Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review
Abstract Background Rosai-Dorfman disease (RDD) is a rare benign non-langerhans cell histiocytosis, mainly involving lymph nodes and skin. It is even rarer occurring only in central airway of lung and in diffuse form. Central airway RDD is similar to malignant tumor in imaging by radiological method...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-03-01
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| Series: | BMC Pulmonary Medicine |
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| Online Access: | https://doi.org/10.1186/s12890-023-02363-1 |
| _version_ | 1797865696120537088 |
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| author | Lei Wu Yan Zhang Xiaoya Guo Xing Tang Keke Xin Li Yang Zhe Wang Wen Jian Feng Zhao |
| author_facet | Lei Wu Yan Zhang Xiaoya Guo Xing Tang Keke Xin Li Yang Zhe Wang Wen Jian Feng Zhao |
| author_sort | Lei Wu |
| collection | DOAJ |
| description | Abstract Background Rosai-Dorfman disease (RDD) is a rare benign non-langerhans cell histiocytosis, mainly involving lymph nodes and skin. It is even rarer occurring only in central airway of lung and in diffuse form. Central airway RDD is similar to malignant tumor in imaging by radiological method and in bronchoscopy features. It is difficult to differentiate it from primary airway malignant tumor and to diagnose correctively in time. Case presentation Here we present a rare case of 18-year-old male diagnosed with primary diffuse RDD in central airway. Although the features examined by enhanced chest computed tomography, positron emission tomography/computed tomography, diffusion-weighted imaging of enhanced chest MRI and bronchoscopy indicate to be malignant tumor, the patient was definitely confirmed by multiple transbronchial biopsies and immunohistochemistry. After two transbronchial resections, the patient's symptoms of paroxysmal cough, whistle sound and shortness of breath were significantly reduced, as well as the airway stenosis was significantly improved. After 5 months of follow-up, the patient had no symptoms and the central airway were unobstructed. Conclusions Primary diffuse RDD in central airway is characterized by intratracheal neoplasm, which is usually suspected as malignant tumor according to radiological image and bronchoscopy. Pathology and immunohistochemistry are necessary for definite diagnosis. Transbronchial resection is effective and safe for patients with primary diffuse RDD in central airway. |
| first_indexed | 2024-04-09T23:12:17Z |
| format | Article |
| id | doaj.art-bba5a86b49e4418c8890c5a6dc32499d |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-04-09T23:12:17Z |
| publishDate | 2023-03-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-bba5a86b49e4418c8890c5a6dc32499d2023-03-22T10:19:15ZengBMCBMC Pulmonary Medicine1471-24662023-03-012311810.1186/s12890-023-02363-1Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature reviewLei Wu0Yan Zhang1Xiaoya Guo2Xing Tang3Keke Xin4Li Yang5Zhe Wang6Wen Jian7Feng Zhao8Department of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical UniversityDepartment of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical UniversityDepartment of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical UniversityDepartment of Radiology, Xijing Hospital, Fourth Military Medical UniversityDepartment of Nuclear Medicine, Xijing Hospital, Fourth Military Medical UniversityDepartment of Pathology, Xijing Hospital and Basic Medicine School, Fourth Military Medical UniversityDepartment of Pathology, Xijing Hospital and Basic Medicine School, Fourth Military Medical UniversityDepartment of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical UniversityDepartment of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical UniversityAbstract Background Rosai-Dorfman disease (RDD) is a rare benign non-langerhans cell histiocytosis, mainly involving lymph nodes and skin. It is even rarer occurring only in central airway of lung and in diffuse form. Central airway RDD is similar to malignant tumor in imaging by radiological method and in bronchoscopy features. It is difficult to differentiate it from primary airway malignant tumor and to diagnose correctively in time. Case presentation Here we present a rare case of 18-year-old male diagnosed with primary diffuse RDD in central airway. Although the features examined by enhanced chest computed tomography, positron emission tomography/computed tomography, diffusion-weighted imaging of enhanced chest MRI and bronchoscopy indicate to be malignant tumor, the patient was definitely confirmed by multiple transbronchial biopsies and immunohistochemistry. After two transbronchial resections, the patient's symptoms of paroxysmal cough, whistle sound and shortness of breath were significantly reduced, as well as the airway stenosis was significantly improved. After 5 months of follow-up, the patient had no symptoms and the central airway were unobstructed. Conclusions Primary diffuse RDD in central airway is characterized by intratracheal neoplasm, which is usually suspected as malignant tumor according to radiological image and bronchoscopy. Pathology and immunohistochemistry are necessary for definite diagnosis. Transbronchial resection is effective and safe for patients with primary diffuse RDD in central airway.https://doi.org/10.1186/s12890-023-02363-1Rosai-Dorfman diseaseCentral airwayTransbronchial resection |
| spellingShingle | Lei Wu Yan Zhang Xiaoya Guo Xing Tang Keke Xin Li Yang Zhe Wang Wen Jian Feng Zhao Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review BMC Pulmonary Medicine Rosai-Dorfman disease Central airway Transbronchial resection |
| title | Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review |
| title_full | Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review |
| title_fullStr | Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review |
| title_full_unstemmed | Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review |
| title_short | Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review |
| title_sort | primary diffuse rosai dorfman disease in central airway a case report and literature review |
| topic | Rosai-Dorfman disease Central airway Transbronchial resection |
| url | https://doi.org/10.1186/s12890-023-02363-1 |
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