局灶性Castleman病的临床特点及治疗

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...

Clinical features and treatment of localized Castleman's diaease

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.