Gao Bei-li, Hu Jia-an, Wan Huan-ying, Chen Zhong-yuan, Wu Hua-cheng
Department of Respiratory Medicine, Affiliated Ruijin Hospital, Medical College of Shanghai Jiaotong University, Shanghai 200025, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2006 Mar;29(3):164-6.
To improve the understanding of pulmonary sclerosing hemangioma (PSH).
The clinical data of 15 cases of PSH were analyzed, and the literature was reviewed. The etiology, clinical manifestations, differential diagnosis, treatment and outcome of PSH were described.
The etiology and histological origin of PSH were unclear. Most cases were asymptomatic or only with mild symptoms. The radiology of PSH often showed isolated nodule with distinct margin in the lung field. The characteristics of its pathological manifestation were as follows: (1) background of cell gathering or mucin matrix with scattered white blood cells; (2) proliferation of hemangioma with sclerosis of vessel wall; (3) papillary proliferation of small vessels intruding into the air space; (4) existence of hemorrhage or sclerosis zone. Immunohistological studies had not defined the correct histological origin of PSH. A pre-operation diagnosis of PSH was difficult. Thirteen cases had been misdiagnosed as malignancy. The outcome of the disease was good when early surgical resection was performed.
PSH is an uncommon disease, and can be easily misdiagnosed. More attention should be paid to its clinical features and management.
提高对肺硬化性血管瘤(PSH)的认识。
分析15例PSH患者的临床资料,并复习相关文献。描述PSH的病因、临床表现、鉴别诊断、治疗及预后。
PSH的病因及组织学起源尚不清楚。多数病例无症状或仅有轻微症状。PSH的影像学表现常为肺野内边界清晰的孤立结节。其病理表现特点如下:(1)细胞聚集或黏液基质背景,有散在白细胞;(2)血管瘤样增生伴血管壁硬化;(3)小血管乳头样增生突入气腔;(4)存在出血或硬化区。免疫组织化学研究尚未明确PSH正确的组织学起源。术前诊断PSH困难。13例曾被误诊为恶性肿瘤。早期手术切除,该病预后良好。
PSH是一种罕见疾病,易被误诊。应更多关注其临床特征及处理。