Table 3 summarizes the literature review of gene mutations described in RDD [21,28,29,30,31,32,33,34,35,51,54,55,56]. Approximately, 33–50% of RDD cases were detected with the genomic mutation involving KRAS, NRAS, ARAF, and MAP2K1 [21,28,29,30,31,32,33], and only two cases with BRAF mutation were reported [34,35]. Here, MAP2K1 is linked to sinus histiocytosis with massive lymphadenopathy.