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Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion
Erdheim–Chester disease (ECD) is a rare, multisystem disorder of macrophages. Patients manifest with histiocytic infiltrates that lead to xanthogranulomatous lesions in multiple organ systems. The cytologic features of this disorder are not well characterized. As a result, the cytologic diagnosis of...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Medknow Publications & Media Pvt Ltd
2011
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3263029/ https://www.ncbi.nlm.nih.gov/pubmed/22279491 http://dx.doi.org/10.4103/1742-6413.91242 |
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author | Purgina, Bibianna Jaffe, Ronald Monaco, Sara E. Khalbuss, Walid E. Beasley, H. Scott Dunn, John A. Pantanowitz, Liron |
author_facet | Purgina, Bibianna Jaffe, Ronald Monaco, Sara E. Khalbuss, Walid E. Beasley, H. Scott Dunn, John A. Pantanowitz, Liron |
author_sort | Purgina, Bibianna |
collection | PubMed |
description | Erdheim–Chester disease (ECD) is a rare, multisystem disorder of macrophages. Patients manifest with histiocytic infiltrates that lead to xanthogranulomatous lesions in multiple organ systems. The cytologic features of this disorder are not well characterized. As a result, the cytologic diagnosis of ECD can be very challenging. The aim of this report is to describe the cytomorphology of ECD in a patient presenting with a retroperitoneal soft tissue lesion. A 54-year-old woman with proptosis and diabetes insipidus was found on imaging studies to have multiple intracranial lesions, sclerosis of both femurs and a retroperitoneal soft tissue mass. Fine needle aspiration (FNA) and a concomitant core biopsy of this abnormal retroperitoneal soft tissue revealed foamy, epithelioid and multinucleated histiocytes associated with fibrosis. The histiocytes were immunoreactive for CD68, CD163, Factor XIIIa and fascin, and negative for S100, confirming the diagnosis of ECD. ECD requires a morphologic diagnosis that fits with the appropriate clinical context. This case describes the cytomorphologic features of ECD and highlights the role of cytology in helping reach a diagnosis of this rare disorder. |
format | Online Article Text |
id | pubmed-3263029 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-32630292012-01-25 Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion Purgina, Bibianna Jaffe, Ronald Monaco, Sara E. Khalbuss, Walid E. Beasley, H. Scott Dunn, John A. Pantanowitz, Liron Cytojournal Case Report Erdheim–Chester disease (ECD) is a rare, multisystem disorder of macrophages. Patients manifest with histiocytic infiltrates that lead to xanthogranulomatous lesions in multiple organ systems. The cytologic features of this disorder are not well characterized. As a result, the cytologic diagnosis of ECD can be very challenging. The aim of this report is to describe the cytomorphology of ECD in a patient presenting with a retroperitoneal soft tissue lesion. A 54-year-old woman with proptosis and diabetes insipidus was found on imaging studies to have multiple intracranial lesions, sclerosis of both femurs and a retroperitoneal soft tissue mass. Fine needle aspiration (FNA) and a concomitant core biopsy of this abnormal retroperitoneal soft tissue revealed foamy, epithelioid and multinucleated histiocytes associated with fibrosis. The histiocytes were immunoreactive for CD68, CD163, Factor XIIIa and fascin, and negative for S100, confirming the diagnosis of ECD. ECD requires a morphologic diagnosis that fits with the appropriate clinical context. This case describes the cytomorphologic features of ECD and highlights the role of cytology in helping reach a diagnosis of this rare disorder. Medknow Publications & Media Pvt Ltd 2011-12-27 /pmc/articles/PMC3263029/ /pubmed/22279491 http://dx.doi.org/10.4103/1742-6413.91242 Text en © 2011 Purgina, et al.; licensee Cytopathology Foundation Inc. http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Purgina, Bibianna Jaffe, Ronald Monaco, Sara E. Khalbuss, Walid E. Beasley, H. Scott Dunn, John A. Pantanowitz, Liron Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title | Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title_full | Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title_fullStr | Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title_full_unstemmed | Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title_short | Cytomorphology of Erdheim–Chester disease presenting as a retroperitoneal soft tissue lesion |
title_sort | cytomorphology of erdheim–chester disease presenting as a retroperitoneal soft tissue lesion |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3263029/ https://www.ncbi.nlm.nih.gov/pubmed/22279491 http://dx.doi.org/10.4103/1742-6413.91242 |
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