Virchows Archiv

, Volume 448, Issue 3, pp 369–374

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

Case Report


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.


Langerhans cell sarcoma Histiocytosis S-100 protein CD1a 

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Boris Dominik Jülg
    • 1
  • Sven Weidner
    • 1
  • Doris Mayr
    • 2
  1. 1.Medizinische PoliklinikLudwig, Maximilian UniversityMunichGermany
  2. 2.Institute of PathologyLudwig Maximilian UniversityMunichGermany

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