| Objective: To investigate the clinical features and diagnosis and treatment of tracheobronchial sinus histiocytosis with giant lymphadenopathy(also known as Rosai-Dorfman disease,RDD),so as to improve the understanding of tracheobronchial RDD and reduce misdiagnosis.Methods: We retrospective reviewed a case of Rosai-Dorfman disease misdiagnosed with times of bronchoscopes and confirmed as RDD after lobectomy in hospital of Guangxi Medical University in April 2015.Retrieveing relevant tracheobronchial RDD literatures to analyzed its characteristics.Results: 1.Date in this case:(1)General information: male patient,36 years old;(2)Clinical manifestations: Start with cough,sputum,shortness of breath after exercise.Physical examination: the right lung breath sound disappeared.(3)Chest CT showed right hilar mass with atelectasis in the lower and middle lobes of the right lung.Fiberbronchoscope showed obstruction of the opening of right middle trunk.(4)Pathology: Gross pathology: The mass is located at the opening of the bronchus of the lower right lung lobe,almost completely blocking the middle bronchus,and the mediastinal hilar lymph nodes are enlarged.Microscopic pathology: lymph nodes: Sinus histiocytes proliferated.Mass:Many mononuclear macrophages were sheet like and small nodular hyperplasia.There were emperioplesis with active lymphocytes and red blood cells in cytoplasm.There is a lot of fibrous tissue hyperplasia around the lymph and histiocytes.Immunohistochemistry: CD138(-),CD1a(-),CD38(++),CD68(+++),CK(-),CK14(-),CK7(-),CK high(-),Napsin A(-),P63(-),S-100(+++),TTF-1(-).(5)Diagnosis and treatment process: Lung cancer and inflammatory pseudotumor were diagnosed in the external hospital.Lobectomy was performed in our hospital with diagnosis of lung cancer.Postoperative pathological diagnosis was RDD.There was no recurrence and progression after 3 months of follow-up.2.Literature review: A total of 19 cases of tracheobronchial RDD have been reported since 1978 and 201.All of them are reported individually.(1)The clinical manifestations of tracheobronchial RDD are mainly airway symptoms including cough,wheezing,dyspnea,and enlargement of lymph nodes of neck,mediastinum,and paratracheal.(2)Imaging manifested as a mass extending into the tracheobronchial cavity.PET-CT showed high uptake of the mass and enlarged lymph nodes.(3)Immunohistochemical analysis demonstrated that the histiocytes were positive for S-100 protein and CD68,negative for CD1 a.(4)14 patients(73.7%)were relieved of airway obstruction by rigid bronchoscope therapy,laser resection,and surgery.5 patients were additionally treated with hormones,and 2patients perform radiotherapy,1 patient received additional chemotherapy.5patients experienced recurrence,3 of them were stable after follow-up treatment,1 patient had an unknown prognosis,1 patient relapsed again.The rest of 9patients were in stable condition after treatment.5 patients were treated with observation,one died due to rapid progression,one showed spontaneous remission,one worsened,and his condition was stable after endoscopic treatment,the rest of two patients were stable.The patients relapsed or exacerbated were all with lymph node or multiple organ involvement.Conclusions: 1.Main manifestations of RDD are cough,dyspnea,and wheezing.The most common manifestation of chest CT is intraluminal mass,which can involve the whole layer.The incidence is low and the diagnosis is difficult.2.The diagnosis depends on the biopsy pathology and immunohistochemistry.The pathological features are characteristic histiocytes and emperipolesis.The immunohistochemistry features are positive for S100 protein,CD68 and negative for CD1 a.3.The course of RDD is benign.The treatment methods include surgery,hormones,and chemotherapy.At present,there is no unified treatment standard.Targeted drugs are the current research hotspot.4.The recurrence rate of tracheobronchial RDD is high,those who have multiple organ involvement have worse prognosis,which requires long-term follow-up and regular review. |
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