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Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case
Rosai-Dorfman disease (RDD) is a rare, idiopathic, benign histioproliferative disorder. Extranodal involvement is seen in around 25–40% of patients. Central nervous system manifestation of RDD is uncommon and suprasellar location of the lesion is a distinct rarity. Surgery is the cornerstone of mana...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Medknow Publications & Media Pvt Ltd
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5532945/ https://www.ncbi.nlm.nih.gov/pubmed/28761538 http://dx.doi.org/10.4103/1793-5482.209994 |
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author | Das, Sudeep Biswas, Ahitagni Roy, Soumyajit Sable, Mukund N. Singh, Daljit Jana, Manisha Sharma, Mehar Chand Julka, Pramod Kumar |
author_facet | Das, Sudeep Biswas, Ahitagni Roy, Soumyajit Sable, Mukund N. Singh, Daljit Jana, Manisha Sharma, Mehar Chand Julka, Pramod Kumar |
author_sort | Das, Sudeep |
collection | PubMed |
description | Rosai-Dorfman disease (RDD) is a rare, idiopathic, benign histioproliferative disorder. Extranodal involvement is seen in around 25–40% of patients. Central nervous system manifestation of RDD is uncommon and suprasellar location of the lesion is a distinct rarity. Surgery is the cornerstone of management of intracranial RDD. However, tumor recurrence or regrowth is a potential problem. Hence, low dose conformal radiotherapy (RT) should be considered in patients undergoing sub-total resection or having unresectable recurrent disease. Though cranial RT usually leads to satisfactory improvement of symptoms and long-term disease stabilization or regression, in few patients there may be an eventual progression of disease for which systemic chemotherapy may be considered. We have highlighted the salient features of this enigmatic disease by citing a case of a 50-year-old male patient with suprasellar RDD treated by maximal safe surgery and deferred radiation therapy on progression. |
format | Online Article Text |
id | pubmed-5532945 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-55329452017-07-31 Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case Das, Sudeep Biswas, Ahitagni Roy, Soumyajit Sable, Mukund N. Singh, Daljit Jana, Manisha Sharma, Mehar Chand Julka, Pramod Kumar Asian J Neurosurg Case Report Rosai-Dorfman disease (RDD) is a rare, idiopathic, benign histioproliferative disorder. Extranodal involvement is seen in around 25–40% of patients. Central nervous system manifestation of RDD is uncommon and suprasellar location of the lesion is a distinct rarity. Surgery is the cornerstone of management of intracranial RDD. However, tumor recurrence or regrowth is a potential problem. Hence, low dose conformal radiotherapy (RT) should be considered in patients undergoing sub-total resection or having unresectable recurrent disease. Though cranial RT usually leads to satisfactory improvement of symptoms and long-term disease stabilization or regression, in few patients there may be an eventual progression of disease for which systemic chemotherapy may be considered. We have highlighted the salient features of this enigmatic disease by citing a case of a 50-year-old male patient with suprasellar RDD treated by maximal safe surgery and deferred radiation therapy on progression. Medknow Publications & Media Pvt Ltd 2017 /pmc/articles/PMC5532945/ /pubmed/28761538 http://dx.doi.org/10.4103/1793-5482.209994 Text en Copyright: © 2016 Asian Journal of Neurosurgery http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Das, Sudeep Biswas, Ahitagni Roy, Soumyajit Sable, Mukund N. Singh, Daljit Jana, Manisha Sharma, Mehar Chand Julka, Pramod Kumar Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title | Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title_full | Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title_fullStr | Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title_full_unstemmed | Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title_short | Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case |
title_sort | recurrent intracranial rosai-dorfman disease: management of a challenging case |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5532945/ https://www.ncbi.nlm.nih.gov/pubmed/28761538 http://dx.doi.org/10.4103/1793-5482.209994 |
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