肺郎格汉斯细胞组织细胞增生症的HRCT影像表现及病理基础文献复习

HRCT Features and Pathological Basis of Pulmonary Langerhans Cell Histiocytosis

目的提高对肺郎格汉斯细胞组织细胞增生症(PLCH)的高分辨CT(HRCT)影像表现的认识。方法回顾性分析9例PLCH患者的HRCT影像资料,结合文献分析其病理基础。结果 9例患者胸部HRCT均表现为星芒状结节、"空洞"结节、厚壁囊腔、薄壁囊腔及不规则形囊腔等五种形态病灶,间隔以正常肺组织,部分结节显示为长形结构。其中6例星芒状结节多见,6例厚壁囊腔多见,8例薄壁囊腔多见,4例不规则形囊腔多见,所有病例"空洞"结节少见。4例病灶多数分布于两侧中上肺,余5例两肺病灶广泛分布;其中3例两侧中上肺囊性病灶较多,而两下肺病变多为结节病灶;1例以两肺广泛结节病灶为主;1例以两肺广泛纤维化囊性病灶为主,且部分囊壁呈"火焰"状改变。8例患者两上肺可见大量薄壁囊腔病灶,其中7例出现自发性气胸。结论 HRCT可清晰显示PLCH病灶的形态及分布,且具有特征性的影像表现。正确理解PLCH病灶的病理基础有助于提高对该病HRCT影像表现的认识。

Objective To discuss the HRCT features of Pulmonary Langerhans Cell Histiocytosis （PLCH）. Methods Clinical and HRCT data of nine patients with PLCH were reviewed retrospectively. The correlation between the HRCT findings and pathological results were analyzed based on related literature. Results Chest HRCT of all patients showed the stellate shaped nodules, cavitary nodules, thick wall cysts, thin wall cysts and irregular shaped cysts. Some nodules showed elongated structure. Stellate shaped nodules were common in six cases, and thick wall cysts in six cases, thin wall cysts in eight cases, irregular shaped cysts in four cases. Cavitary nodules were few in all patients. Most lesions were seen in upper and middle lung zones in four cases. Cysts were predominantly distributed in the upper and middle lung zones while nodules in lung bases in three cases. One case demonstrated nodular pattern and numerous nodules were widely dis- tributed throughout lung. One case demonstrated numerous fibrotic lesion that widely distributed in both lungs, and some cysts wall were irregular and flame like. In eight cases, numerous thin wall cysts could be seen in upper and middle lung zones, and among them, there were seven cases suffering from spontaneous pneumothorax. Conclusion HRCT is useful to demonstrate the morphology and distribution of lung abnormalities in patients with PLCH. The HRCT appearances of PLCH are characteristic. Correct understanding of PLCH lesions on pathological basis is helpful to improve awareness of HRCT performance.