儿童Castleman病CT影像表现

CT findings of Castleman's disease in children

目的：Castleman病又称巨大淋巴结增生症，是一种介于炎症和肿瘤之间的少见的不典型淋巴组织增生性疾病，儿童病例更是罕见。总结分析儿童CD的病理及CT影像学表现，以期提高对儿童CD的cT表现的认识及诊断。方法：回顾性分析2009年-2012年临床及CT影像资料完整经手术病理证实的6例儿童CD患者。结果：6例中男4例，女2例，年龄10～12岁的5例，4岁1例，平均年龄9．7岁。局限型CD5例（83％），病理类型均为透明血管型，多中心型1例（17％），病理类型为浆细胞型。5例透明血管型中的4例CT平扫均表现为团块状均匀的软组织密度影，增强后明显均匀强化，呈类似血管样强化，可见丰富的滋养血管，未见明显钙化、坏死及囊变征象。1例发生于腹膜后的透明血管型CD表现为巨大不规则混杂密度影，伴有典型中央分支状钙化，并可见明显坏死、囊变，增强后明显不均匀强化，亦可见较多滋养血管。1例浆细胞型CD表现为多发淋巴结肿大，CT平扫密度均匀，边界清楚，增强后轻到中度强化，未见明显滋养血管及钙化、囊变等。结论：儿童CD的，临床表现多样，诊断较为复杂，CD病变均发生在淋巴组织丰富的部位。年长学龄儿童多见，CT表现与病理分型密切相关，透明血管型CD在CT增强后的类似血管样强化有一定的特征，能提供较大的诊断价值。

Objective：Castleman＇s disease （CD） ,or giant angiofollicular lymph node hyperplasia,is a rare atypical lym- phoproliferative disorder between inflammatory disorder and neoplasm, which is even rarer in children. The purpose is to an alyze the CT findings and pathology in pediatric patients,in order to improve the understanding of CT features and diagnosis accuracy of CD. Methods：The clinical and CT materials of 6 pediatric CD patients proved by surgery and pathology collected from 2009 to 2012 in Children＇s Hospital affiliated to Chongqing Medical University were retrospectively analyzed. Results： Of these 6 cases,there were 4 males and 2 females,the age ranged from 10-12y （n=5） and 4y （n= 1） with the mean age as 9.7y. Five patients （83 % ） were focal CD with the pathology as hyaline-vascular type;1 patient （17 %） was multicentric, which was plasma cell type. Of the 5 patients with hyaline-vascular type, mass-like homogeneous soft tissue density was showed on plain CT in 4 patients,and was markedly as well as homogeneously enhanced, similar to that of vascular attenuation after contrast administration. Rich feeding blood vessels were revealed, yet with no obvious calcification, necrosis or cystic change. The rest one case located within retroperitoneum showed a large irregular mass with heterogeneous density accompanied with typical central branching calcifications, necrosis and cystic change, obvious uneven enhancement and rich feeding blood vessels after contrast administration. One cases of plasma cell type CD showed multiple lymphadenopathy with homogeneous attenuation on plain CT,mild to moderate enhancement after contrast with neither obvious nourish blood vessels nor calcification, cystic change, etc. Conclusion：The clinical manifestations of CD in pediatric patients varied, usually oc cur at areas rich of lymph tissue and commonly seen in elder children of school-age. The CT manifestations are closely related to pathological subtype. Marked enhancement with the attenuation similar to that of blood vessels is the characteristic finding of hyaline-vascular type of CD,which provides important value in the diagnosis.