Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report

Langerhans Cell Histiocytosis (LCH) is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhan’s cell. Any organ or system can be af...

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Main Authors: VISHWANATH VIJAY JOSHI, NEEMISH KAMAT, MITUSHA VERMA, ANKUR GUPTA, DEEPAK PATKAR
Format: Article
Language:English
Published: JCDR Research and Publications Pvt. Ltd. 2021-01-01
Series:International Journal of Anatomy Radiology and Surgery
Subjects:
Online Access:http://www.ijars.net/articles/PDF/2597/44569_CE[Ra1]_F(SHU)_PF1(ShG_KM)_PFA(SHU_ShG)_PN(SHU).pdf
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spelling doaj-d9bb7a2b90f14ea4944231c3ffdb75012021-07-08T04:28:26ZengJCDR Research and Publications Pvt. Ltd.International Journal of Anatomy Radiology and Surgery2277-85432455-68742021-01-01101RC01RC0410.7860/IJARS/2021/44569:2597Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case ReportVISHWANATH VIJAY JOSHI0 NEEMISH KAMAT1MITUSHA VERMA2ANKUR GUPTA3 DEEPAK PATKAR4Fellow, Department of Radiodiagnosis, Nanavati Superspeciality Hospital, Mumbai, Maharashtra, IndiaConsultant, Department of Radiodiagnosis, Nanavati Superspeciality Hospital, Mumbai, Maharashtra, IndiaConsultant, Department of Radiodiagnosis, Nanavati Superspeciality Hospital, Mumbai, Maharashtra, India.Clinical Associate, Department of Radiodiagnosis, Nanavati Superspeciality Hospital, Mumbai, Maharashtra, India.Head, Department of Radiodiagnosis, Nanavati Superspeciality Hospital, Mumbai, Maharashtra, India.Langerhans Cell Histiocytosis (LCH) is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhan’s cell. Any organ or system can be affected. Here, a case of multisystem LCH with skeletal, lung and hepatobiliary involvement in a two-year child who presented with painless forehead swelling following trauma. The patient was systematically worked up with blood investigations, imaging and histopathological analysis which ultimately revealed the diagnosis of LCH. This case report is unique in that it presented with involvement of temporal bone wherein it can be confused with inflammatory pathologies like Cholesteatoma and tumours like Rhabdomyosarcoma. The child was started on vinblastine based chemotherapy and showed good response to therapy. This case report discusses the imaging differential diagnosis in temporal bone LCH along with utility of Positron Emission Tomography and Computed Tomography (PET-CT) in planning treatment of these cases.http://www.ijars.net/articles/PDF/2597/44569_CE[Ra1]_F(SHU)_PF1(ShG_KM)_PFA(SHU_ShG)_PN(SHU).pdfhistiocytemultisystemtemporal bone
collection DOAJ
language English
format Article
sources DOAJ
author VISHWANATH VIJAY JOSHI
NEEMISH KAMAT
MITUSHA VERMA
ANKUR GUPTA
DEEPAK PATKAR
spellingShingle VISHWANATH VIJAY JOSHI
NEEMISH KAMAT
MITUSHA VERMA
ANKUR GUPTA
DEEPAK PATKAR
Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
International Journal of Anatomy Radiology and Surgery
histiocyte
multisystem
temporal bone
author_facet VISHWANATH VIJAY JOSHI
NEEMISH KAMAT
MITUSHA VERMA
ANKUR GUPTA
DEEPAK PATKAR
author_sort VISHWANATH VIJAY JOSHI
title Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
title_short Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
title_full Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
title_fullStr Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
title_full_unstemmed Langerhans Cell Histiocytosis with Temporal Bone Involvement- A Case Report
title_sort langerhans cell histiocytosis with temporal bone involvement- a case report
publisher JCDR Research and Publications Pvt. Ltd.
series International Journal of Anatomy Radiology and Surgery
issn 2277-8543
2455-6874
publishDate 2021-01-01
description Langerhans Cell Histiocytosis (LCH) is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhan’s cell. Any organ or system can be affected. Here, a case of multisystem LCH with skeletal, lung and hepatobiliary involvement in a two-year child who presented with painless forehead swelling following trauma. The patient was systematically worked up with blood investigations, imaging and histopathological analysis which ultimately revealed the diagnosis of LCH. This case report is unique in that it presented with involvement of temporal bone wherein it can be confused with inflammatory pathologies like Cholesteatoma and tumours like Rhabdomyosarcoma. The child was started on vinblastine based chemotherapy and showed good response to therapy. This case report discusses the imaging differential diagnosis in temporal bone LCH along with utility of Positron Emission Tomography and Computed Tomography (PET-CT) in planning treatment of these cases.
topic histiocyte
multisystem
temporal bone
url http://www.ijars.net/articles/PDF/2597/44569_CE[Ra1]_F(SHU)_PF1(ShG_KM)_PFA(SHU_ShG)_PN(SHU).pdf
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