16例Castleman病患者临床与病理分析

Clinico-pathologic Analysis of 16 Cases of Castleman

目的:探讨Castleman病临床表现、病理学特征、治疗及预后。方法:回顾分析2002年1月-2014年12月16例Castleman病患者的临床与病理资料。结果:中位年龄28.5(7-73)岁;单中心型14例,其病理类型透明血管型占92.8%(13/14);多中心型2例,病理类型均为浆细胞型。16例患者均予以手术切除,中位随访时间55.5(2-150)个月。13例单中心型术后持续缓解,1例单中心患者术后60个月复发,其病理类型为浆细胞型。结论:临床分型与病理类型影响患者预后。单中心型呈良性经过,治疗首选手术切除;多中心型可稳定或进展,进展病例缺乏有效治疗方法,预后差。

Objective:To investigate the clinico-pathologic features,treatment and prognosis of Castleman disease.Methods:The clinico-pathologic data of 16 patients diagnosed as Castleman disease from January 2002 to December2014 were analyzed retrospectively.Results:The median age was 28.5(7-73) years old.There were 14 unicentric cases,92.8%(13/14) of which was diagnosed as hyaline-vascular type.Two multicentric cases was diagnosed as plasmatcyic type.All the patients were treated by surgical resection and their median follow-up was 55.5(2-150)months.As a result,13 unicentric cases achieved sustained remission,1 unicentric case with plasmatocytic type relapsed at 60 th month after surgical resection.Conclusion:Clinical subtype and histopathogenic type are the dominating progonostic factors in Castleman patients.The clinical presentation of unicentric disease has been found to be benigns and the surgical resection can be used as first-line treatment method in clinic.The clinical presentation of multicentric disease may be stable or advanced,and the prognosis of advanced cases is poor as there are no effective treatments.