A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report

Erdheim-Chester disease (ECD) is a rare and systemic non-Langerhans cell histiocytosis. Recently, ECD was classified as an inflammatory medullary tumor that affects a diverse group of organ systems. The purpose of this report is to present the radiological features of this disease in a 51-year-old m...

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Autores principales: Zhou, Xiuzhi, Zhai, Duchang, Yang, Junlin, Shi, Dai, Lu, Kuan, Cai, Wu, Fan, Guohua, Ju, Shenghong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Oncology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8966039/
https://www.ncbi.nlm.nih.gov/pubmed/35372026
http://dx.doi.org/10.3389/fonc.2022.849578
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author Zhou, Xiuzhi
Zhai, Duchang
Yang, Junlin
Shi, Dai
Lu, Kuan
Cai, Wu
Fan, Guohua
Ju, Shenghong
author_facet Zhou, Xiuzhi
Zhai, Duchang
Yang, Junlin
Shi, Dai
Lu, Kuan
Cai, Wu
Fan, Guohua
Ju, Shenghong
author_sort Zhou, Xiuzhi
collection PubMed
description Erdheim-Chester disease (ECD) is a rare and systemic non-Langerhans cell histiocytosis. Recently, ECD was classified as an inflammatory medullary tumor that affects a diverse group of organ systems. The purpose of this report is to present the radiological features of this disease in a 51-year-old man with intestinal obstruction as the initial presentation. In this case, X-ray computed tomography (CT) and emission computed tomography (ECT) clearly showed lesions in various systems, especially in the skeletal images. The survival benefit of treatment with interferon α (IFN-α) and BRAF inhibitors is well established, while other treatments focus on symptom relief.
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spelling pubmed-89660392022-03-31 A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report Zhou, Xiuzhi Zhai, Duchang Yang, Junlin Shi, Dai Lu, Kuan Cai, Wu Fan, Guohua Ju, Shenghong Front Oncol Oncology Erdheim-Chester disease (ECD) is a rare and systemic non-Langerhans cell histiocytosis. Recently, ECD was classified as an inflammatory medullary tumor that affects a diverse group of organ systems. The purpose of this report is to present the radiological features of this disease in a 51-year-old man with intestinal obstruction as the initial presentation. In this case, X-ray computed tomography (CT) and emission computed tomography (ECT) clearly showed lesions in various systems, especially in the skeletal images. The survival benefit of treatment with interferon α (IFN-α) and BRAF inhibitors is well established, while other treatments focus on symptom relief. Frontiers Media S.A. 2022-03-16 /pmc/articles/PMC8966039/ /pubmed/35372026 http://dx.doi.org/10.3389/fonc.2022.849578 Text en Copyright © 2022 Zhou, Zhai, Yang, Shi, Lu, Cai, Fan and Ju 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 Oncology
Zhou, Xiuzhi
Zhai, Duchang
Yang, Junlin
Shi, Dai
Lu, Kuan
Cai, Wu
Fan, Guohua
Ju, Shenghong
A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title_full A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title_fullStr A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title_full_unstemmed A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title_short A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report
title_sort patient with erdheim-chester disease presenting with intestinal obstruction as the initial symptom: a case report
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8966039/
https://www.ncbi.nlm.nih.gov/pubmed/35372026
http://dx.doi.org/10.3389/fonc.2022.849578
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