Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment
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 p...
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Wiley
2022-01-01
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| Series: | Case Reports in Surgery |
| Online Access: | http://dx.doi.org/10.1155/2022/8440836 |
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| author | Niteesha Betini Alana M. Munger Douglas Rottmann Andrew Haims José Costa Dieter M. Lindskog |
| author_facet | Niteesha Betini Alana M. Munger Douglas Rottmann Andrew Haims José Costa Dieter M. Lindskog |
| author_sort | Niteesha Betini |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-d89c24424cd44ba7bec950b95e4baf4a |
| institution | OA Journals |
| issn | 2090-6919 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Surgery |
| spelling | doaj-art-d89c24424cd44ba7bec950b95e4baf4a2025-08-20T02:20:58ZengWileyCase Reports in Surgery2090-69192022-01-01202210.1155/2022/8440836Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical TreatmentNiteesha Betini0Alana M. Munger1Douglas Rottmann2Andrew Haims3José Costa4Dieter M. Lindskog5Drexel University College of MedicineDepartment of Orthopaedics and RehabilitationDepartment of PathologyDepartment of Radiology and Biomedical ImagingDepartment of PathologyDepartment of Orthopaedics and RehabilitationIntroduction 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.http://dx.doi.org/10.1155/2022/8440836 |
| spellingShingle | Niteesha Betini Alana M. Munger Douglas Rottmann Andrew Haims José Costa Dieter M. Lindskog Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment Case Reports in Surgery |
| title | Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment |
| title_full | Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment |
| title_fullStr | Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment |
| title_full_unstemmed | Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment |
| title_short | Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment |
| title_sort | rare presentation of rosai dorfman disease in soft tissue diagnostic findings and surgical treatment |
| url | http://dx.doi.org/10.1155/2022/8440836 |
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