局限性巨淋巴结增生的临床特征及治疗

Clinical characteristics and surgical treatment for localized Castleman＇s disease

目的探讨局限性巨淋巴结增生的临床特征及治疗方法，以提高对该病的诊治水平。方法回顾性分析20例经手术切除的局限性巨淋巴结增生患者的临床、病理、影像学特点，并经随访评价手术治疗效果。结果患者多为中青年（平均年龄37．7岁）。肿瘤位于腹膜后9例，纵隔7例，盆腔1例，颈部1例，上臂1例，腋窝1例。5例行腔镜手术，15例行开放手术。术后病理诊断为透明血管型17例，浆细胞型1例，混合型2例。16例患者随访12～165个月，其中15例未见肿瘤复发，1例复发。结论局限性巨淋巴结增生以透明血管型为主，临床症状不典型，实验室检查大多正常，影像学检查对诊断有一定帮助，确诊需要靠病理诊断，手术彻底切除疗效好。

Objective To investigate the clinical characteristics and treatment of localized Castleman＇s disease （CD）, and review the literatures to improve the diagnosis and management of this disease. Methods The clinical symptoms, histopathology, CT, MRI findings and results of surgery in 20 patients with localized CD were evaluated retrospectively. Results The average age of the patients was 37.7 years. The lesions were located in the retroperitoneal space （9 cases）, mediastinum （7 cases）, pelvic cavity （1 case）, neck （1 case）, upper arm （1 case）, and axillary （1 case）. All patients underwent surgical resection, including 9 cases for retroperitoneal resection （6 cases had open operation and 3 cases laparoscopic resection） and 7 cases for mediastinal resection （open operation in 5 cases and thoracoscopic resection in 2 cases）. The Castleman＇s disease was confirmed by histopathology. There were hyaline vascular type of CD in 17 cases, plasma cell type of CD in 1 case, and mixed cellularity type of CD in 2 cases. The duration of follow-up ranged from 12 to 165 months for 16 cases. Among them 15 patients were alive without recurrence, and 1 case had recurrence in the primary site at 47 months after the operation. Conclusions Patients with Castleman＇s disease have no typical clinical symptoms and have normal laboratory results. The majority of patients are of hyaline vascular type of the disease. Imaging examination is helpful to diagnosis, and the final diagnosis depends on pathologic examination. Complete surgical resection of the tumor is the best treatment for localized Castleman＇s disease.