Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report

Abstract Introduction Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disorder. It may be associated with autoimmune diseases, but there are few reports of concurrent ankylosing spondylitis. RDD is typically characterized by mas...

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Main Authors: Jun Xu, Tian Xia, Zhongcheng Li, Jun Zhao, Sheng Ju
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Journal of Cardiothoracic Surgery
Subjects:
Online Access:https://doi.org/10.1186/s13019-024-03147-2
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author Jun Xu
Tian Xia
Zhongcheng Li
Jun Zhao
Sheng Ju
author_facet Jun Xu
Tian Xia
Zhongcheng Li
Jun Zhao
Sheng Ju
author_sort Jun Xu
collection DOAJ
description Abstract Introduction Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disorder. It may be associated with autoimmune diseases, but there are few reports of concurrent ankylosing spondylitis. RDD is typically characterized by massive bilateral and painless cervical lymphadenopathy, which can also involve extranodal sites and constitutional symptoms, but rarely affect the chest cavity. Case presentation In this report, we present a case of a patient with a suspicious invasive anterior mediastinal lesion affecting multiple blood vessels and adjacent tissues. The patient has a history of ankylosing spondylitis. Despite extensive radiological and interventional examinations, the possibility of malignancy could not be completely ruled out. Following surgical resection of the tumor, the final pathology diagnosis suggested RDD. Conclusion Our report emphasizes the importance of considering RDD in the differential diagnosis of invasive thoracic malignancies. Thoracoscopy or mediastinoscopy biopsy can improve diagnostic accuracy. Our study contributes to an improved understanding among clinicians regarding the diagnosis and treatment of intrathoracic RDD and provides relevant evidence for future exploration of potential associations between RDD and ankylosing spondylitis.
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spelling doaj-art-a7e79bb92fbc48dbb0ecd69397109b122024-12-08T12:45:48ZengBMCJournal of Cardiothoracic Surgery1749-80902024-12-011911610.1186/s13019-024-03147-2Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case reportJun Xu0Tian Xia1Zhongcheng Li2Jun Zhao3Sheng Ju4Department of Thoracic Surgery, The First Affiliated Hospital of Soochow UniversityDepartment of Thoracic Surgery, The First Affiliated Hospital of Soochow UniversityDepartment of Thoracic Surgery, The First Affiliated Hospital of Soochow UniversityDepartment of Thoracic Surgery, The First Affiliated Hospital of Soochow UniversityDepartment of Thoracic Surgery, The First Affiliated Hospital of Soochow UniversityAbstract Introduction Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disorder. It may be associated with autoimmune diseases, but there are few reports of concurrent ankylosing spondylitis. RDD is typically characterized by massive bilateral and painless cervical lymphadenopathy, which can also involve extranodal sites and constitutional symptoms, but rarely affect the chest cavity. Case presentation In this report, we present a case of a patient with a suspicious invasive anterior mediastinal lesion affecting multiple blood vessels and adjacent tissues. The patient has a history of ankylosing spondylitis. Despite extensive radiological and interventional examinations, the possibility of malignancy could not be completely ruled out. Following surgical resection of the tumor, the final pathology diagnosis suggested RDD. Conclusion Our report emphasizes the importance of considering RDD in the differential diagnosis of invasive thoracic malignancies. Thoracoscopy or mediastinoscopy biopsy can improve diagnostic accuracy. Our study contributes to an improved understanding among clinicians regarding the diagnosis and treatment of intrathoracic RDD and provides relevant evidence for future exploration of potential associations between RDD and ankylosing spondylitis.https://doi.org/10.1186/s13019-024-03147-2Rosai-Dorfman diseaseMediastinumThymusAnkylosing spondylitis
spellingShingle Jun Xu
Tian Xia
Zhongcheng Li
Jun Zhao
Sheng Ju
Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
Journal of Cardiothoracic Surgery
Rosai-Dorfman disease
Mediastinum
Thymus
Ankylosing spondylitis
title Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
title_full Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
title_fullStr Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
title_full_unstemmed Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
title_short Extranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report
title_sort extranodal rosai dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis a case report
topic Rosai-Dorfman disease
Mediastinum
Thymus
Ankylosing spondylitis
url https://doi.org/10.1186/s13019-024-03147-2
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