“ENDOSCOPIC MANAGEMENT OF ROSAI DORFMAN DISEASE OF PARANASAL SINUS AND ORBIT: A CASE SERIES”

Abstract

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive
lymphadenopathy, is a rare, benign histiocytic disorder of uncertain etiology. Initially
identified in African children with lymphadenopathy, the disease was later characterized by
Rosai and Dorfman in 1969. The clinical presentation typically includes painless cervical
lymphadenopathy, fever, and extranodal involvement, particularly in the head and neck region.
Diagnosis is confirmed histopathologically by the presence of large histiocytes exhibiting
emperipolesis and positivity for S100, CD68, and CD163, with negativity for CD1a. We
present five cases of extranodal RDD involving the paranasal sinuses, an uncommon site of
manifestation. Patients ranged from 23 to 70 years old, presenting with symptoms such as nasal obstruction and postnasal discharge. Imaging revealed soft tissue masses in the nasal cavity and paranasal sinuses. All cases were managed successfully through endoscopic surgical excision, with subsequent symptom relief and no recurrence during follow-up.
Histopathological examination confirmed RDD with characteristic histiocytic infiltration and
emperipolesis. These cases underscore the importance of considering RDD in the differential
diagnosis of nasal masses, particularly when typical lymphadenopathy is absent. Given the
rarity of paranasal sinus involvement, management requires a tailored approach, including
endoscopic debulking and regular follow-up. Our findings highlight the efficacy of surgical  intervention in isolated extra nodal RDD and the necessity of vigilant postoperative monitoring. Further studies with larger cohorts are needed to establish standardized treatment protocols for this rare manifestation of RDD