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A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain

INTRODUCTION: Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare disease, and much of the available literature to date has consisted of case reports. A recent systematic review revealed heterogeneity in the data as not all reports documented treatment regimens and course of disease. The recent 2...

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Autores principales: Lim, Li Xian, De Robles, Marie Shella, Winn, Robert Duncan, Hart, Kimberly Anne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264006/
https://www.ncbi.nlm.nih.gov/pubmed/32480333
http://dx.doi.org/10.1016/j.ijscr.2020.04.065
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author Lim, Li Xian
De Robles, Marie Shella
Winn, Robert Duncan
Hart, Kimberly Anne
author_facet Lim, Li Xian
De Robles, Marie Shella
Winn, Robert Duncan
Hart, Kimberly Anne
author_sort Lim, Li Xian
collection PubMed
description INTRODUCTION: Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare disease, and much of the available literature to date has consisted of case reports. A recent systematic review revealed heterogeneity in the data as not all reports documented treatment regimens and course of disease. The recent 2019 WHO update on neuroendocrine carcinoma nomenclature adds to the pre-existing classification system based on biologic activity, to better represent the spectrum of neuroendocrine non-neuroendocrine tumours (Frizziero et al., 2020). PRESENTATION OF CASE: We present a case of a patient who presented with anal pain, had a wide local excision which on histopathology revealed poorly differentiated MANEC. Despite adjuvant chemotherapy with cisplatin and etoposide as well as pelvic radiotherapy, the patient developed bi-lobar liver metastases within 9 months of initial presentation. The patient succumbed to colonic perforation 10 months after initial presentation. DISCUSSION: Most patients present with advanced disease with site-specific symptoms, and despite treatment of localised disease, many recur with distant metastasis. CONCLUSION: Although rare, this disease is highly aggressive, thus it is hoped that more clinicians can be made aware about its various clinical manifestations and disease course.
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spelling pubmed-72640062020-06-05 A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain Lim, Li Xian De Robles, Marie Shella Winn, Robert Duncan Hart, Kimberly Anne Int J Surg Case Rep Article INTRODUCTION: Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare disease, and much of the available literature to date has consisted of case reports. A recent systematic review revealed heterogeneity in the data as not all reports documented treatment regimens and course of disease. The recent 2019 WHO update on neuroendocrine carcinoma nomenclature adds to the pre-existing classification system based on biologic activity, to better represent the spectrum of neuroendocrine non-neuroendocrine tumours (Frizziero et al., 2020). PRESENTATION OF CASE: We present a case of a patient who presented with anal pain, had a wide local excision which on histopathology revealed poorly differentiated MANEC. Despite adjuvant chemotherapy with cisplatin and etoposide as well as pelvic radiotherapy, the patient developed bi-lobar liver metastases within 9 months of initial presentation. The patient succumbed to colonic perforation 10 months after initial presentation. DISCUSSION: Most patients present with advanced disease with site-specific symptoms, and despite treatment of localised disease, many recur with distant metastasis. CONCLUSION: Although rare, this disease is highly aggressive, thus it is hoped that more clinicians can be made aware about its various clinical manifestations and disease course. Elsevier 2020-05-11 /pmc/articles/PMC7264006/ /pubmed/32480333 http://dx.doi.org/10.1016/j.ijscr.2020.04.065 Text en © 2020 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Lim, Li Xian
De Robles, Marie Shella
Winn, Robert Duncan
Hart, Kimberly Anne
A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title_full A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title_fullStr A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title_full_unstemmed A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title_short A case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
title_sort case report of metastatic mixed adeno-neuroendocrine carcinoma of the anus presenting as anal pain
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264006/
https://www.ncbi.nlm.nih.gov/pubmed/32480333
http://dx.doi.org/10.1016/j.ijscr.2020.04.065
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