR, Shi C, et al. Should Ki67 immunohistochemistry be performed on all lesions in multifocal small intestinal neuroendocrine tumours? Histopathology. 2019; 74(3): 424–429.
  75. Gangi A, Siegel E, Barmparas G, et al. Multifocality in Small Bowel Neuroendocrine Tumors. J Gastrointest Surg. 2018; 22(2): 303–309.
  76. Kim MK, Warner RRP, Roayaie S, et al. Revised staging classification improves outcome prediction for small intestinal neuroendocrine tumors. J Clin Oncol. 2013; 31(30): 3776–3781.
  77. Fata CR, Gonzalez RS, Liu E, et al. Mesenteric Tumor Deposits in Midgut Small Intestinal Neuroendocrine Tumors Are a Stronger Indicator Than Lymph Node Metastasis for Liver Metastasis and Poor Prognosis. Am J Surg Pathol. 2017; 41(1): 128–133.
  78. Wright MF, Cates J, Gonzalez RS, et al. Impact of Peritoneal Metastasis on Survival of Patients With Small Intestinal Neuroendocrine Tumor. Am J Surg Pathol. 2019; 43(4): 559–563.
  79. Klimstra DS, Kloppel G, La Ro, Rindi G. Classification of neuroendocrine neoplasms of the digestive system. In: WHO Classification of Tumours. 5th edition. Digestive system tumours. International Agency for Research on Cancer, Lyon 2019: 16–19.
  80. Bellizzi AM. Pathologic Considerations in Gastroenteropancreatic Neuroendocrine Tumors. Surg Oncol Clin N Am. 2020; 29(2): 185–208.
  81. Bellizzi AM. Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: what can brown do for you? Hum Pathol. 2020; 96: 8–33.
  82. Liu Q, Polydorides AD. Diagnosis and prognostic significance of extramural venous invasion in neuroendocrine tumors of the small intestine. Mod Pathol. 2020; 33(11): 2318–2329.
  83. Uccella S, La Rosa S, Volante M, et al. Immunohistochemical Biomarkers of Gastrointestinal, Pancreatic, Pulmonary, and Thymic Neuroendocrine Neoplasms. Endocr Pathol. 2018; 29(2): 150–168.
  84. Brierley JD, Gosporadowicz MK, Wittekind C. (eds). TNM classification of malignant tumours. 8th ed. Wiley Blackwell, Oxford 2017.
  85. Luong TV, Watkins J, Chakrabarty B, Wang LM. Standards and datasets for reporting cancers. Dataset for histopathological reporting of neuroendocrine neoplasms of the gastroenteropancreatic tract. October 2019. Royal College of Pathologists. https://www.rcpath.org/profession/guidelines/cancer-datasets-and-tissue-pathways.html (May 23th, 2021).
  86. Perren A, Couvelard A, Scoazec JY, et al. Antibes Consensus Conference participants. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Pathology: Diagnosis and Prognostic Stratification. Neuroendocrinology. 2017; 105(3): 196–200.
  87. Boudreaux JP, Klimstra DS, Hassan MM, et al. North American Neuroendocrine Tumor Society (NANETS). The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum. Pancreas. 2010; 39(6): 753–766.
  88. Strosberg JR, Halfdanarson TR, Bellizzi AM, et al. The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Medical Management of Midgut Neuroendocrine Tumors. Pancreas. 2017; 46(6): 707–714.
  89. Standardy organizacyjne oraz standardy postępowania w patomorfologii. Polskie Towarzystwo Patologów, 2020. http://pol-pat.pl/index.php/standardy-i-wytyczne-w-patomorfologii/ (May 26th, 2021).
  90. Couvelard A, Perren A, Sipos B. Appendiceal neuroendocrine neoplasms. In: WHO Classification of Tumours. 5th edition. Digestive system tumours. International Agency for Research on Cancer, Lyon 2019: 152–155.
  91. Rosenblum RC, Klein N, Paran H, et al. Appendiceal tumor incidence and an in-depth look at appendiceal neuroendocrine neoplasm in a cohort of 8,162 appendectomies: Full dataset. Data Brief. 2020; 33: 106456.
  92. Pawa N, Clift AK, Osmani H, et al. Surgical Management of Patients with Neuroendocrine Neoplasms of the Appendix: Appendectomy or More. Neuroendocrinology. 2018; 106(3): 242–251.
  93. Volante M, Daniele L, Asioli S, et al. Tumor staging but not grading is associated with adverse clinical outcome in neuroendocrine tumors of the appendix: a retrospective clinical pathologic analysis of 138 cases. Am J Surg Pathol. 2013; 37(4): 606–612.
  94. Rault-Petit B, Do Cao C, Guyétant S, et al. Current Management and Predictive Factors of Lymph Node Metastasis of Appendix Neuroendocrine Tumors: A National Study from the French Group of Endocrine Tumors (GTE). Ann Surg. 2019; 270(1): 165–171.
  95. Shi C, Adsay V, Bergsland EK, et al. Protocol for the examination of specimens from patients with neuroendocrine tumors (carcinoid tumors) of the appendix. College of American Pathologists. 2020. version 4.0.0.2. https://www.cap.org/protocols-and-guidelines/cancer-reporting-tools/cancer-protocol-templates (May 18th, 2021).
  96. Wen KW, Grenert JP, Joseph NM, et al. Genomic profile of appendiceal goblet cell carcinoid is distinct compared to appendiceal neuroendocrine tumor and conventional adenocarcinoma. Hum Pathol. 2018; 77: 166–174.
  97. Volante M, Grillo F, Massa F, et al. Neuroendocrine neoplasms of the appendix, colon and rectum. Pathologica. 2021; 113(1): 19–27.
  98. Rindi G, Klöppel G, Couvelard A, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007; 451(4): 757–762.
  99. Klöppel G, Rindi G, Perren A, et al. The ENETS and AJCC/UICC TNM classifications of the neuroendocrine tumors of the gastrointestinal tract and the pancreas: a statement. Virchows Arch. 2010; 456(6): 595–597.
  100. Galanopoulos M, McFadyen R, Drami I, et al. Challenging the Current Risk Factors of Appendiceal Neuroendocrine Neoplasms: Can They Accurately Predict Local Lymph Nodal Invasion? Results from a Large Case Series. Neuroendocrinology. 2019; 109(2): 179–186.
  101. Skancke M, Sharp SP, Maron DJ, et al. Elevated prevalence of nodal positivity in carcinoid tumours of the appendix smaller than 2 cm has a negative impact on overall survival. Colorectal Dis. 2020; 22(12): 1958–1964.
  102. Daskalakis K, Alexandraki K, Kassi E, et al. The risk of lymph node metastases and their impact on survival in patients with appendiceal neuroendocrine neoplasms: a systematic review and meta-analysis of adult and paediatric patients. Endocrine. 2020; 67(1): 20–34.
  103. Brighi N, La Rosa S, Rossi G, et al. Morphological Factors Related to Nodal Metastases in Neuroendocrine Tumors of the Appendix: A Multicentric Retrospective Study. Ann Surg. 2020; 271(3): 527–533.
  104. Howe JR, Cardona K, Fraker DL, et al. The Surgical Management of Small Bowel Neuroendocrine Tumors: Consensus Guidelines of the North American Neuroendocrine Tumor Society. Pancreas. 2017; 46(6): 715–731.
  105. Bertani E, Falconi M, Grana C, et al. Small intestinal neuroendocrine tumors with liver metastases and resection of the primary: Prognostic factors for decision making. Int J Surg. 2015; 20: 58–64.
  106. Moris D, Tsilimigras DI, Vagios S, et al. Neuroendocrine Neoplasms of the Appendix: A Review of the Literature. Anticancer Res. 2018; 38(2): 601–611.
  107. Alexandraki KI, Kaltsas GA, Grozinsky-Glasberg S, et al. Appendiceal neuroendocrine neoplasms: diagnosis and management. Endocr Relat Cancer. 2016; 23(1): R27–R41.
  108. Maxwell JE, Sherman SK, O'Dorisio TM, et al. Liver-directed surgery of neuroendocrine metastases: What is the optimal strategy? Surgery. 2016; 159(1): 320–333.
  109. Xermelo — Charakterystyka Produktu Leczniczego. https://www.ema.europa.eu/en/documents/product-information/xermelo-epar-product-information_pl.pdf.
  110. Cella D, Beaumont JL, Hudgens S, et al. Relationship Between Symptoms and Health-related Quality-of-life Benefits in Patients With Carcinoid Syndrome: Post Hoc Analyses From TELESTAR. Clin Ther. 2018; 40(12): 2006–2020.e2.
  111. Pavel M, Gross DJ, Benavent M, et al. Telotristat ethyl in carcinoid syndrome: safety and efficacy in the TELECAST phase 3 trial. Endocr Relat Cancer. 2018; 25(3): 309–322.
  112. Pobłocki J, Jasińska A, Syrenicz A, et al. The Neuroendocrine Neoplasms of the Digestive Tract: Diagnosis, Treatment and Nutrition. Nutrients. 2020; 12(5).
  113. Artale S, Barzaghi S, Grillo N, et al. Role of Diet in the Management of Carcinoid Syndrome: Clinical Recommendations for Nutrition in Patients with Neuroendocrine Tumors. Nutr Cancer. 2022; 74(1): 2–11.
  114. Del Olmo-García MI, Muros MA, López-de-la-Torre M, et al. Prevention and Management of Hormonal Crisis during Theragnosis with LU-DOTA-TATE in Neuroendocrine Tumors. A Systematic Review and Approach Proposal. J Clin Med. 2020; 9(7).
  115. Massimino K, Harrskog O, Pommier S, et al. Octreotide LAR and bolus octreotide are insufficient for preventing intraoperative complications in carcinoid patients. J Surg Oncol. 2013; 107(8): 842–846.
  116. Caplin ME, Pavel M, Phan AT, et al. CLARINET Investigators. Lanreotide autogel/depot in advanced enteropancreatic neuroendocrine tumours: final results of the CLARINET open-label extension study. Endocrine. 2021; 71(2): 502–513.
  117. Strosberg J, El-Haddad G, Wolin E, et al. NETTER-1 Trial Investigators. Phase 3 Trial of Lu-Dotatate for Midgut Neuroendocrine Tumors. N Engl J Med. 2017; 376(2): 125–135.
  118. Buscombe JR, Cwikla JB, Caplin ME, et al. Long-term efficacy of low activity meta-[131I]iodobenzylguanidine therapy in patients with disseminated neuroendocrine tumours depends on initial response. Nucl Med Commun. 2005; 26(11): 969–976.
  119. Yao J, Fazio N, Singh S, et al. Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (RADIANT-4): a randomised, placebo-controlled, phase 3 study. Lancet. 2016; 387(10022): 968–977.
  120. Fine RL, Gulati AP, Krantz BA, et al. Capecitabine and temozolomide (CAPTEM) for metastatic, well-differentiated neuroendocrine cancers: The Pancreas Center at Columbia University experience. Cancer Chemother Pharmacol. 2013; 71(3): 663–670.
  121. Berruti A, Fazio N, Ferrero A, et al. Bevacizumab plus octreotide and metronomic capecitabine in patients with metastatic well-to-moderately differentiated neuroendocrine tumors: the XELBEVOCT study. BMC Cancer. 2014; 14: 184.
  122. Koumarianou A, Kaltsas G, Kulke MH, et al. Temozolomide in Advanced Neuroendocrine Neoplasms: Pharmacological and Clinical Aspects. Neuroendocrinology. 2015; 101(4): 274–288.
  123. National Comprehensive Cancer Network. https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf.
  124. Knigge U, Capdevila J, Bartsch DK, et al. Antibes Consensus Conference Participants, Antibes Consensus Conference participants. ENETS Consensus Recommendations for the Standards of Care in Neuroendocrine Neoplasms: Follow-Up and Documentation. Neuroendocrinology. 2017; 105(3): 310–319.
  125. European Society for Medical Oncology. Standard Operating Procedures (SOPs) for Authors and templates for ESMO Clinical Practice Guidelines (CPGs) and ESMO-MCBS Scores 2021. https://www.esmo.org/content/download/77789/1426712/file/ESMO-Clinical-Practice-Guidelines-Standard-Operating-Procedures.pdf.
  126. The National Comprehensive Cancer Network. About the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) 2020. https://www.nccn.org/professionals/default.aspx.

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Via MedicaWydawcą jest  VM Media Group sp. z o.o., Grupa Via Medica, ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail:  viamedica@viamedica.pl