Department of Respiratory and Critical Care Medicine, Xijing Hospital, Fourth Military Medical University, No. 127 West Changle Road, Xi'an, 710032, Shaanxi, China.
Department of Radiology, Xijing Hospital, Fourth Military Medical University, No. 127 West Changle Road, Xi'an, 710032, Shaanxi, China.
BMC Pulm Med. 2023 Mar 10;23(1):82. doi: 10.1186/s12890-023-02363-1.
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.
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.
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.
罗道尔夫-多夫曼病（RDD）是一种罕见的良性非朗格汉斯细胞组织细胞增生症，主要累及淋巴结和皮肤。仅发生于肺部中央气道且呈弥漫性的情况更为罕见。中央气道 RDD 在影像学和支气管镜表现上与恶性肿瘤相似。因此，很难将其与原发性气道恶性肿瘤区分开来，并且难以及时正确诊断。
本文报道了 1 例 18 岁男性原发性弥漫性中央气道 RDD 的罕见病例。尽管增强胸部 CT、正电子发射断层扫描/计算机断层扫描、增强胸部 MRI 弥散加权成像和支气管镜检查的特征提示为恶性肿瘤，但患者通过多次经支气管活检和免疫组织化学检查得到明确诊断。行 2 次经支气管镜切除术后，患者阵发性咳嗽、喘鸣和呼吸困难的症状明显减轻，气道狭窄明显改善。随访 5 个月后，患者无症状，中央气道通畅。
中央气道原发性弥漫性 RDD 的特征为气管内肿瘤，根据影像学图像和支气管镜检查，通常怀疑为恶性肿瘤。病理学和免疫组织化学检查对于明确诊断是必要的。经支气管镜切除对于中央气道原发性弥漫性 RDD 患者是有效且安全的。
