Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Abstract

ABSTRACTBACKGROUNDWe hypothesize that lymphadenopathy and extraosseous manifestation affect the management protocol and our aim is to aid in development of standardized diagnosis and treatment protocols.METHODSPubMed and Google Scholar were searched from January 2012 through December 2022 using keywords “Rosai-Dorfman”, “Rosai and Dorfman,” and “bone“. Out of 98 researches, meta-analysis of 51 full-text studies involving 53 individuals with osseous RDD was conducted.RESULTSA significant association was found between lymphadenopathy with steroid therapy (OR: 20.125, 95%CI: 3.02, 134.09, P=0.0004). Extraosseous RDD showed significant association with steroid therapy (P=0.0145), T1-isointense MRI (OR: 0.173, 95%CI: 0.0304, 0.986, P=0.035), T1-hyperintense MRI (OR: 0.16, 95%CI: 0.0314, 0.815, P=0.019) and T2-hyperintense MRI (OR: 0.115, 95%CI= 0.0206, 0.6456, P=0.007).CONCLUSIONOsseous RDD is rare and lacks standardized diagnosis and treatment protocols. Patients with lymphadenopathy were 9 times more likely to receive steroids, and those with extraosseous manifestations were less likely to undergo MRI. RDD is often not initially considered in differential diagnoses, leading to worsened conditions. We propose that further testing is required in affected individuals to develop standardized protocols.Key PointsRevealed variations in osseous and extraosseous involvement and immunohistochemistry played a crucial role in diagnosis.Found association of lymphadenopathy and extraosseous involvement with steroid therapy and diagnostic modalities of RDD.Limitations include reliance on case reports (low-quality evidence), potential inclusion bias in reported data.