局灶性Castleman病的临床特点及治疗  

Clinical features and treatment of localized Castleman＇s diaease

作　　者：刁德昌[1] 彭俊生[1] 周李[2] 杨祖立[1] 吴晓滨[1] 刘中辉[1] 王华摄[1] 汪建平[1] 

机构地区：[1]中山大学附属第六医院胃肠外科,广州510655 [2]中山大学附属第一医院小儿外科

出　　处：《中华普通外科杂志》2011年第4期309-311,共3页Chinese Journal of General Surgery

摘　　要：目的 深化对局灶性Castleman病（localized Castleman disease,LCD）的认识,提高LCD的诊治水平.方法回顾性分析26例LCD患者的临床特征及诊疗情况,总结其临床特点及治疗策略.结果 26例患者有临床症状者10例,主要表现为以局部胀痛为主的压迫症状,3例合并副肿瘤天疱疮;孤立性淋巴结肿大,最大直径1.2～15.0 cm,位于腹膜后10例,位于纵隔7例;22例行CT检查,具有局灶性钙化、坏死等特异性表现;组织病理学检查22例为透明血管型,4例为浆细胞型,均为术后病理证实.26例患者中有25例肿物获得完整切除,随访5～206个月,平均随访（48±13）个月,术后复发2例.另1例由于肿瘤深在且毗邻重要器官难于完整切除,仅行姑息切除,该例及1例术后复发病例给予联合化疗方案肿瘤消失,随访至2010年5月未见复发;另1例复发患者未行化疗,于术后11年死亡.结论 LCD主要表现为孤立性肿大淋巴结,部分患者有全身症状以及实验室检查阳性结果,掌握其影像学特点有助于术前诊断,完整切除肿物是该病最重要的治疗方式,可达到治愈的目的.Objective To improve our understanding of localized Castleman＇s disease （ Localized Castleman＇s disease, LCD） ,and to improve its diagnosis and treatment. Methods Clinical characteristics and treatment of 26 LCD cases were retrospectively analyzed, and its clinical features and treatment strategies were reviewed. Results Among the 26 cases, there were 10 cases with clinical symptoms, which mainly showed local pain induced by the compression of the tumors, and 3 in the 10 cases associated with paraneoplastic pemphigus. The swollen lymph node was at a localized area, which was mostly at retroperitoneal （10 cases） and mediastinum （7 cases）. The CT scan of LCD had its special characters including local calcification and necrosis. 22 cases were of hyaline vascular type, and the other 4 cases were of plasma type based on histopathologic examination. Twenty-five patients received complete tumor resection and 2 cases of them recurred after a follow-up of 5 to 206 months averaging at 48 ± 13 months. In one case the tumor adjoining vital organs deep in the mediastinum couldn＇t be completely resected. This patient and another with complete tumor resection recurred and received combined chemotherapy with complete tumor disappearance and were all alive without recurrence as found by follow up to May, 2010. The other patient with recurrent tumor after tumor resection didn＇t receive chemotherapy and died 11 years later. Conclusions LCD patients mainly have isolated lymphadenectasis, and some patients may have systemic symptom and show abnormal laboratory results. CT scan is helpful in establishing a diagnosis of LCD.Complete surgical resection offers a favorite result for this disease.

关 键 词：巨淋巴结增生 外科手术 体征和症状 

分 类 号：R551.2[医药卫生—血液循环系统疾病]