Johns Hopkins University, United States.
Pathol Res Pract. 2013 Aug;209(8):486-9. doi: 10.1016/j.prp.2013.05.002. Epub 2013 May 25.
Spontaneous pneumothorax (SP) affects only a subset of patients in a variety of disorders. This multi-institutional study attempted to identify unique clinicopathologic features of spontaneous pneumothorax (SP) in a large cohort. A total of 111 cases from 109 patients were retrieved from 3 institutions over an 11-year period: 27 women, 82 men. 66 were smokers, 16 were non-smokers, 27 unknown. Sixty-six cases (61%) had identifiable disorders (secondary SP). Seven patients had a family history of lung disease: 2 of pneumothorax, 2 of lung cancer, and 1 each of emphysema, COPD, and not further classified. Forty-three cases (32 men, 11 women) were primary. The average age was 28 (men) and 37 (women). 17 smoked, 14 were non-smokers, and 12 unknown. In 26%, a unique recently described entity, pneumothorax-associated fibroblastic lesion (PAFL), was noted. 16% of this subset also had cellular alveolar septae. Abundant intraalveolar macrophages were noted in 12 cases. Five cases of unknown etiology had extensive bullous lung disease not otherwise classified. One patient had unusual cellular areas suggestive of LAM but negative for HMB-45. In conclusion, several distinct morphologic lesions were identified: PAFL, a subset with cellular septae, and a third with numerous intraalveolar macrophages.
自发性气胸 (SP) 仅影响多种疾病患者中的一部分。这项多机构研究试图在大型队列中确定自发性气胸 (SP) 的独特临床病理特征。在 11 年期间，从 3 个机构共检索到 109 名患者的 111 例病例：27 名女性，82 名男性。66 名是吸烟者，16 名是非吸烟者，27 名情况未知。66 例（61%）有可识别的疾病（继发性 SP）。7 名患者有肺部疾病家族史：2 例气胸，2 例肺癌，1 例肺气肿，1 例 COPD，1 例未进一步分类。43 例（32 名男性，11 名女性）为原发性。平均年龄为 28 岁（男性）和 37 岁（女性）。17 名吸烟者，14 名非吸烟者，12 名情况未知。在 26%的病例中，发现了一种最近描述的独特实体，即气胸相关成纤维细胞病变（PAFL）。该亚组中有 16%还具有细胞性肺泡间隔。在 12 例中注意到丰富的肺泡内巨噬细胞。5 例病因不明的患者有广泛的非分类特发性大疱性肺疾病。1 例患者有不寻常的细胞区域，提示 LAM，但 HMB-45 为阴性。总之，确定了几种不同的形态病变：PAFL、具有细胞间隔的亚组，以及第三个有大量肺泡内巨噬细胞的亚组。
