Diagnosis and Treatment of Extranodal Rosai-Dorfman Disease in Nasal Cavity and Nasopharynx_West China Medical Journal

Authors：

 CHENXiao-li ¹ ,  ZHENGHong ¹ , ZHUXiang-lan ² , DAIWei ¹

1. Department of Otolaryngology, Head and Neck Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

ZHENGHong, Email: hxzhengh@126.com

Keywords：

Rosai-Dorfman disease; Extranodalsinus histiocytosis with massive lymphadenopathy; Nasal cavity; Treatment

DOI：

10.7507/1002-0179.20150199

Video：

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Objective To investigate the clinical manifestations, diagnosis, treatment and prognosis of Rosai-Dorfman disease (RDD) in nasal cavity and nasopharynx. Methods We retrospectively analyzed the clinical data of 4 patients with extranodal RDD admitted to the Department of Otolaryngology, Head and Neck Surgery of our hospital between November 2009 and August 2013. Results All patients received complete surgical removal of the lesions. Histopathological examination showed that large histocytes had an abundant foamy cytoplasm containing ingested lymphocytes as a result of emperipolesis, and were strongly positive for S-100 and CD68 on immunohistochemical analysis, but negative for CD1a. All patients were followed up for 28 to 45 months (averaging 35 months), and no recurrence was observed during the follow-up period. Conclusion RDD is a rare disease, and the exact etiology and pathogenesis of RDD are not fully understood. There is no unified treatment plan recommended, and complete surgical removal of the lesions is an effective method. Minimally invasive surgery may be a good choice for treatment of this disease.

Citation： CHENXiao-li, ZHENGHong, ZHUXiang-lan, DAIWei. Diagnosis and Treatment of Extranodal Rosai-Dorfman Disease in Nasal Cavity and Nasopharynx. West China Medical Journal, 2015, 30(4): 696-699. doi: 10.7507/1002-0179.20150199 Copy

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3.	Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease):review of the entity[J]. Semin Diagn Pathol, 1990, 7(1):19-73.
4.	吕丹, 赵宇, 郑永波. 鼻腔鼻窦Rosai-Dorfman病1例[J]. 第三军医大学学报, 2010, 32(9):958, 962.
5.	Sodhi KS, Suri S, Nijhawan R, et al. Rosai-Dorfman disease:unusual cause of diffuse and massive retroperitoneal lymphadenopathy[J]. Br J Radiol, 2005, 78(933):845-847.
6.	Ensari S, Selçuk A, Dere H, et al. Rosai-Dorfman disease presenting as laryngeal masses[J]. Kulak Burun Bogaz Ihtis Derg, 2008, 18(2):110-114.
7.	Pinto DC, Vidigal Tde A, Castro Bd, et al. Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy[J]. Braz J Otorhinolaryngol, 2008, 74(4):632-635.
8.	Kumari JO. Coexistence of rhinoscleroma with Rosai-Dorfman disease:is rhinoscleroma a cause of this disease?[J]. J Laryngol Otol, 2012, 126(6):630-632.
9.	Wenig B, Abbondanzo S, Childers E, et al. Extranodalsinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck[J]. Hum Pathol, 1993, 24(5):483-492.
10.	Goodnight JW, Wang MB, Sercarz JA, et al. Extranodal Rosai-Dorfman disease of the head and neck[J]. Laryngoscope, 1996, 106 (3 Pt 1):253-256.
11.	Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease):review of the entity[J]. Semin Diagn Pathol, 1990, 7(1):19-73.
12.	Bist SS, Bisht M, Varshney S, et al. Rosai-Dorfman disease[J].EarNose Throat, 2008, 87(1):16-17.
13.	车子刚, 储成凤. 鼻窦Rosai-Dorfman病的临床与影像诊断(附1例报告及文献复习)[J]. 实用放射学杂志, 2011, 27(1):138-140.
14.	Chen HH, Zhou SH, Wang SQ, et al. Factors associated with recurrence and therapeutic strategies for sinonasal Rosai-Dorfman disease[J]. Head Neck, 2012, 34(10):1504-1513.

1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity[J]. Arch Pathol, 1969, 87(1):63-70.
2. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy[J]. Arch Otolaryngol, 1978, 104(12):687-693.
3. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease):review of the entity[J]. Semin Diagn Pathol, 1990, 7(1):19-73.
4. 吕丹, 赵宇, 郑永波. 鼻腔鼻窦Rosai-Dorfman病1例[J]. 第三军医大学学报, 2010, 32(9):958, 962.
5. Sodhi KS, Suri S, Nijhawan R, et al. Rosai-Dorfman disease:unusual cause of diffuse and massive retroperitoneal lymphadenopathy[J]. Br J Radiol, 2005, 78(933):845-847.
6. Ensari S, Selçuk A, Dere H, et al. Rosai-Dorfman disease presenting as laryngeal masses[J]. Kulak Burun Bogaz Ihtis Derg, 2008, 18(2):110-114.
7. Pinto DC, Vidigal Tde A, Castro Bd, et al. Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy[J]. Braz J Otorhinolaryngol, 2008, 74(4):632-635.
8. Kumari JO. Coexistence of rhinoscleroma with Rosai-Dorfman disease:is rhinoscleroma a cause of this disease?[J]. J Laryngol Otol, 2012, 126(6):630-632.
9. Wenig B, Abbondanzo S, Childers E, et al. Extranodalsinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck[J]. Hum Pathol, 1993, 24(5):483-492.
10. Goodnight JW, Wang MB, Sercarz JA, et al. Extranodal Rosai-Dorfman disease of the head and neck[J]. Laryngoscope, 1996, 106 (3 Pt 1):253-256.
11. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease):review of the entity[J]. Semin Diagn Pathol, 1990, 7(1):19-73.
12. Bist SS, Bisht M, Varshney S, et al. Rosai-Dorfman disease[J].EarNose Throat, 2008, 87(1):16-17.
13. 车子刚, 储成凤. 鼻窦Rosai-Dorfman病的临床与影像诊断(附1例报告及文献复习)[J]. 实用放射学杂志, 2011, 27(1):138-140.
14. Chen HH, Zhou SH, Wang SQ, et al. Factors associated with recurrence and therapeutic strategies for sinonasal Rosai-Dorfman disease[J]. Head Neck, 2012, 34(10):1504-1513.

West China Medical Journal

Diagnosis and Treatment of Extranodal Rosai-Dorfman Disease in Nasal Cavity and Nasopharynx

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