A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma

Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case o...

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Autores principales: Addenan, Maftuhim, May, Choo May, Hooi, Teoh Kean, Ismail, Fazliana, Kamalden, Tengku Ain
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6219334/
https://www.ncbi.nlm.nih.gov/pubmed/30505126
http://dx.doi.org/10.4103/ojo.OJO_149_2017
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author Addenan, Maftuhim
May, Choo May
Hooi, Teoh Kean
Ismail, Fazliana
Kamalden, Tengku Ain
author_facet Addenan, Maftuhim
May, Choo May
Hooi, Teoh Kean
Ismail, Fazliana
Kamalden, Tengku Ain
author_sort Addenan, Maftuhim
collection PubMed
description Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management.
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spelling pubmed-62193342018-11-30 A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma Addenan, Maftuhim May, Choo May Hooi, Teoh Kean Ismail, Fazliana Kamalden, Tengku Ain Oman J Ophthalmol Case Report Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC6219334/ /pubmed/30505126 http://dx.doi.org/10.4103/ojo.OJO_149_2017 Text en Copyright: © 2018 Oman Ophthalmic Society http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Addenan, Maftuhim
May, Choo May
Hooi, Teoh Kean
Ismail, Fazliana
Kamalden, Tengku Ain
A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title_full A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title_fullStr A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title_full_unstemmed A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title_short A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma
title_sort rare case of solitary unifocal langerhans cell histiocytosis with orbital extension: diagnostic dilemma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6219334/
https://www.ncbi.nlm.nih.gov/pubmed/30505126
http://dx.doi.org/10.4103/ojo.OJO_149_2017
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