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Case Reports in Pathology
Volume 2017, Article ID 9842605, 5 pages
https://doi.org/10.1155/2017/9842605
Case Report

Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion

1Department of Cellular Pathology, Plymouth Hospitals NHS Trust, Plymouth PL6 8DH, UK
2Department of Cellular Pathology, Royal Devon and Exeter NHS Foundation Trust, Old Pathology Building, Church Lane, Exeter EX2 5DW, UK
3Department of Plastic Surgery, Royal Devon and Exeter NHS Foundation Trust, Exeter EX2 5DW, UK

Correspondence should be addressed to Rasika Singh; ten.shn@hgnis.akisar

Received 21 November 2016; Accepted 7 May 2017; Published 24 May 2017

Academic Editor: Piero Tosi

Copyright © 2017 Rasika Singh et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Langerhans cells are antigen presenting dendritic cells and tumours arising from these are rare. The tumours arising from these dendritic cells are divided into two categories according to a WHO classification: Langerhans cell histiocytosis and Langerhans cell sarcoma. It is the degree of atypia and clinical aggressiveness that distinguishes the two subtypes. Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells which can occur in skin, bone marrow, lymph nodes, spleen, liver, and lung. LCS can present with multiple cutaneous and systemic lesions. We present a case of Langerhans cell sarcoma with a unique morphological appearance and variable immunohistochemical profile within a single cutaneous lesion. LCS is a rare malignancy and its diagnosis is based on morphology, immunophenotypical examination, and ultrastructural analysis by electron microscopy. Our case highlights a unique morphological description of LCS wherein the pleomorphic neoplastic cells show epidermotropism and are surrounded by a variable amount of inflammatory infiltrate within a single cutaneous lesion. A single cutaneous lesion of Langerhans cell sarcoma with variable immunohistochemical profile has not been described so far.