Castleman病9例报道及文献复习

Castleman Disease in 9 Cases and Literature Review

目的探讨Castleman病(CD)的临床及病理形态特征。方法对小儿CD 9例的临床资料、病理切片和随访结果进行分析。结果本组男5例,女4例.年龄(8.9±3 2)岁。局限性7例,系统性2例。局限性病例大多有局部压迫症状,发热、体质量减轻等全身表现较轻;系统性病例均有显著全身表现。肿块位于腹腔5例,纵隔2例,颈部及腋下各1例,肿块直径2.5 ～12.0 cm。镜下浆细胞(PC)型6例,透明血管(HV)型2例,混合(MV)型1例。随访结果示局限性病例预后良好,系统性预后不良。结论小儿有较长时间发热、消瘦,B超及CT等检查有肿块时应考虑CD可能。最常见发病部位为腹腔,PC型组织学类型常见。局限性病例手术切除良好,系统性病例预后不佳。

Objective To investigate the clinical, pathological features of castleman disase (CD) in children. Methods The clinical data, pathological sections and prognosis in 9 cases of CD were analysed. Results There were five boys and four girls, the average age was (8.9 ± 3.2) years. There were 7 localized type and 2 systematic type. Most of localized type patients had local pressure symptoms, and a minor systematic symptoms, such as fever and loss of weight, two systematic type patients had a remarkable systematic sysptoms. Five cases in abdomen, 2 in mediastinum, one at the cervix and the other at the axilla. The diameter of tumors were 2. 5 to 12.0 cm. Microscopically, 6 plasma cell (PC) type, 2 hyaline vascular (HV) type, the other was a mixed variant (MV) type. The follow-up indicated that the prognosis of localized type patients was excellent, while the systematic type was poor. Conclusions It should be thinking of the CD diagnosis when a child has symptoms of long period fever, weight loss, and accompany the mass by B ultrasono-graphy and CT. The abdomen is the common primary location, and PC type is predominant in children. The effect of resecting localized tumor is excellent, while the prognosis of systematic CD is poor