Autopsy and Case Reports
Autopsy and Case Reports
Article / Clinical Case Report

Rosai-Dorfman disease affecting the maxilla

Thaís Gimenez Miniello, Juliane Piragine Araujo, Norberto Nobuo Sugaya, Fernando Melhem Elias, Oslei Paes de Almeida, Fabio Abreu Alves


Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.


Histiocytosis, Sinus, Maxilla, Emperipolesis, Diagnosis, Oral


1. Thomson ER, Newman P, Dunstan S, Coull H. The Rosai Dorfman syndrome in a 50-year-old male. Br J Oral Maxillofac Surg. 1989;27(1):39-45. PMid:2920162.

2. Yontz L, Franco A, Sharma S, Lewis K, McDonough C. A case of Rosai-Dorfman disease in a pediatric patient with cardiac involvement. J Radiol Case Rep. 2012;6(1):1-8. PMid:22690274.

3. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 Cases. Cancer. 1972;30(5):1174-88. PMid:5083057.<1174::AID-CNCR2820300507>3.0.CO;2-S.

4. McAlister WH, Herman T, Dehner LP. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Pediatr Radiol. 1990;20(6):425-32. PMid:2202971.

5. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19-73. PMid:2180012.

6. Cardoso CL, Damante JH, Santos PSS, et al. Rosai-Dorfman disease with widespread oral-maxillofacial manifestations: a case report. J Oral Maxillofac Surg. 2012;70(11):2600-4. PMid:22330332.

7. Kademani D, Patel SG, Prasad ML, Huvos AG, Shah JP. Intraoral presentation of Rosai-Dorfman disease: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;93(6):699-704. PMid:12142877.

8. Keskin A, Genç F, Günhan Ö. Rosai-Dorfman disease involving maxilla: a case report. J Oral Maxillofac Surg. 2007;65(12):2563-8. PMid:18022485.

9. Akyigit A, Akyol H, Sakallioglu O, Polat C, Keles E, Alatas O. Rosai-Dorfman disease originating from nasal septal mucosa. Case Rep Otolaryngol. 2015; 2015:1-3.

10. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis. 1992;166(2):291-5. PMid:1321861.

11. Wang KH, Cheng CJ, Hu CH, Leew WR. Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai–Dorfman disease. Br J Dermatol. 2002;147(4):770-4. PMid:12366428.

12. O’Malley D, Duong A, Barry TS, et al. Co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: possible relationship of two histiocytic disorders in rare cases. Mod Pathol. 2010;23(12):1616-23. PMid:20729813.

13. Kutty SA, Sreehari S. Co-occurrence of intracranial Rosai-Dorfman disease and Langerhans histiocytosis of the skull: case report and review of literature. Turk Neurosurg. 2015;25(3):496-9. PMid:26037195.

14. Castillo BTD, Mata-Fernandez C, Soria VJR, Blanco VP, Loughlin G, Campos-Domínguez M. Self-healing extranodal cutaneous Rosai-Dorfman in a child. Pediatr Dermatol. 2015;32(6):e249-59. PMid:26391332.

15. Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):67-71. PMid:11835335.

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