腹膜后局限性Castleman病20例临床诊治及预后分析

Surgical treatment and prognosis analysis of localized retroperitoneal Castleman disease：a study of 20 cases

目的探讨腹膜后局限性Castleman病的临床特征、诊治策略及影响预后的因素，提高伴副肿瘤性天疱疮（PNP）的Castleman病的诊治水平。方法回顾性分析1993年1月至2009年5月在北京大学第一医院普通外科接受手术治疗的20例腹膜后Castleman病患者的临床资料，并将13例腹膜后Castleman病伴PNP患者的临床资料与同期7例单纯腹膜后Castleman病患者进行比较，分析两者在发病特点、部位、实验室检查、手术策略、病理特征及外科治疗效果的不同。结果（1）本组腹膜后Castleman病多见于中青年（中位年龄36岁），发病部位多位于肾旁（14／20，70％）及髂窝（4／20，20％），左侧腹膜后多见，病理分型以透明血管型为主，腹膜后Castleman病合并PNP组的性别、年龄、肿瘤发病部位、大小及病理分型与单纯腹膜后Castleman病组无明显区别（P〉0．05）；（2）本组腹膜后Castleman病合并PNP患者较易合并闭塞性细支气管炎（8／13），有血清肿瘤标志物癌胚抗原或CA242升高现象（3／8）；（3）本组腹膜后Castleman病常有包膜，与邻近脏器边界清晰，手术较为容易，但合并PNP的腹膜后Casfleman病的生物学行为有恶性倾向及伴发子灶特征，有侵及邻近血管及术后局部复发可能；（4）Kaplan—Meier及Log—Rank生存分析显示，腹膜后Castleman病合并PNP患者5年生存率为42．8％，平均生存时间30个月，明显低于单纯腹膜后Castleman病组（P〈0．05），是否合并闭塞性细支气管炎以及肿瘤能否根治切除是影响腹膜后Castleman病患者预后的重要因素。结论腹膜后Casdeman病伴PNP具有独特的临床特征，早期诊断和切除肿瘤、及时终止致病抗体的产生，是成功治愈的关键。

Objective To investigate the clinical characteristics, surgical treatment and prognosis analysis of localized retroperitoneal Castleman disease （CD）, and to improve the level of diagnosis and treatment of retroperltoneal Castleman disease with paraneoplastic pemphigus（PNP）. Methods The clinical data of retroperitoneal CD with PNP from January 1993 to May 2009 were compared with CD without PNP retrospectively, including clinical features, tumor site, lab examination, surgical treatment, pathologic subtype and results of surgery. Results （ 1 ） Retroperitoneal Castleman disease more likely originated in para-kidney and iliae fossa with middle age of 36 years old, especially in left retroperitoneum. Of the 20 cases, 18 tumors （90%） were hyaline vascular variants and 2 were mixed variants. There was no statistical difference in gender,age,tumor site and pathological subtype between two groups. （2） Retroperitoneal CD with PNP more likely complicated with bronchiolitis obliterans （BO） and high level of serum CEA/CA242. （3） Retroperitoneal Castleman tumors had clear margin, intact envelop and were easily resectable, however the biological behavior of CD with PNP might tend malignant changing, invade adjacent blood vessel or seed locally, and eventually relapse after operation. （4）The 5-year survival rate of retroperitoneal CD with PNP was 42. 8%, significantly lower than those without PNP. The average survival time was 30 months. Bronchiolitis obliterans and radical resection were the key effect in prognosis of retroperitoneal CD. Conclusions Retroperltoneal CD with PNP has distinctive clinical features. Early diagnosis , prompt removal of tumor and termination secretion of causative antibody are critical to the management of this disease.