Abstract
Langerhans cell histiocytosis (LCH) is a neoplastic histiocytic disorder characterized by a broad spectrum of clinical manifestations, ranging from single organ lesions to a multi-systemic disease. Even though it is more common during the childhood, the incidence in adults is less defined and it may be misdiagnosed due to its clinical heterogeneity [1]. Main sites involved are bones (skull, vertebral bodies), lungs (typically in adult smokers) and skin (common in children). It is also important to assess the involvement of risk organs (liver, spleen or haemopoietic system) to determinate the severity of the diseas [2]. The definitive diagnosis requires a confirmation through histological and immunochemistry examinations, and molecular tests are also recommended to identify BRAF mutations and to estimate the prognosis and decide between different treatment strategies [3].
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