[The clinicopathologic features of unicentric Castleman disease in the children's neck]

【儿童颈部单中心Castleman病的临床病理特征】

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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.

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