关键词: Langerhans细胞肉瘤, 病理诊断, 临床特点, 预后

Abstract:

A 41-year-old male patient presented with masses at bilateral inguina and left waist for more than six months. The lesions were soft at the beginning, then gradually grew large, hardened and painful. Two weeks prior to the presentation, the masses ruptured. Routine blood test was normal and serum glucose level was a little higher. Since the tumors poorly responded to treatment with antibiotics, surgical excision was carried out. The masses were found to be located in subcutaneous tissue with a well-defined boundary, but synechia was severe with focal necrosis in the tumors. Pathologically, there were a large quantity of tumor cells diffusely arranged into patches in dermis and subcutaneous tissue; most of the cells were large with plenty eosinophilic cytoplasm as well as large, irregular nuclei lobulated or with concave marks. Some cells had longitudinal nuclear grooves and marked nucleoli. Also, numerous multinucleated giant cells were observed. Immunohistochemistry revealed that tumor cells were positive for S-100 protein, CD1a and vimentin, weakly positive for lysozyme, partly positive for CD68, but negative for CD20, CD3, CD30, CKpan, actin, EMA, AFP, LCA, CD21 and HMB45. A diagnosis of Langerhans cell sarcoma was made. There was no obvious response to radiotherapy combined with chemotherapy, and the patient died within one year after the confirmation of diagnosis.