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Purgina B, Jaffe R, Monaco SE, et al. Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion. Cytojournal. 2011;8:22doi: 10.4103/1742-6413.91242.
Purgina, B., Jaffe, R., Monaco, S. E., Khalbuss, W. E., Beasley, H. S., Dunn, J. A., & Pantanowitz, L. (2011). Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion. CytoJournal, 822. https://doi.org/10.4103/1742-6413.91242
Purgina, Bibianna, et al. "Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion." CytoJournal vol. 8 (2011): 22. doi: https://doi.org/10.4103/1742-6413.91242
Purgina B, Jaffe R, Monaco SE, Khalbuss WE, Beasley HS, Dunn JA, Pantanowitz L. Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion. Cytojournal. 2011;8:22. doi: 10.4103/1742-6413.91242. Epub 2011 Dec 27. PMID: 22279491; PMCID: PMC3263029.
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Cytojournal. 2011;8:22. doi: 10.4103/1742-6413.91242. Epub 2011 Dec 27.

Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion.

CytoJournal

Bibianna Purgina, Ronald Jaffe, Sara E Monaco, Walid E Khalbuss, H Scott Beasley, John A Dunn, Liron Pantanowitz

Affiliations

  1. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

PMID: 22279491 PMCID: PMC3263029 DOI: 10.4103/1742-6413.91242

Abstract

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.

Keywords: Cytology; Erdheim–Chester disease; fine needle aspiration; histiocytosis; langerhans cell histiocytosis; retropeitoneum; xanthomatous

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