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Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms....
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787619/ https://www.ncbi.nlm.nih.gov/pubmed/24151559 http://dx.doi.org/10.1155/2013/315109 |
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author | Chippagiri, Prashanthi Banavar Ravi, Spoorthi Patwa, Neha |
author_facet | Chippagiri, Prashanthi Banavar Ravi, Spoorthi Patwa, Neha |
author_sort | Chippagiri, Prashanthi |
collection | PubMed |
description | Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here, we report a case of Cowdens syndrome of a 30-year-old female patient who came with a complaint of multiple growths in the oral cavity of a three-month duration. On examination, multiple skin-colored, flat-topped papules over her forehead and right malar bone and multiple papillomatous papules involving all the mucosal surfaces intraorally were observed. This syndrome is associated with the development of several types of malignancies, especially breast carcinoma and thyroid carcinoma, which is why early recognition and regular and vigilant surveillance of individuals with the syndrome are important. |
format | Online Article Text |
id | pubmed-3787619 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-37876192013-10-22 Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations Chippagiri, Prashanthi Banavar Ravi, Spoorthi Patwa, Neha Case Rep Dent Case Report Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here, we report a case of Cowdens syndrome of a 30-year-old female patient who came with a complaint of multiple growths in the oral cavity of a three-month duration. On examination, multiple skin-colored, flat-topped papules over her forehead and right malar bone and multiple papillomatous papules involving all the mucosal surfaces intraorally were observed. This syndrome is associated with the development of several types of malignancies, especially breast carcinoma and thyroid carcinoma, which is why early recognition and regular and vigilant surveillance of individuals with the syndrome are important. Hindawi Publishing Corporation 2013 2013-09-16 /pmc/articles/PMC3787619/ /pubmed/24151559 http://dx.doi.org/10.1155/2013/315109 Text en Copyright © 2013 Prashanthi Chippagiri et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Chippagiri, Prashanthi Banavar Ravi, Spoorthi Patwa, Neha Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title | Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title_full | Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title_fullStr | Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title_full_unstemmed | Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title_short | Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations |
title_sort | multiple hamartoma syndrome with characteristic oral and cutaneous manifestations |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787619/ https://www.ncbi.nlm.nih.gov/pubmed/24151559 http://dx.doi.org/10.1155/2013/315109 |
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