Langerhans cell sarcoma (LCS) is a high grade neoplasm with overtly malignant features of Langerhans type cells that express S-100, CD1a and Langerin and show Birbeck granules by ultrastructural examination. They belong to the histiocytic and dendritic cell family which are antigen presenting cells and occur in skin, bone marrow, lymph nodes, spleen, liver, and lung. While the benign form, Langerhans cell histocytosis (LCH) is more common, the malignant form LCS is a rarity. We present a rare case of LCS in a child with lymph node involvement.

Keywords: Langerhans  cell, Malignant, Sarcoma.

1. Steven H. Swerdlow et al., WHO Classification of tumors of Haematopoietic and Lymphoid Tissues, 2017 Revised Edition.
2. Lakshmaiah KC, Smitha CS, Lokanatha D, Linu AJ, Premalata CS, Usha A and Aparna S et al. Langerhans Cell Sarcoma Review of Literature and a Rare Case Report. J MolBiomarkDiagn 2014, 5:4
3. Uchida K et al., Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J OrthopSci 2008;13: 89–93.
4. TammieFerringer et al. Langerhans cell sarcoma. Am J Dermatopathol 2006; 28:36–39
5. Howard JEF, Dwivedi RC,Masterson L,Jani P. Langerhans cell sarcoma: A systematic review. Cancer Treat Rev (2015), 41:3320-21
6. Jong- Sil Lee et al.  Langerhans Cell  Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report. j Korean med sci 2006 jun: 21 (30) 577-580
7. Bohn OL,Ruiz-Arguelles G, Navarro L, Saldivar J, Sanchez-Sosa S .  Cutaneous Langerhans cell sarcoma.A Case Report and review of literature. Int J Hematol.2007 Feb;85(2):116-20
8. Ferringer T,Banks PM,Metcalf JS. Langerhans cell sarcoma. Am. J. Dermatopathol 2006 28(1) :36-39.
9. Pileri SA,Grogan TM, Harris NL, Banks P, Campo E, Chan JK. Tumors of histiocytic and accessory dendritic cells. An immunohistochemical approach to classification from the international lymphoma study group based on 61 cases.Histopathology 2002,41:1-29.
10. Geissmann F, Dieu-Nosjean MC, Dezutlerc, et al Accumulation of immature Langerhans cells in human lymph nodes in draining chronically inflamed skin. J. Exp.Med 2002 19;196(4):417-430.
11. Murakami I,Matsushita M,Iwasaki T,etal. High viral load of Merkel cell polyomavirus DNA sequences in Langerhans cell sarcoma issues. Infect Agent cancer 2014 9:15
12. Chen W.Jaffe R.  zhang L et al( 2013) Langerhans cell sarcoma arising from Chronic lymphocytic lymphoma/Small lymphocytic leukemia. Lineage analysis and BRAF V 600E mutation study.N Am J Med Sci 2013 ;5(6):386 -391
13. Elaine Jaffe et al.  Hematopathology 1^st   edition ISBN: 978-0-7216-0040-6

Champaka G

Department of Pathology, Kidwai Cancer Institute, Bengaluru, India

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