Rosai-Dorfman Disease in Cervical Lymph Nodes: The Challenges of Diagnosis in a Resource Limited Setting and Use of Immunohistochemistry in the Diagnosis

We report a case of Rosai-Dorfman disease in a 28-year-old Nigerian woman with a 7-year history of painless prominent bilateral neck swelling which waxed and waned over the years. She had two misdiagnosis hence appropriate therapy was not initiated over the years. She was eventually referred for independent opinion and was diagnosed correctly with review of the histology sections and with the use of CD45, S100, CD 68, CD 15, CD 20, synaptophysin and AE1/AE3 immunohistochemistry markers. Classic features on Haematoxylin and eosin stained sections and positivity of the lesional cells for S100 and CD68 were diagnostic of Rosai-Dorfman disease. She was subsequently placed on oral steroids with minimal objective reduction in the neck circumference from 57 to 46 cm. After two months, she was managed with three courses of chemotherapy (cyclophosphamide, doxorubicin hydrochloride, vincristine and prednisolone) which resulted to a significant sustained reduction in her neck circumference to 36 cm. She has been on follow up for about a year without a recurrence.