Multifocal facial Rosai Dorfman disease with postsurgical recurrence


  • Ishita Sen Department of ENT, Suri Superspeciality Hospital, Birbhum, West Bengal, India
  • Subhadeep Chowdhury Department of ENT, R. G. Kar Medical College and Hospital, West Bengal, India
  • Tithi Debnath Department of ENT, R. G. Kar Medical College and Hospital, West Bengal, India



Sinus histiocytosis, Multifocal, Postsurgical recurrence, Emperipolesis, Radiotherapy


Rosai Dorfman disease (RDD) is a histiocytic proliferative disorder with massive painless lymphadenopathy that though is rare yet has been reported more than seldom. Our report aimed to emphasis on a case with multifocal facial involvement with postsurgical recurrence, which had not yet been reported and an approach to alleviate the patient’s symptoms and avert a fatal outcome. This study was a case report and literature review. A 19 year old female presented with swelling involving right sided cheek, periorbital region and bilateral nasal cavity with palatal perforation for last 6 months. She had a past history of debulking of orbital RDD. Contrast enhanced computed tomography revealed an extraconal mass of the right orbit and left nasal mass extending to the nasopharynx. Biopsy taken from the nasal masses showed RDD on histopathology and immunohistochemistry. Though the disease was self-resolving, this patient required radiotherapy as debulking was not sufficient for its multifocal presentation and was followed by palatal reconstruction.


Agarwal A, Pathak S, Gujral S. Sinus histiocytosis with massive lymphadenopathy-a review of seven cases. Indian J Pathol Microbiol. 2006;49(4):509-15.

Dalia S, Sagatys E, Sokol L, Kubal T. Rosai-Dorfman disease: tumor biology, clinical features, pathology and treatment. Cancer Control. 2014;21(4):322-7.

Ha H, Kim KH, Ahn YJ, Kim JH, Kim JE, Yoon SS. A rare case of Rosai-Dorfman disease without lymphadenopathy. Korean J Intern Med. 2016;31(4):802-4.

National Organization for Rare Diseases. Fact sheet: Rosai-Dorfman Disease, 2014. Available at: Accessed on 8 May 2021.

Feriante J, Lee RT. Case report Rosai‐Dorfman disease: self‐resolving unilateral lymphadenopathy and a brief review of literature. Case reports Oncol Med. 2018;2018(10):1‐4.

Mantilla JG, Goldberg-Stein SA, Wang Y. Extranodal Rosai-Dorfman disease: Clinicopathologic series of 10 patients with radiologic correlation and review of the literature. Am J Clin Pathol. 2016;145(2):211-21.

Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile J, et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018;131(26):2877-90.

Sanli A, Eken M, Oktay ZA, Paksoy M, Hardal U. Rosai-Dorfman disease: sinus histiocytosis with massive lymphadenopathy. BMJ Case Rep. 2009;2009:11.

Narjess BR, Meriem Z, Leila K, Fafani BHH, Hachmi M. Bilateral isolated Rosai-Dorfman disease of the orbit. Can J Opthalmol. 2018;54(2):77-81.

Wang E, Anzai Y, Paulino A, Wong J. Rosai-Dorfman disease presenting with isolated bilateral orbital masses: report of two cases. AJNR Am J Neuroradiol. 2001;22(7):1386-8.

Pradhananga RB, Dangol K, Shrestha A, Baskota DK. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a case report and literature review. Int Arch Otorhinolaryngol. 2014;18(4):406-8.

Janku F, Patel BSH, Raghavan VK, Goodman A, Barnes TG, Kurzrock R. MEK inhibition with trametinib in patients with non-langerhans cell histiocytosis. Blood. 2019;134(1):2319.

Chen H, Zhou S, Wang S, Teng X, Fan J. Factors associated with recurrence and therapeutic strategies for sinonasal Rosai-Dorfman disease. Head Neck. 2012;34(10):1504-13.

Komaragiri M, Sparber LS, Santos-Zabala ML, Dardik M, Chamberlain RS. Extranodal Rosa-Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma. World J Surg Onc. 2013;11:63.






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