title: Multifocal nodal and extranodal Rosai-Dorfman disease initially diagnosed as histiocytic lymphoma.
abstract: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a systemic disease involving nodal and extranodal tissues. We report a 48-year-old female with recurrent nasal obstruction due to polypoidal masses involving the nasal sinuses, turbinates and septum bilaterally, and lumps in the right infra-orbital region and region of the right lacrimal sac. A 4 cm right upper neck mass was also noted, which was initially diagnosed as histiocytic lymphoma. Histopathology of the nasal and infraorbital lesions revealed fibro-inflammatory masses containing histiocytic cells with large vesicular nuclei and abundant foamy cytoplasm exhibiting emperipolesis and lymphophagocytosis, admixed with scattered plasma cells and lymphocytes. These histiocytes revealed immunohistochemical positivity for S-100 protein and CD68, but were negative for CDla. The findings supported a diagnosis of RDD. This report serves to remind pathologists and clinicians of the extranodal manifestations of RDD and its potential confusion with lymphomas.
authors.affiliation: Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor Darul Ehsan. mshiran@yahoo.com
authors.forename: M S
authors.initials: MS
authors.lastname: Shiran
authors.forename: G C
authors.initials: GC
authors.lastname: Tan
authors.forename: M S
authors.initials: MS
authors.lastname: Kenali
authors.forename: A R
authors.initials: AR
authors.lastname: Sabariah
authors.forename: R
authors.initials: R
authors.lastname: Pathmanathan
mesh_terms: D003951:Diagnostic Errors; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis*; D008875:Middle Aged; D015508:Nasal Obstruction / Q000209:etiology*