A ten year old girl presented to us with inferonasally displaced, non-axial proptosis of oculus dexter (OD), with ipsilateral, painless massive cervical lymphadenopathy of ten weeks duration. She also complained of diplopia on dextroelevation for the past four weeks. There was no history of systemic disease, surgery, trauma or associated visual or ocular complaints such as redness, discharge, lacrimation etc.

After a detailed hematological work-up and imaging, the histological evidence obtained on cervical lymph node biopsy established the diagnosis of Rosai-Dorfman disease.

Only 50% of cases require definitive management, as the condition may be self-limited. There is no definite treatment protocol. Management options include observation for mild manifestations with no cosmetic or functional abnormality; surgical excision or debulking for lesion in surgically accessible locations and/or systemic corticosteroids, chemotherapy (vinca alkaloids, alkylating agents etc.) or radiotherapy in patients with severe symptoms where vital organ function is compromised causing severe handicap

In view of the young age of the patient, medical management was adhered to, so as to avoid surgical trauma and post-operative scarring due to debulking. After consulting the paediatric oncologist, medical management was started with intravenous dexamethasone for five days, followed by oral steroids and 6-mercaptopurine for eight weeks with excellent resolution of lacrimal gland swelling clinically.

Considering the rapid and sustained response to corticosteroids and chemotherapy, in addition to prevention of residual disease or recurrence, this line of management seems to be a promising approach in the management of RDD.

Although Rosai-Dorfman disease of unilateral lacrimal gland is extremely infrequent, a high degree of suspicion is warranted in a case of young patient presenting with unilateral isolated lacrimal gland swelling and associated lymphadenopathy, wherein no other cause is found. Treatment protocol should be individualized as per the extent of systemic involvement and functional disability.

Recent Publications:

1.     Sujata G. Multifocal, Extranodal Sinus Histiocytosis With Massive Lymphadenopathy. An Overview. Arch Pathol Lab Med. 2007; 131(7):1117-1121.
2.     Geeta K Vemuganti, Milind N Naik, Santosh Honavar. Rosai Dorfman disease of the orbit. J Hematol Oncol. 2008;1:7.
3.     Pinto DCG, Vitigal TA, Castro B, Santos BH, DeSousa NJA: Rosai- Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Braz J Otorhinolaringol 2008,74:632-635.
4.     Mantilla JG, Goldberg-Stein S, Wang Y. Extranodal Rosai-Dorfman Disease: Clinicopathologocail Series of 10 Patients with Radiologic Correlation and Review of the Literature. Am J Clin Pathol. 2016;145(2):211-221
5.     Dalia S, Sagatys E, Sokol L et al. Rosai-Dorfman disease: tumour biology, clinical features, pathology and treatment. Cancer Control. 2014;21(4):322-327