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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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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