Author:
Wu Lei,Zhang Yan,Guo Xiaoya,Tang Xing,Xin Keke,Yang Li,Wang Zhe,Jian Wen,Zhao Feng
Abstract
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.
Publisher
Springer Science and Business Media LLC
Subject
Pulmonary and Respiratory Medicine
Reference34 articles.
1. Goyal G, Young JR, Koster MJ, Tobin WO, Vassallo R, Ryu JH, Davidge-Pitts CJ, Hurtado MD, Ravindran A, Sartori Valinotti JC, et al. The mayo clinic histiocytosis working group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: erdheim-chester disease, langerhans cell histiocytosis, and Rosai-Dorfman disease. Mayo Clinic Pro. 2019;94(10):2054–71.
2. Jin F, Li Q, Li S, Wang H, Bai C, Zeng Y, Zhou R, Cai Z, Chen L, Feng J, et al. Interventional bronchoscopy for the treatment of malignant central airway stenosis: an expert recommendation for China. Respiration. 2019;97(5):484–94.
3. Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bull Soc pathol Exot Filiales. 1965;58(6):1169–75.
4. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70.
5. Histiocytosis syndromes in children. Writing group of the histiocyte society. Lancet. 1987;1(8526):208–9.
Cited by
1 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献