Abstract
Rosai–Dorman disease, also known as sinus histiocytosis with massive lymphadenopathy, typically presents as massive enlargement of lymph nodes accompanied by systemic symptoms. Extranodal involvement is also known usually in head and neck regions. Rarely the disease presents at extranodal sites only without involvement of lymph nodes.We present a case of 58 years-old female who presented with mass in nasal septum without involvement of lymph nodes. The mass was removed surgically and diagnosis was made on histological grounds with classical indings of histiocytic proliferation and emperipolesis.
Highlights
Rosai–Dorman disease (RDD), known as sinus histiocytosis with massive lymphadenopathy (SHML), a rare and usually self limiting disease, was originally described by Destombes in 1965.1 Subsequently; in 1969, it was characterized as a distinct clinicopathological disorder by Rosai and Dorfman.[2]
In 43% cases disease may have extra nodal involvement[4], most common sites being nasal cavities, skin, eyes, orbit, eyelids, bones, soft tissue, the central nervous system, upper respiratory tract and less commonly, the gastrointestinal tract, But it may present itself at extra nodal sites only in absence of obvious lymphadenopathy.[5,6,7,8,9,10,11]
Rosai–Dorman disease typically manifests in childhood or in early adulthood, mostly in the second and third decades of life.[12]
Summary
Rosai–Dorman disease (RDD), known as sinus histiocytosis with massive lymphadenopathy (SHML), a rare and usually self limiting disease, was originally described by Destombes in 1965.1 Subsequently; in 1969, it was characterized as a distinct clinicopathological disorder by Rosai and Dorfman.[2]. In 43% cases disease may have extra nodal involvement[4], most common sites being nasal cavities, skin, eyes, orbit, eyelids, bones, soft tissue, the central nervous system, upper respiratory tract and less commonly, the gastrointestinal tract, But it may present itself at extra nodal sites only in absence of obvious lymphadenopathy.[5,6,7,8,9,10,11]. The disease usually follows an insidious course but could be fatal due to involvement of vital organs. It is characterized by looding of lymphatic sinuses by histiocytes and plasma cells. The lesion was causing expansions of the nasal cavity with smooth scalloping and remodeling of adjacent bones. Histopathological and immunohistochemical indings were consistent with the diagnosis of RDD– extranodal involvement
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