胸部局限性Castleman病的临床特征及外科治疗

目的 探讨胸部局限性Castleman病的临床特征及外科治疗方法.方法 回顾性分析1996年6月至2008年11月收治的32例胸部局限性Castleman病患者的临床资料,探讨该病的临床和病理特点,总结其外科治疗效果.32例患者中男性14例,女性18例,年龄16～48岁,平均34.2岁.其中8例患者表现为胸闷、气短;3例患者表现为刺激性干咳;2例患者表现为胸痛;14例患者无临床症状,为体检时偶尔发现.CT检查均发现纵隔或肺门单发肿瘤.结果 除1例因术中麻醉意外死亡外,其余患者全部行手术完整切除肿瘤,病理改变符合Castleman病,病理类型以透明血管型为主.5例患者伴有副肿瘤性天疱疮,其中3例出现闭塞性细支气管炎;常规激素治疗均尤效,肿瘤切除后药物治疗1～2个月皮损明显好转,但肺部病变持续加重.术后随访全部患者均未见肿瘤复发.结论 胸部局限性Castleman病是一种罕见的胸部疾病,副肿瘤性天疱疮和肺部异常为Castleman病的少见而严重的合并症,手术切除肿瘤为首选治疗.

Clinical features and surgical treatment of thoracic Castleman's disease

Objective To explore the clinical features and surgical treatment of thoracic Castleman's disease. Methods The clinical symptoms, pathological, laboratory, CT findings and results of surgery in 32 patients with Castleman's disease from June 1996 to November 2008 were evaluated. Among the 32 patients, there were 14 male and 18 female, aged from 16 to 48 years old with a mean age of 34.2 years old. Thirteen cases had symtoms including short of breath, irritable cough, or chest pain, while 14 cases had no symptoms. Mediastinal or hilar tumors were found by CT examination. Results Tumor was surgically removed in all the 32 patients except one died with anesthetic aceidenL Castleman's disease was conformed by pathology. Five cases were diagnosed as with paraneoplastie pemphigus, 3 of them were attacked by bronchiolitis obliterans. All 5 cases were failed by the use of predisone. The signs of PNP were dissolved after operation, but pulmonary lesions failed to improve. There was no recurrence in all cases.Conclusions PNP and lung abnormalities are the rare and severe complications of thoracic Castleman's disease. Surgical resection of the tumor is the first choice for treatment.