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Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare disorder of an unknown etiology, characterized by a benign histiocytic proliferation in the lymph nodes, as well as the extranodal sites. Painless bilateral lymphadenopathy is the classic presentation of RDD in the majority of patients. The exteranodal disease i...

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Autores principales: NAZARI, Shiva, MOLLAEI TAVANA, Parastoo, KHALILI, Mitra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Shahid Beheshti University of Medical Sciences 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9376019/
https://www.ncbi.nlm.nih.gov/pubmed/36213157
http://dx.doi.org/10.22037/ijcn.v15i4.30629
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author NAZARI, Shiva
MOLLAEI TAVANA, Parastoo
KHALILI, Mitra
author_facet NAZARI, Shiva
MOLLAEI TAVANA, Parastoo
KHALILI, Mitra
author_sort NAZARI, Shiva
collection PubMed
description Rosai-Dorfman disease (RDD) is a rare disorder of an unknown etiology, characterized by a benign histiocytic proliferation in the lymph nodes, as well as the extranodal sites. Painless bilateral lymphadenopathy is the classic presentation of RDD in the majority of patients. The exteranodal disease involves the skin, soft tissues, bones, the genitourinary system, the lower respiratory tract, and the central nervous system. A seven-year-old boy was referred to our hospital with left parietal swelling, headache, fever, imbalance, weight loss, and speech and walking impairments. In early examinations, he showed a hyposignal infiltrative lesion in the lateral ventricle and the choroid plexus, expanding to the subcortical white matter of the bilateral temporo-occipital areas. After surgery and sampling, he was diagnosed with cerebral RDD. According to his history, he had bilateral cervical lymphadenopathy at the age of two years, femoral soft tissue involvement at the age of three, and a skin disorder that improved with local treatments at the age of five. However, at the time of referral to the hospital, there were no other symptoms in other areas, except for brain symptoms. In the differential diagnosis of brain lesions with specific borders in high-contrast radiological views, the probability of RDD should be considered, similar to meningioma. The presence of painless and extensive bilateral cervical lymphadenopathy can improve the diagnosis of this disease. Isolated brain involvement in RDD is very rare, and it can be seen in less than 5% of cases. Nevertheless, by early diagnosis and intervention, the risk of complications is reduced, and the prognosis is improved.
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spelling pubmed-93760192022-10-06 Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease NAZARI, Shiva MOLLAEI TAVANA, Parastoo KHALILI, Mitra Iran J Child Neurol Case Report Rosai-Dorfman disease (RDD) is a rare disorder of an unknown etiology, characterized by a benign histiocytic proliferation in the lymph nodes, as well as the extranodal sites. Painless bilateral lymphadenopathy is the classic presentation of RDD in the majority of patients. The exteranodal disease involves the skin, soft tissues, bones, the genitourinary system, the lower respiratory tract, and the central nervous system. A seven-year-old boy was referred to our hospital with left parietal swelling, headache, fever, imbalance, weight loss, and speech and walking impairments. In early examinations, he showed a hyposignal infiltrative lesion in the lateral ventricle and the choroid plexus, expanding to the subcortical white matter of the bilateral temporo-occipital areas. After surgery and sampling, he was diagnosed with cerebral RDD. According to his history, he had bilateral cervical lymphadenopathy at the age of two years, femoral soft tissue involvement at the age of three, and a skin disorder that improved with local treatments at the age of five. However, at the time of referral to the hospital, there were no other symptoms in other areas, except for brain symptoms. In the differential diagnosis of brain lesions with specific borders in high-contrast radiological views, the probability of RDD should be considered, similar to meningioma. The presence of painless and extensive bilateral cervical lymphadenopathy can improve the diagnosis of this disease. Isolated brain involvement in RDD is very rare, and it can be seen in less than 5% of cases. Nevertheless, by early diagnosis and intervention, the risk of complications is reduced, and the prognosis is improved. Shahid Beheshti University of Medical Sciences 2021 2021-03-01 /pmc/articles/PMC9376019/ /pubmed/36213157 http://dx.doi.org/10.22037/ijcn.v15i4.30629 Text en © 2022 The Authors. Published by Shahid Beheshti University of Medical Sciences. https://creativecommons.org/licenses/by-nc/4.0/This work is published as an open access article distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/). Non-commercial uses of the work are permitted, provided the original work is properly cited.
spellingShingle Case Report
NAZARI, Shiva
MOLLAEI TAVANA, Parastoo
KHALILI, Mitra
Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title_full Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title_fullStr Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title_full_unstemmed Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title_short Cerebral Infiltrative Lesion and Chronic Clinical Course of the Rosai-Dorfman Disease
title_sort cerebral infiltrative lesion and chronic clinical course of the rosai-dorfman disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9376019/
https://www.ncbi.nlm.nih.gov/pubmed/36213157
http://dx.doi.org/10.22037/ijcn.v15i4.30629
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