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Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature

Rosai-Dorfman disease (RDD) is an extremely rare histiocytic disorder characterized by cervical lymphadenopathy, while the involvement of sellar region is less observed. Here we report a pediatric patient who was initially suspected as sellar germinoma but later identified as RDD. We also conducted...

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Autores principales: Zhang, Yi, Liu, Jie, Zhu, Jianyu, Zhou, Xiang, Zhang, Kun, Wang, Shirui, Ma, Wenbin, Pan, Hui, Wang, Renzhi, Zhu, Huijuan, Yao, Yong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7734128/
https://www.ncbi.nlm.nih.gov/pubmed/33330575
http://dx.doi.org/10.3389/fmed.2020.613756
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author Zhang, Yi
Liu, Jie
Zhu, Jianyu
Zhou, Xiang
Zhang, Kun
Wang, Shirui
Ma, Wenbin
Pan, Hui
Wang, Renzhi
Zhu, Huijuan
Yao, Yong
author_facet Zhang, Yi
Liu, Jie
Zhu, Jianyu
Zhou, Xiang
Zhang, Kun
Wang, Shirui
Ma, Wenbin
Pan, Hui
Wang, Renzhi
Zhu, Huijuan
Yao, Yong
author_sort Zhang, Yi
collection PubMed
description Rosai-Dorfman disease (RDD) is an extremely rare histiocytic disorder characterized by cervical lymphadenopathy, while the involvement of sellar region is less observed. Here we report a pediatric patient who was initially suspected as sellar germinoma but later identified as RDD. We also conducted a systematic review about RDD involving sellar region. A total of only 14 cases were included and analyzed in our study in terms of clinical presentation, endocrine abnormality, radiological features, pathology, treatment, and follow up. The most common neurological manifestations of sellar RDD is diabetes insipidus and visual changes. Two typical kinds of MRI manifestations were presented in sellar RDD; one is like meningioma-like mass lesions, another showing infiltrative pattern that demonstrates hyperintense areas on T2WI. Currently, the treatment of RDD is tailored to the individual clinical circumstances. For sellar RDD, surgical treatment can be considered to completely remove or debulk the tumor.
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spelling pubmed-77341282020-12-15 Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature Zhang, Yi Liu, Jie Zhu, Jianyu Zhou, Xiang Zhang, Kun Wang, Shirui Ma, Wenbin Pan, Hui Wang, Renzhi Zhu, Huijuan Yao, Yong Front Med (Lausanne) Medicine Rosai-Dorfman disease (RDD) is an extremely rare histiocytic disorder characterized by cervical lymphadenopathy, while the involvement of sellar region is less observed. Here we report a pediatric patient who was initially suspected as sellar germinoma but later identified as RDD. We also conducted a systematic review about RDD involving sellar region. A total of only 14 cases were included and analyzed in our study in terms of clinical presentation, endocrine abnormality, radiological features, pathology, treatment, and follow up. The most common neurological manifestations of sellar RDD is diabetes insipidus and visual changes. Two typical kinds of MRI manifestations were presented in sellar RDD; one is like meningioma-like mass lesions, another showing infiltrative pattern that demonstrates hyperintense areas on T2WI. Currently, the treatment of RDD is tailored to the individual clinical circumstances. For sellar RDD, surgical treatment can be considered to completely remove or debulk the tumor. Frontiers Media S.A. 2020-11-30 /pmc/articles/PMC7734128/ /pubmed/33330575 http://dx.doi.org/10.3389/fmed.2020.613756 Text en Copyright © 2020 Zhang, Liu, Zhu, Zhou, Zhang, Wang, Ma, Pan, Wang, Zhu and Yao. http://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
Zhang, Yi
Liu, Jie
Zhu, Jianyu
Zhou, Xiang
Zhang, Kun
Wang, Shirui
Ma, Wenbin
Pan, Hui
Wang, Renzhi
Zhu, Huijuan
Yao, Yong
Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title_full Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title_fullStr Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title_full_unstemmed Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title_short Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature
title_sort case report: rosai-dorfman disease involving sellar region in a pediatric patient: a case report and systematic review of literature
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7734128/
https://www.ncbi.nlm.nih.gov/pubmed/33330575
http://dx.doi.org/10.3389/fmed.2020.613756
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