International Journal of Case Reports and Images, 2012

Coluna/Columna

Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single syst...

INTERNATIONAL JOURNAL OF ANATOMY RADIOLOGY AND SURGERY, 2021

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 a...

Turkish Journal of Internal Medicine, 2021

HSOA journal of cancer biology & treatment, 2017

Pediatric Radiology, 2005

Pediatric Radiology, 2004

Bangladesh Journal of Otorhinolaryngology, 2020

Langerhans cells histiocytosis (LCH) or histiocytosis-X is a rare group diseases that includes three overlapping diseases of Hand-Schuller-Christian disease (HSC), Letterer-Siwe disease (LS)and Eosinophilic granuloma(EG). It results from clonal proliferation of histiocytes having similar morphology and immunophenotype to Langerhans cells in skin and mucosa Though head and neck manifestation is common, isolated simultaneous bilateral temporal bone Langerhans Cell Histiocytosis is an extremely rare presentation. Having same otological menifestations, high resolution CT scan is advocated for early identification and differentiation of LCH from other common conditions such as mastoidits,otitis externa ,chronic suppurative otitis media. Diagnosis of LCH is confirmed by biopsy and immunohistochemical staining of S-100 protein and or CD1a antigen. Chemotherapy is main mode of treatment.The prognosis is worse when presenting age of children is younger than 2 years. Bangladesh J Otorhinolary...

Indonesian Journal of Cancer, 2019

Orphanet Journal of Rare Diseases, 2021

Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was...