Spheno-Temporal Langerhans Cell Histiocytosis: A Case Report
Spheno-Temporal Langerhans Cell Histiocytosis: A Case Report作者机构：Department of Neurosurgery Mohamed V University Rabat Morocco Department of Histopathology Mohamed V University Rabat Morocco
出 版 物：《Open Journal of Modern Neurosurgery》 (现代神经外科学进展（英文）)
年 卷 期：2020年第10卷第2期
页 面：260-266页
学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学]
主 题：Langerhans Cell Histocytosis Temporal Sphenoid Exophthalmia
摘 要：Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children. The objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare our case with previously reported series. We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and also an indurated swelling of posterior face of the left shoulder on pathological fracture. A cerebro-orbital MRI performed at home showed an extra-conical process with extension at the temporal fossa. The patient, then, underwent an open-air biopsy of the temporal mass and the pathological analysis returned to favor Langerhans cell histiocytosis. The diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The child was treated by chemotherapy with a good outcome. Our results concord with literature. The prognosis is different and better in children with unifocal bone disease than those with a multifocal disease with a survival rate of 65% - 100%.
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