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Salivary glands

Other tumors

Rosai-Dorfman disease

Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 28 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Histiocytic proliferation with emperipolesis in background of plasma cells and lymphocytes
● First described by Destombes (1965), Rosai and Dorfman (Arch Pathol 1969;87:63)
● Also called sinus histiocytosis with massive lymphadenopathy
● Unknown origin, but associated with immunologic abnormalities

Clinical features

● Rarely presents as salivary gland involvement without significant lymphadenopathy
● Usually long clinical course with exacerbations and remissions and eventual complete remission
● Primarily submandibular and parotid glands

Case reports

● 9 and 10 year old brothers (Dentomaxillofac Radiol 2007;36:428)
● 44 year old man with involvement of salivary glands, cervical lymph nodes, nasal and maxillary sinus mucosa (J Cytol 2009;26:111)
● 45 yr old woman with enlarged submandibular gland (Diagn Cytopathol 2005;33:187)
● 48 year old with systemic lupus erythematosus and parotid gland involvement (Arch Pathol Lab Med 2001;125:1348)
● 71 year old man with parotid mass (J Laryngol Otol 1997;111:1091)
● Child with involvement of submandibular glands (Laryngoscope 2010;120:S228)
● Involving parotid, cervical lymph nodes, breast (J La State Med Soc 2008;160:35)

Micro description

● Histiocytic proliferation with emperipolesis in background of plasma cells and lymphocytes, with eventually effacement of organ and formation of fibrous bands

Micro images

Emperipolesis, histiocytic proliferation, S100+

Nasal mucosa / cervical lymph nodes

Cytology description

● Moderate cellularity, large histiocytes, lymphoplasmocytic infiltrate, histiocytic lymphagocytosis

Cytology images

Cervical lymph nodes

Positive stains

● S100

Differential diagnosis

Melanoma: may phagocytose hematopoietic cells, HMB45+, neoplastic melanocytes with marked atypia
Metastatic carcinoma
● Sinus histiocytosis

End of Salivary glands > Other tumors > Rosai-Dorfman disease

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