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Oesophageal neuroendocrine tumours—case series of a rare malignancy

Oesophageal neuroendocrine neoplasms (NENs) are rare tumours. Neuroendocrine carcinomas (NECs) are the highest grade of NENs, with aggressive biological behaviour and poor outcomes. No standardized treatment pathways exist for these tumours, with management being individualized based on patient and...

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Autores principales: Nikolic, Amanda L, Gullifer, James, Johnson, Mary Ann, Hii, Michael W
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9769948/
https://www.ncbi.nlm.nih.gov/pubmed/36570553
http://dx.doi.org/10.1093/jscr/rjac582
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author Nikolic, Amanda L
Gullifer, James
Johnson, Mary Ann
Hii, Michael W
author_facet Nikolic, Amanda L
Gullifer, James
Johnson, Mary Ann
Hii, Michael W
author_sort Nikolic, Amanda L
collection PubMed
description Oesophageal neuroendocrine neoplasms (NENs) are rare tumours. Neuroendocrine carcinomas (NECs) are the highest grade of NENs, with aggressive biological behaviour and poor outcomes. No standardized treatment pathways exist for these tumours, with management being individualized based on patient and tumour factors. We present five cases, four men and one women between 63 and 68 years old, who were diagnosed with symptomatic primary oesophageal NECs. Three were diagnosed with localized disease, and two were diagnosed with metastatic disease. Endoscopy, biopsy and staging scan results are outlined. Two patients received neoadjuvant chemotherapy. Three patients with localized disease underwent oesophagectomy. Two of these patients received neoadjuvant chemotherapy. Four patients have succumbed to their disease, with a median survival following a diagnosis of 18 months (5–34 months). This case series highlights the variability of presentation and stage at diagnosis of oesophageal NECs. Multimodal treatment is commonly utilized; however, outcomes are universally poor. Further research is required to determine the optimal treatment regimen for oesophageal NENS.
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spelling pubmed-97699482022-12-22 Oesophageal neuroendocrine tumours—case series of a rare malignancy Nikolic, Amanda L Gullifer, James Johnson, Mary Ann Hii, Michael W J Surg Case Rep Case Series Oesophageal neuroendocrine neoplasms (NENs) are rare tumours. Neuroendocrine carcinomas (NECs) are the highest grade of NENs, with aggressive biological behaviour and poor outcomes. No standardized treatment pathways exist for these tumours, with management being individualized based on patient and tumour factors. We present five cases, four men and one women between 63 and 68 years old, who were diagnosed with symptomatic primary oesophageal NECs. Three were diagnosed with localized disease, and two were diagnosed with metastatic disease. Endoscopy, biopsy and staging scan results are outlined. Two patients received neoadjuvant chemotherapy. Three patients with localized disease underwent oesophagectomy. Two of these patients received neoadjuvant chemotherapy. Four patients have succumbed to their disease, with a median survival following a diagnosis of 18 months (5–34 months). This case series highlights the variability of presentation and stage at diagnosis of oesophageal NECs. Multimodal treatment is commonly utilized; however, outcomes are universally poor. Further research is required to determine the optimal treatment regimen for oesophageal NENS. Oxford University Press 2022-12-20 /pmc/articles/PMC9769948/ /pubmed/36570553 http://dx.doi.org/10.1093/jscr/rjac582 Text en Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2022. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Series
Nikolic, Amanda L
Gullifer, James
Johnson, Mary Ann
Hii, Michael W
Oesophageal neuroendocrine tumours—case series of a rare malignancy
title Oesophageal neuroendocrine tumours—case series of a rare malignancy
title_full Oesophageal neuroendocrine tumours—case series of a rare malignancy
title_fullStr Oesophageal neuroendocrine tumours—case series of a rare malignancy
title_full_unstemmed Oesophageal neuroendocrine tumours—case series of a rare malignancy
title_short Oesophageal neuroendocrine tumours—case series of a rare malignancy
title_sort oesophageal neuroendocrine tumours—case series of a rare malignancy
topic Case Series
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9769948/
https://www.ncbi.nlm.nih.gov/pubmed/36570553
http://dx.doi.org/10.1093/jscr/rjac582
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