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

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...

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Main Authors: Rasika Singh, Charles Edward Keen, Christopher Stone, Patrick Sarsfield
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2017/9842605
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author Rasika Singh
Charles Edward Keen
Christopher Stone
Patrick Sarsfield
author_facet Rasika Singh
Charles Edward Keen
Christopher Stone
Patrick Sarsfield
author_sort Rasika Singh
collection DOAJ
description 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.
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spelling doaj-art-e9a714288ad74b17ab5cbf3be89baf912025-02-03T05:58:59ZengWileyCase Reports in Pathology2090-67812090-679X2017-01-01201710.1155/2017/98426059842605Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single LesionRasika Singh0Charles Edward Keen1Christopher Stone2Patrick Sarsfield3Department of Cellular Pathology, Plymouth Hospitals NHS Trust, Plymouth PL6 8DH, UKDepartment of Cellular Pathology, Royal Devon and Exeter NHS Foundation Trust, Old Pathology Building, Church Lane, Exeter EX2 5DW, UKDepartment of Plastic Surgery, Royal Devon and Exeter NHS Foundation Trust, Exeter EX2 5DW, UKDepartment of Cellular Pathology, Royal Devon and Exeter NHS Foundation Trust, Old Pathology Building, Church Lane, Exeter EX2 5DW, UKLangerhans 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.http://dx.doi.org/10.1155/2017/9842605
spellingShingle Rasika Singh
Charles Edward Keen
Christopher Stone
Patrick Sarsfield
Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
Case Reports in Pathology
title Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
title_full Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
title_fullStr Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
title_full_unstemmed Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
title_short Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion
title_sort langerhans cell sarcoma a case report demonstrating morphological and immunophenotypical variability within a single lesion
url http://dx.doi.org/10.1155/2017/9842605
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AT christopherstone langerhanscellsarcomaacasereportdemonstratingmorphologicalandimmunophenotypicalvariabilitywithinasinglelesion
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