Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterized by mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tracts. About 11% of female PJS patients are diagnosed with Gastric-type endocervical adenocarcinoma (G-EAC) and about one...

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Autores principales: Li, Xuanyan, Qi, Yue, Zhang, Wenwen, Rao, Yang, Zhang, Na, Qu, Pengpeng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Medicine
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10072263/
https://www.ncbi.nlm.nih.gov/pubmed/37025955
http://dx.doi.org/10.3389/fmed.2023.1094839
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author Li, Xuanyan
Qi, Yue
Zhang, Wenwen
Rao, Yang
Zhang, Na
Qu, Pengpeng
author_facet Li, Xuanyan
Qi, Yue
Zhang, Wenwen
Rao, Yang
Zhang, Na
Qu, Pengpeng
author_sort Li, Xuanyan
collection PubMed
description Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterized by mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tracts. About 11% of female PJS patients are diagnosed with Gastric-type endocervical adenocarcinoma (G-EAC) and about one third have a sex-cord tumor with annular tubules (SCTATs). Gastric-type endocervical adenocarcinoma is a special subtype of cervical adenocarcinoma which accounts for only 1–3%. Here we report a rare case of a 31-year-old woman affected with G-EAC and SCTAT accompanied by PJS. After surgery, we followed up for 5 years without recurrence.
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spelling pubmed-100722632023-04-05 Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report Li, Xuanyan Qi, Yue Zhang, Wenwen Rao, Yang Zhang, Na Qu, Pengpeng Front Med (Lausanne) Medicine Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterized by mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tracts. About 11% of female PJS patients are diagnosed with Gastric-type endocervical adenocarcinoma (G-EAC) and about one third have a sex-cord tumor with annular tubules (SCTATs). Gastric-type endocervical adenocarcinoma is a special subtype of cervical adenocarcinoma which accounts for only 1–3%. Here we report a rare case of a 31-year-old woman affected with G-EAC and SCTAT accompanied by PJS. After surgery, we followed up for 5 years without recurrence. Frontiers Media S.A. 2023-03-21 /pmc/articles/PMC10072263/ /pubmed/37025955 http://dx.doi.org/10.3389/fmed.2023.1094839 Text en Copyright © 2023 Li, Qi, Zhang, Rao, Zhang and Qu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Li, Xuanyan
Qi, Yue
Zhang, Wenwen
Rao, Yang
Zhang, Na
Qu, Pengpeng
Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title_full Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title_fullStr Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title_full_unstemmed Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title_short Peutz-Jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: A case report
title_sort peutz-jeghers syndrome with gastric-type mucinous endocervical adenocarcinoma and sex-cord tumor with annular tubules: a case report
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10072263/
https://www.ncbi.nlm.nih.gov/pubmed/37025955
http://dx.doi.org/10.3389/fmed.2023.1094839
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