Rosai-Dorfman disease: a retrospective analysis of 13 cases
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 re...
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Published in | The American journal of the medical sciences Vol. 345; no. 3; p. 200 |
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Main Authors | , , , , , , , |
Format | Journal Article |
Language | English |
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United States
01.03.2013
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Abstract | 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. |
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AbstractList | 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. |
Author | Xing, Xiao-wei Zhang, Jia-tang Lang, Sen-yang Zhu, Ru-yuan Zhang, Qun Wang, Hong-tian Zhu, Fei Wang, Dian-jun |
Author_xml | – sequence: 1 givenname: Fei surname: Zhu fullname: Zhu, Fei organization: Departments of Neurology, Chinese People's Liberation Army General Hospital, Beijing, China – sequence: 2 givenname: Jia-tang surname: Zhang fullname: Zhang, Jia-tang – sequence: 3 givenname: Xiao-wei surname: Xing fullname: Xing, Xiao-wei – sequence: 4 givenname: Dian-jun surname: Wang fullname: Wang, Dian-jun – sequence: 5 givenname: Ru-yuan surname: Zhu fullname: Zhu, Ru-yuan – sequence: 6 givenname: Qun surname: Zhang fullname: Zhang, Qun – sequence: 7 givenname: Hong-tian surname: Wang fullname: Wang, Hong-tian – sequence: 8 givenname: Sen-yang surname: Lang fullname: Lang, Sen-yang |
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SubjectTerms | 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 |
Title | Rosai-Dorfman disease: a retrospective analysis of 13 cases |
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