The clinicopathologic features of unicentric Castleman disease in the children's neck
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摘要: 目的 探讨儿童颈部单中心型Castleman病的临床特点、影像学表现、病理表型、治疗和预后，以期提高耳鼻咽喉头颈外科医师对Castleman病的认识。方法 回顾性横断面分析2015年7月-2020年6月在昆明市儿童医院经病理诊断为Castleman病、肿物在颈部的6例患儿临床资料，总结其影像学和病理特征，探讨其发生机制。结果 6例Castleman病患儿中，男5例，女1例。组织病理学：透明血管型5例，混合型1例。透明血管型中观察到一致的病理特征是：萎缩的生发中心伴淋巴细胞削减、增生的套细胞呈同心圆状排列、大量的血管增生，且在滤泡间区高内皮小静脉的增生占据主导地位。2例患儿表现出生发中心双胞胎/花椰菜形多胞胎的同时，观察到“棒棒糖”外观的形成。所有患儿均接受了病灶完整手术切除治疗，随访中位时间48(26，84)个月，预后良好。结论 儿童颈部单中心型Castleman病大多数延迟确诊，多表现为无痛性淋巴结肿大，病理类型以透明血管型为主，外科手术治疗整体预后良好。
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关键词:
- Castleman病 /
- 头颈部肿瘤 /
- 儿童
Abstract: Objective To investigate the clinical features, imaging findings, pathological phenotype, treatment and prognosis of unicentric Castleman disease in the children's neck, in order to improve the understanding of CD among Otolaryngology Head and Neck Surgery.Methods Retrospective cross-sectional, observational study was undertaken in Kunming Children's Hospital, from the archival data between January July 2015 and June 2020. Only 6 cases of CD were identified after studying the histomorphological characteristics and neck mass diagnosed. The imaging and pathological features were summarized and the pathogenesis was discussed.Results Among the 6 cases of Castleman disease, five were male and one was female. Histopathology: Five cases were hyaline vascular subtype, one was mixed type. The uniform clinicopathologic features seen in all hyaline vascular subtype of CD included atrophic germinal centre with lymphocyte depletion, concentric rings of small lymphocytes, increased vascularity and predominance of high endothelial vessels in interfollicular region. Twinning, in which two or more germinal centers are combined and surrounded by lymphocytes in the mantle zone was observed in two cases with lollipop pattern at the same time. All the cases underwent complete surgical resection, the median follow-up time was 48 months(26, 84), both of them had good prognosis.Conclusion Most cases of unicentric type CD in children are diagnosed late, which is clinical showed by painless lymphadenopathy. The most common pathological type is hyaline vascular. The overall prognosis of surgical treatment was good.-
Key words:
- Castleman disease /
- head and neck tumors /
- children
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表 1 6例UCD患儿的临床资料
编号 性别 年龄 病史/月 肿块部位 肿块大小/cm2 CT值/HU 病理类型 1 男 9岁3个月 24 左颈Ⅲ~Ⅴ区 2.4×1.9 46 HV-CD 2 男 10岁6个月 36 右颈Ⅴ~Ⅵ区 3.0×2.9 55 HV-CD 3 男 10岁5个月 6 左颈Ⅲ区 2.1×5.3 49 HV-CD 4 男 7岁6个月 12 左颈Ⅴ区 1.7×2.3 53 HV-CD 5 女 15岁7个月 1 左颈Ⅲ~Ⅴ区 4.3×2.7 51 HV-CD 6 男 4岁 24 右颈Ⅱ~Ⅲ区 2.9×3.7 42 MT-CD 
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