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...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Pvt. Ltd.
2021-01-01
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| 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 |
| Summary: | 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. |
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| ISSN: | 2277-8543 2455-6874 |