Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion
Rosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. However, a subset of patients with RDD will display extranodal manifestations that are highly variable in presentation, more challenging to diagnose, and less likely...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2018/3915319 |
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| _version_ | 1832561837402488832 |
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| author | Andrew R. Orr Daniel Lefler C. Deshpande Pallavi Kumar |
| author_facet | Andrew R. Orr Daniel Lefler C. Deshpande Pallavi Kumar |
| author_sort | Andrew R. Orr |
| collection | DOAJ |
| description | Rosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. However, a subset of patients with RDD will display extranodal manifestations that are highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress compared to nodal disease. While case reports of extranodal involvement in nearly every organ system exist, documented instances of mediastinal and pulmonary artery involvement are particularly rare. This study describes the case of a middle-aged woman presenting with new onset right heart failure who was found to have extranodal RDD in the form of a large mediastinal mass with invasion and occlusion of the main pulmonary arteries. |
| format | Article |
| id | doaj-art-5317ed8bca39426c929367d0af19ff9a |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-5317ed8bca39426c929367d0af19ff9a2025-02-03T01:23:59ZengWileyCase Reports in Hematology2090-65602090-65792018-01-01201810.1155/2018/39153193915319Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery InvasionAndrew R. Orr0Daniel Lefler1C. Deshpande2Pallavi Kumar3Department of Internal Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USADepartment of Internal Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USADepartment of Pathology, Hospital of the University of Pennsylvania, Philadelphia, PA, USADepartment of Internal Medicine, Division of Hematology and Oncology, Hospital of the University of Pennsylvania, Philadelphia, PA, USARosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. However, a subset of patients with RDD will display extranodal manifestations that are highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress compared to nodal disease. While case reports of extranodal involvement in nearly every organ system exist, documented instances of mediastinal and pulmonary artery involvement are particularly rare. This study describes the case of a middle-aged woman presenting with new onset right heart failure who was found to have extranodal RDD in the form of a large mediastinal mass with invasion and occlusion of the main pulmonary arteries.http://dx.doi.org/10.1155/2018/3915319 |
| spellingShingle | Andrew R. Orr Daniel Lefler C. Deshpande Pallavi Kumar Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion Case Reports in Hematology |
| title | Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion |
| title_full | Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion |
| title_fullStr | Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion |
| title_full_unstemmed | Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion |
| title_short | Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion |
| title_sort | extranodal rosai dorfman disease presenting as a mediastinal mass with pulmonary artery invasion |
| url | http://dx.doi.org/10.1155/2018/3915319 |
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