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Journal article - Case Report
Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment
Case reports in surgery, v 2022, pp 1-6
30 Mar 2022
PMID: 35402057
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Introduction and Importance. Rosai-Dorfman disease (RDD) is a rare, benign type II histiocytosis characterized by the infiltration of S100+ histiocytes and emperipolesis. The disease may present in the lymph nodes (nodal RDD), in extranodal sites, or in both nodal and extranodal sites. Among those patients who present exclusively in extranodal sites, only a minority of cases present in the soft tissue. Case Presentation. An 18-year-old female presented to orthopedic oncology clinic with a chief complaint of a mass located in her lower back. The patient underwent excision of the lumbosacral mass. Pathologic review demonstrated emperipolesis of lymphocytes and plasma cells within enlarged, eosinophilic histiocytes in a background of lymphoplasmacytic infiltration and collagenous stroma. Immunohistochemical staining demonstrated S100+ and CD163+ histiocytes, consistent with diagnosis of soft tissue RDD. Clinical Discussion. Histologically, RDD is generally characterized by emperipolesis—the presence of intact lymphocytes within the histiocyte cytoplasm—and a mixed infiltrate of S100+ histiocytes, mononuclear cells, plasma cells, and lymphocytes. Although soft tissue RDD may histologically resemble nodal RDD, soft tissue RDD also demonstrates some notable histologic differences including the lack of nodal architecture, the presence of increased fibrosis and collagen deposition, and generally fewer RDD cells. Conclusion. This case presentation demonstrates one few reports of isolated soft tissue RDD within the lumbosacral region without associated lymphadenopathy or skin changes and highlights the heterogeneity that still exists in the treatment paradigm of extranodal RDD.
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Details
- Title
- Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment
- Creators
- Niteesha Betini - Drexel UniversityAlana M. Munger - Yale UniversityDouglas Rottmann - University of MichiganAndrew Haims - Yale UniversityJosé Costa - Yale UniversityDieter M. Lindskog - Yale University
- Publication Details
- Case reports in surgery, v 2022, pp 1-6
- Publisher
- Hindawi
- Number of pages
- 6
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- College of Medicine
- Web of Science ID
- WOS:000832929200001
- Other Identifier
- 991021860726904721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Surgery