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

• Published in: Virchows Archiv : an international journal of pathology Vol. 448; no. 3; pp. 369 - 374
• Main Authors: JÜLG, Boris Dominik, WEIDNER, Sven, MAYR, Doris
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01.03.2006; Springer Nature B.V
• Subjects: Aged, 80 and over; Antigens, CD1 - metabolism; Biological and medical sciences; Biomarkers, Tumor - metabolism; Dendritic Cells - metabolism; Dendritic Cells - pathology; Diagnosis, Differential; Hematologic and hematopoietic diseases; Histiocytosis, Langerhans-Cell - diagnosis; Humans; Investigative techniques, diagnostic techniques (general aspects); Langerhans Cell Sarcoma - metabolism; Langerhans Cell Sarcoma - pathology; Lung Neoplasms - metabolism; Lung Neoplasms - pathology; Male; Medical sciences; Mitosis; Other diseases. Hematologic involvement in other diseases; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; S100 Proteins - metabolism; Sarcoma; S 100 Protein; Sarcoma; Langerhans cell histiocytosis; Lung; Hemopathy; Malignant tumor; Review; Histiocytosis; Respiratory system; CD1a; Case study; Anatomic pathology; Langerhans cell; Antigen presenting cell; S-100 protein; Langerhans cell sarcoma; Bibliographic review
• ISSN: 0945-6317; 1432-2307
• DOI: 10.1007/s00428-005-0115-z

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.