Refractory Extra nodal Rosai-Dorfman disease of cheek and upper eyelid: A rare case

  • Manjusha Biswas Vivekananda Institute of Medical Sciences, Kolkata, India.
  • Tirthankar Sarkar
  • Anjan Das
  • Snehanshu Naskar
Keywords: Rosai Dorfman Disease, Extra nodal, Refractory, Cheek mass, S-100, CD1a


Rosai-dorfman disease (RDD) is a rare benign disorder of histiocytes characterized histologically by intracellular engulfment of lymphocytes. Extra-nodal RDD has been reported in 43% of cases. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. A 31-year-old female presented with a history of right cheek mass and right upper eyelid mass gradually increasing for 8 months, with no palpable cervical lymphadenopathy. The masses were excised and sent for histopathological examination. The histiocytes showed positive immunostaining for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient was on systemic steroids and was doing well. After 12 months of follow-up, patient had recurrence in right cheek, which was again excised. Again the mass recurred after 10 months, was excised and patient was put on radiotherapy to the cheek mass. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. The causes of RDD are not fully understood, and treatment strategies can be different according to severity or vital organ involvement.

Author Biography

Manjusha Biswas, Vivekananda Institute of Medical Sciences, Kolkata, India.
Assistant Professor, Department of Pahology,


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