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Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature
AIM: Anorectal malformation (ARM) is a rare congenital disorder of the anus and rectum. In the last 30 years virtually all patients born with ARM have survived and surgeons from adult care may be called to deal with new and long-term sequelae, including tumors of the pulled-through anorectum. Two ne...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer International Publishing
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5030204/ https://www.ncbi.nlm.nih.gov/pubmed/27722042 http://dx.doi.org/10.1186/s40064-016-3263-5 |
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author | Midrio, P. Battaglia, S. Urso, E. Castagnetti, M. Gamba, P. |
author_facet | Midrio, P. Battaglia, S. Urso, E. Castagnetti, M. Gamba, P. |
author_sort | Midrio, P. |
collection | PubMed |
description | AIM: Anorectal malformation (ARM) is a rare congenital disorder of the anus and rectum. In the last 30 years virtually all patients born with ARM have survived and surgeons from adult care may be called to deal with new and long-term sequelae, including tumors of the pulled-through anorectum. Two new cases of colorectal carcinoma in young adults born with ARM and a review of the literature is reported to emphasize the importance of a multidisciplinary follow-up. METHODS: A man and a woman, with previous history of ARM, presented at 34 years of age with symptoms of intestinal occlusion and a large pelvic mass. Both patients had no familial history of colorectal carcinoma. RESULTS: The patients underwent biopsies (mucinous rectal adenocarcinoma) and stadiation (T4N0M0). In one case the microsatellite instability showed a stable profile. Despite maximal treatments, including surgery, chemo- and radio-therapy, they both died a few years after diagnosis for progression of disease. CONCLUSION: Case studies are too limited to suggest guidelines for prevention and treatment of such complications, but the life-long follow-up is mandatory in the framework of a well-established network between pediatric and adult surgeons. The risk of tumor development in these patients should not be neglected and colleagues from adult care should be aware of the possibility this occurs in their practice. |
format | Online Article Text |
id | pubmed-5030204 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Springer International Publishing |
record_format | MEDLINE/PubMed |
spelling | pubmed-50302042016-10-09 Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature Midrio, P. Battaglia, S. Urso, E. Castagnetti, M. Gamba, P. Springerplus Review AIM: Anorectal malformation (ARM) is a rare congenital disorder of the anus and rectum. In the last 30 years virtually all patients born with ARM have survived and surgeons from adult care may be called to deal with new and long-term sequelae, including tumors of the pulled-through anorectum. Two new cases of colorectal carcinoma in young adults born with ARM and a review of the literature is reported to emphasize the importance of a multidisciplinary follow-up. METHODS: A man and a woman, with previous history of ARM, presented at 34 years of age with symptoms of intestinal occlusion and a large pelvic mass. Both patients had no familial history of colorectal carcinoma. RESULTS: The patients underwent biopsies (mucinous rectal adenocarcinoma) and stadiation (T4N0M0). In one case the microsatellite instability showed a stable profile. Despite maximal treatments, including surgery, chemo- and radio-therapy, they both died a few years after diagnosis for progression of disease. CONCLUSION: Case studies are too limited to suggest guidelines for prevention and treatment of such complications, but the life-long follow-up is mandatory in the framework of a well-established network between pediatric and adult surgeons. The risk of tumor development in these patients should not be neglected and colleagues from adult care should be aware of the possibility this occurs in their practice. Springer International Publishing 2016-09-20 /pmc/articles/PMC5030204/ /pubmed/27722042 http://dx.doi.org/10.1186/s40064-016-3263-5 Text en © The Author(s) 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Review Midrio, P. Battaglia, S. Urso, E. Castagnetti, M. Gamba, P. Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title | Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title_full | Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title_fullStr | Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title_full_unstemmed | Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title_short | Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
title_sort | rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5030204/ https://www.ncbi.nlm.nih.gov/pubmed/27722042 http://dx.doi.org/10.1186/s40064-016-3263-5 |
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