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Infantile Rosai-Dorfman disease: an unusual case of neck swelling and a literature review

机译:Infantile Rosai-Dorfman病:一个不寻常的颈部肿胀和文献综述

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摘要

Rosai-Dorfman disease (RDD) is a benign, rare histiocytic disorder presenting as a wide clinical spectrum, which mainly affects bilateral dilated lymph node enlargement. This study aimed to show an infantile RDD and review the clinicopathologic features, imaging, and histological features of RDD, including pitfalls in diagnosis. We report here an infant who had a 3-month history of painless bilateral cervical masses progressed to tracheal compression. Postoperative immunohistochemical results with positive characteristic staining of CD68 and S-100, negative for CD1a, which helped to determine the diagnosis. This peculiar case is the youngest case of RDD presenting with painless massive lymph node progressing to severe dyspnea due to tracheal compression. The optimal treatment still remains challenging. Future research should focus more on etiology and pathogenesis of RDD, especially relapsing cases.
机译:Rosai-Dorfman病(RDD)是一种良性,稀有的组织细胞疾病,作为一种宽临床光谱,主要影响双侧扩张淋巴结扩大。本研究旨在显示婴儿RDD,并审查RDD的临床病理特征,成像和组织学特征,包括诊断中的缺陷。我们在这里报道了一个婴儿,婴儿患​​有3个月的无痛双侧宫颈群体的历史,进展了气管压缩。术后免疫组织化学结果具有CD68和S-100的阳性特征染色,CD1a为阴性,有助于确定诊断。这种特殊的案例是由于气管压缩引起严重呼吸困难,患有无痛的大规模淋巴结的RDD最小的案例。最佳治疗仍然持挑战性。未来的研究应更加关注RDD的病因和发病机制,尤其是复发案件。

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