A Rarest of Rare Presentation of ROSAI - DORFMAN Disease Involving Para Nasal Sinuses with Massive Lymphadenopathy and Renal Mass

Gopesh Panwar, Neeraj Sharma, Jitendra Amlani, Vivek Joshi

Abstract: INTRODUCTION Lampert and Lennert in 1961 were the first to describe what is now known as Rosai-Dorfman disease (RDD). Subsequently in 1969 Rosai and Dorfman described 4 cases of a disease they called sinus histiocytosis with massive lymphadenopathy (better known as RDD). CASE A 23-year-old man presented with loss of appetite, significant weight loss( 10 kg in last 6 month) and dull left flank pain for 6 month. There was past history of chronic nasal polyps and sinusitis for which he underwent nasal sinus surgery (Three times in last six years). Physical examination revealed averagely nourished individual without peripheral lymphadenopathy and organomegaly. CONCLUSION Involvement of the paranasal sinuses, kidney and para aortic reteroperitoneal lymph node as encountered by us is an extremely rare presentation even not reported so far in literature of Rosai-Dorfman disease. The diagnosis is primarily based on histopathological one and confirmation by IHC- CD68, S-100 positivity.and CD1a negitivity.

Keywords: ROSAI-DORFMAN DISEASE,histiocytosis,lymphadenopathy,Nephrectomy, paranasal sinuses.