Respiratory tract involvement in Rosai–Dorfman disease – A report of two distinct cases

Rosai-Dorfman Disease (RDD), a rare histiocytic disorder typically characterized by painless cervical lymphadenopathy, can occasionally present extranodal presentations. Here, we report two distinct RDD cases involving the respiratory system, both treated with interventional bronchoscopy resection. The first case involved a solitary tracheal neoplasm with wild-type <i>KRAS</i> (Kirsten rat sarcoma viral oncogene homolog). This patient showed no recurrence or metastasis over a 16-month follow-up period. In contrast, the second case displayed multiple tracheal neoplasms with <i>KRAS</i> mutation. While there was no <i>in situ</i> recurrence at the resected site, the untreated tracheal lesions initially progressed at the first four months of follow-up but remained stable at the ten-month follow-up. These cases illustrate the variability and potential progression of RDD, and highlight the promising application of bronchoscopic treatment for managing respiratory involvement in RDD.

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Journal
Heliyon
Published
2025-02-01
DOI
https://doi.org/10.1016/j.heliyon.2025.e42643
Primary Topic
Histiocytic Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00

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article

Respiratory tract involvement in Rosai–Dorfman disease – A report of two distinct cases

Qing Cai, Wangjian Zha, Mei Su, Jiwang Wang et al.
Heliyon
Histiocytic Disorders and Treatments
article

Respiratory tract involvement in Rosai–Dorfman disease – A report of two distinct cases

Qing Cai, Wangjian Zha, Mei Su, Jiwang Wang, Linling Jin, Qun Zhang, Yi Zhu, Wenqiu Ding
article en

Abstract

Rosai-Dorfman Disease (RDD), a rare histiocytic disorder typically characterized by painless cervical lymphadenopathy, can occasionally present extranodal presentations. Here, we report two distinct RDD cases involving the respiratory system, both treated with interventional bronchoscopy resection. The first case involved a solitary tracheal neoplasm with wild-type <i>KRAS</i> (Kirsten rat sarcoma viral oncogene homolog). This patient showed no recurrence or metastasis over a 16-month follow-up period. In contrast, the second case displayed multiple tracheal neoplasms with <i>KRAS</i> mutation. While there was no <i>in situ</i> recurrence at the resected site, the untreated tracheal lesions initially progressed at the first four months of follow-up but remained stable at the ten-month follow-up. These cases illustrate the variability and potential progression of RDD, and highlight the promising application of bronchoscopic treatment for managing respiratory involvement in RDD.

HeliyonVol. 11(4)
Jiangsu Province Hospital (CN), Nanjing Medical University (CN)
National Natural Science Foundation of China, China Postdoctoral Science Foundation, Jiangsu Postdoctoral Research Foundation
Openalex Percentile: Top 97%
Histiocytic Disorders and Treatments
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