Rosai-Dorfman disease of the paranasal sinuses and orbit

Abstract

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination.

Rosai–Dorfman‐type histiocytes occur in eosinophilic chronic rhinosinusitis and potentially play a role in disease initiation and persistence

“…Isolated sinonasal RDD poses a diagnostic challenge in the absence of accompanying lymphadenopathy. At presentation, symptoms of sinonasal RDD include nasal obstruction, epistaxis, hyposmia or anosmia, nasal and facial deformity . The average age of presentation is 40 years, with a slight male predominance .…”

“…Histologically, cases are characterized by RDH and background lymphocytes and plasma cells, with emperipolesis of these and of red cells. It may involve the anterior nasal space and/or posterior nasal space and sinuses, often with polyps, and may be unilateral or bilateral . Blood parameters may be normal, but sinonasal isolated RDD may show changes seen in classical presentations of RDD such as ESR elevation and plasma protein abnormalities .…”

“…RDD‐type histiocytes (RDH) are characterized by S100 protein expression and they lack CD1a . Sinonasal RDD displays RDH within an inflammatory background, which typically includes lymphocytes and plasma cells . Chronic rhinosinusitis (CRS) is common, with an incidence of between 2 and 14%, and may present with and without nasal and/or sinus polyposis.…”

“…3 Sinonasal RDD displays RDH within an inflammatory background, which typically includes lymphocytes and plasma cells. [4][5][6][7][8] Chronic rhinosinusitis (CRS) is common, with an incidence of between 2 and 14%, and may present with and without nasal and/or sinus polyposis. In western countries eosinophilic CRS (ECR) is the most common category of CRS with polyposis.…”

Anesthetic management of a patient with Rosai-Dorfman Disease and atrial septal defect for orbital surgery

“…For monitoring of temperature, nasopharyngeal probes should be avoided, since the nose is a common site of extranodal involvement in RDD. [6] Care should be taken during positioning as RDD may be associated with the osteolytic skeletal lesions.…”