title: Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.
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.
authors.affiliation: Medizinische Poliklinik, Ludwig, Maximilian University, Munich, Germany. boris.juelg@med.uni-muenchen.de
authors.forename: Boris Dominik
authors.initials: BD
authors.lastname: Jülg
authors.forename: Sven
authors.initials: S
authors.lastname: Weidner
authors.forename: Doris
authors.initials: D
authors.lastname: Mayr
mesh_terms: D000369:Aged, 80 and over; D018949:Antigens, CD1 / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D003713:Dendritic Cells / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D054752:Langerhans Cell Sarcoma / Q000378:metabolism / Q000473:pathology*; D008175:Lung Neoplasms / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008938:Mitosis; D009418:S100 Proteins / Q000378:metabolism
chemical_list: D018949:Antigens, CD1; D014408:Biomarkers, Tumor; C419393:CD1a antigen; D009418:S100 Proteins