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Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects

Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of...

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Autores principales: Amini, Afshin, Masoumi-Moghaddam, Samar, Ehteda, Anahid, Morris, David Lawson
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4013295/
https://www.ncbi.nlm.nih.gov/pubmed/24886459
http://dx.doi.org/10.1186/1750-1172-9-71
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author Amini, Afshin
Masoumi-Moghaddam, Samar
Ehteda, Anahid
Morris, David Lawson
author_facet Amini, Afshin
Masoumi-Moghaddam, Samar
Ehteda, Anahid
Morris, David Lawson
author_sort Amini, Afshin
collection PubMed
description Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article.
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spelling pubmed-40132952014-05-09 Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects Amini, Afshin Masoumi-Moghaddam, Samar Ehteda, Anahid Morris, David Lawson Orphanet J Rare Dis Review Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article. BioMed Central 2014-05-05 /pmc/articles/PMC4013295/ /pubmed/24886459 http://dx.doi.org/10.1186/1750-1172-9-71 Text en Copyright © 2014 Amini et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Review
Amini, Afshin
Masoumi-Moghaddam, Samar
Ehteda, Anahid
Morris, David Lawson
Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title_full Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title_fullStr Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title_full_unstemmed Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title_short Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
title_sort secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4013295/
https://www.ncbi.nlm.nih.gov/pubmed/24886459
http://dx.doi.org/10.1186/1750-1172-9-71
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