Tumours derived from Langerhans cells (LCs) are divided into two main subgroups, according to the degree of cytological atypia and clinical aggressiveness: LC histiocytosis (LCH) and LC sarcoma (LCS). LCS is a high-grade neoplasm with obviously malignant cytologic features and the Langerhans cell phenotype, which is rare. Here the clinic-pathological of LCS will be discussed based on reported cases in the literature
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