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.
Keywords: Rosai-Dorfman disease; infant; neck masses.
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