Rosai-Dorfman disease: a retrospective analysis of 13 cases

• Published in: The American journal of the medical sciences Vol. 345; no. 3; p. 200
• Main Authors: Zhu, Fei, Zhang, Jia-tang, Xing, Xiao-wei, Wang, Dian-jun, Zhu, Ru-yuan, Zhang, Qun, Wang, Hong-tian, Lang, Sen-yang
• Format: Journal Article
• Language: English
• Published: United States 01.03.2013
• Subjects: Cerebellum - metabolism; Cerebellum - pathology; Female; Histiocytosis, Sinus - metabolism; Histiocytosis, Sinus - pathology; Humans; Immunohistochemistry; Male; Middle Aged; Nasal Cavity - metabolism; Nasal Cavity - pathology; Paranasal Sinuses - metabolism; Paranasal Sinuses - pathology; Prognosis; Retrospective Studies; Spinal Cord - metabolism; Spinal Cord - pathology
• ISSN: 1538-2990
• DOI: 10.1097/MAJ.0b013e3182553e2d

Rosai-Dorfman disease (RDD) is a rare, idiopathic, histiocytic proliferative disorder, the infrequent occurrence of which limits in-depth studies. Consequently, many characteristics of this disease remain unknown, restricting early diagnosis and proper treatment. In this study, the literature was reviewed and a retrospective analysis of the medical records of 13 patients with RDD conducted to investigate the demographic data, clinical data, laboratory and imaging results, treatment, and prognosis of this disease. Of the 13 cases in our sample, 10 (77%) were purely extranodal RDD, 2 (15%) were both nodal and extranodal, and 1 (8%) was purely nodal. The locations of the 10 purely extranodal RDD lesions included the central nervous system (n = 6, 60%), nasal cavity and paranasal sinuses (n = 3, 30%), and the cutis (n = 1, 10%). The locations of the central nervous system-related RDD lesions included the cerebral subdura (n = 2, 29%), the sellar region (n = 3, 14%), the cerebral parenchyma (n = 1, 14%) and the spinal subdura (n = 1, 14%). Ten patients (77%) had stable conditions, 3 (23%) experienced recurrence, and 2 (15%) experienced recurrence and lesion metastasis. RDD is rare, requiring knowledge of its clinical manifestations for a rapid and correct diagnosis. In light of the possibility of recurrence and lesion metastasis, long-term follow-up is needed. Treatment is still controversial. Future efforts should be directed at investigating the etiology and postoperative treatment for relapsing cases or those with subresected lesions.