目的總結肺硬化性血管瘤臨床特點、診斷及外科治療的經驗。 方法回顧性分析2008年5月至2010年3月南京醫科大學第一附屬醫院胸心外科行手術治療22例肺硬化性血管瘤患者的臨床資料,其中男1例、女21例,發病年齡18~75(53.5±15.1)歲。22例均行手術治療,包括開胸手術12例,胸腔鏡或胸腔鏡輔助小切口手術10例;肺葉切除術12例,限制性肺切除術10例。 結果腫瘤大小與癥狀無顯著相關性(P=0.848),中央型與外周型癥狀發生率差異無統計學意義(P=0.35)。手術時間55~138(100.5±27.3)min,住院時間12~52(20.9±8.2)d,無手術并發癥及手術死亡。隨訪6~42(26±16)個月,患者均生存,無腫瘤復發及轉移。 結論肺硬化性血管瘤臨床及影像學檢查缺乏特征性,手術是治療肺硬化性血管瘤的有效措施。
Objective To improve the knowledge of pulmonary sclerosing hemangioma ( PSH)especially with bilateral multiple lesions of the lung. Methods The clinical data of 3 cases of PSH ( 1 case with bilateral multiple lesions in the lung) were analyzed, and the related literatures were reviewed. Results All of the 3 cases were females. A 22-year-old female patientwith bilateral multiple nodules in the lungs was complicated with thyroid multiple nodular goiter ( with hypothyroidism) , dysfunctional uterine bleeding ( with anemia) , nodular hyperplasia of the breast, and arteriovenous malformation over forearm. Thoracoscopicbiopsy of left lung and resection of the right pulmonary mass were performed and both the lesions were confirmed as PSH. The clinical manifestations of multiorgan diseases and the presence of PSH suggested Cowden syndrome in this patient. The other 2 cases aged 50 and 53 were asymptomatic with solitary pulmonary nodules identified incidentally. The accessory examinations for malignancies, infections, and autoimmune diseases showed no specific findings. Resection of the lesions were performed by thoracoscopic surgery and thoracotomy respectively, and the histopathological results proved to be PSH. Literature review showed that PSH typically occurred in middle-aged women without clinical symptoms and signs, often presenting as a pulmonary solitary nodule/mass identified incidentally. The differential diagnosis should include peripheral carcinoma, hamartoma, inflammatory pseudotumor and tuberculoma. Multiple PSH, which mainly presented as multiple well-defined nodules /masses of different size in the lungs, was rather rare, but easily confused with metastatic neoplasm. Lung biopsy by surgical operation was a common way to confirm the diagnosis, while FDP-PET and fine needle aspiration biopsy showed some defects. Surgical resection was an effective method of treatment, the residual lesions of multiple PSH should be monitored. Cowden syndrome may be considered if a PSH coexisting with abnormity of multiple organs such as thyoid, breast and vessels. Conclusions PSH should be considered during the differential diagnosis for solitary or multiple nodules /masses in the lung. Surgical biopsy is a common way to confirm the diagnosis. Local excision andnecessary follow-up are effective methods of treatment.