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Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature

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Abstract

In the following, we describe the very rare case of Langerhans cell sarcoma (LCS) in the lung. Throughout the medical literature, only a few cases have been published, and, to the best of our knowledge, this is the first case to be reported in Germany. The patient was an 81-year-old man who showed symptoms such as chronic cough and weight loss. Clinical examination including needle biopsy indicated a high possibility of carcinoma in the right lung and in the mediastinum; however, the final histopathological diagnosis after immunohistochemistry gave evidence of LCS. LCS is a neoplastic proliferation of Langerhans cells with malignant cytological features exhibiting a very aggressive behaviour. LCS can be distinguished from other carcinomas, lymphomas and sarcomas by the typical morphological features of Langerhans cells and the immunophenotype with a consistent expression of S-100 protein and CD1a. In contrast to Langerhans cell histiocytosis, the LCS consists of Langerhans cells with high atypia and a very high mitotic rate.

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Acknowledgement

Prof. Müller-Höcker, Institute of Pathology, University of Munich, is acknowledged.

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Correspondence to Boris Dominik Jülg.

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Jülg, B.D., Weidner, S. & Mayr, D. Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature. Virchows Arch 448, 369–374 (2006). https://doi.org/10.1007/s00428-005-0115-z

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  • DOI: https://doi.org/10.1007/s00428-005-0115-z

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