Objective To summarize the etiology and clinical features of Mounier-Kuhn syndrome (MKS) so as to enhance the acknowledgement of MKS. Methods Three cases of MKS were reported and the keywords with " Tracheobronchomegaly”, " Mounier-Kuhn syndrome” were analyzed through the mode of literature retrieval in CNKI, VIP and Pubmed databases. Results There were 214 cases around the world including 14 cases of MKS in China. With unknown etiology and non-specific clinical feature, MKS is characteristic with congenital absence of tracheal or bronchial elastic tissue. MKS is diagnosed with remarkably dilated trachea and bronchus through chest CT or bronchofibroscope, accompanied with genetic defects. The ratio of men to women is 8.5 to 1. There is no radical cure. Symptomatic treatment and surgical treatment when necessary could be available. Conclusions MKS is a rare progressive hereditary disease, irrelevant to smoking and sharing a similar cause with generalized elastolysis, which need pay much attention to the physical examination of skin or elastic tissue. The diagnosis of MKS should be based on not merely airway diameter but also the overall clinical, pathologic, and radiologic profile.
Objective To enhance the understanding of the primary salivary glandtype lung cancer (PSGLC) and improve the diagnostic rate of the disease. Methods The clinical data of 41 patients with PSGLC pathologically confirmed in West China Hospital between October 2009 and October 2015 were analyzed in terms of clinical features, therapy and prognosis. Results All the 41 patients (21 males and 20 females) accounted for 0.22% (41/18 738) of the primary malignant lung tumor diagnosed in the same period. The patients aged from 16 to 72 with the median age of 43.6. The disease course was 1 month to 6 years, and 12 had smoking history. There were 23 cases of adenoid cystic carcinoma, 15 of mucoepidermoid carcinoma and 3 of acinic cell carcinoma. The symptoms, chest-computed tomography and fiberbronchoscopy examination had no specificity. There were 34 patients who had undergone surgery, in whom 3 had fiberbronchoscopy, 4 had oral traditional Chinese medicine treatment, 5 had chemotherapy and radiotherapy at the same time, 9 received chemotherapy only, and 4 recived radiotherapy only. Follow-up period lasted for 2-65 months, with the median time of 38 months. Two patients had recrudescence, 6 patients had distant metastasis and 1 patient died. Conclusions PSGLC have no specific clinical features; its diagnosis basically rely on pathological examination. After comprehensive treatment mainly on surgery, the prognosis of the disease is better than other malignant lung cancers.