Meng Q L, Li B, Wang Y M, Liu Y F, Bao X H, Wang S F, Zhang Shufeng
Department of Pediatric Surgery, Henan Provincial People's Hospital, Zhengzhou 450003, China.
Department of Pediatric Hematology and Oncology, Children's Hospital, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Zhonghua Er Ke Za Zhi. 2022 Mar 2;60(3):227-231. doi: 10.3760/cma.j.cn112140-20210601-00471.
To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman's disease in children. Clinical data of 15 children diagnosed with Castleman's disease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Among the 15 Castlemans disease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological type was hyaline vascular (11 cases). Unicentric lesions were most common in the neck (11 cases), all 13 patients received complete surgical resection of the lesions, the follow-up time was 20.0 (13.5, 50.5) months, and the prognosis was good. Two cases were multicentric type, the pathological types were mixed variant, meeting the criteria of idiopathic Castleman's disease, the two children underwent partial surgical resection, one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone. The follow-up time was 32 months and 10 month, both of them had good prognosis. Most cases of Castleman's disease in children are diagnosed late, and the unicentric type is dominant. The most common pathological type is hyaline vascular, which is characterized by painless lymphadenopathy, while multicentric type has systemic symptoms and both of them have a good overall prognosis.
探讨儿童Castleman病的临床特征、病理表型、治疗及预后。回顾性分析2010年5月至2019年10月在河南省人民医院和郑州大学第一附属医院确诊的15例儿童Castleman病的临床资料。分析其临床特征、实验室检查及组织病理学资料。15例Castleman病患者中，男12例，女3例。首次就诊年龄为12（10，15）岁。从发现肿块到病理诊断的时间为9.0（2.0，13.0）个月。大多数患者为单中心型（13例），组织病理学类型为透明血管型（11例）。单中心病变最常见于颈部（11例），13例患者均接受了病变的完整手术切除，随访时间为20.0（13.5，50.5）个月，预后良好。2例为多中心型，病理类型为混合变异型，符合特发性Castleman病的标准，2例患儿均接受了部分手术切除，1例接受利妥昔单抗和泼尼松治疗，另1例接受沙利度胺和泼尼松治疗。随访时间分别为32个月和10个月，二者预后均良好。儿童Castleman病多数诊断较晚，以单中心型为主。最常见的病理类型为透明血管型，表现为无痛性淋巴结肿大，而多中心型有全身症状，二者总体预后均良好。
