Rosai-Dorfman Disease

Rosai-Dorfman disease, also known as sinus histiocytosis with massive adenopathy, is a macrophage-related histiocytic disorder of varied biological behavior.

Rosai-Dorfman disease predominantly affects children and adolescents, who most commonly with bilateral painless cervical lymphadenopathy and fever. There is a slight male predominance.

Extranodal involvement, seen in about 40% of cases, can occur with or without lymphadenopathy and can be solitary or multiple. Common extranodal sites include the nasal cavity and paranasal sinuses (shown above), although involvement has also been reported of the soft tissue, central nervous system, orbit, skin, oral cavity, bone, kidneys, upper respiratory tract, gastrointestinal tract, testicles and salivary glands.

The image above is from an atypical presentation in a middle-aged woman who presented with isolated paranasal sinus involvement. Biopsy of the left maxillary sinus lesion revealed the characteristic histopathologic feature of emperipolesis: Histiocytes with intracytoplasmic lymphocytes, plasma cells, erythrocytes, and/or polymorphonuclear leukocytes (from phagocytosis by the histiocytes). Another feature that can be seen is intracytoplasmic eosinophilic globules (Russell bodies) in the phagocytized plasma cells. These features are usually less prominent in extranodal Rosai-Dorfman disease, which was the case in this patient, who required a surgical biopsy for definitive diagnosis.

References

• La Barge DV 3rd, Salzman KL, Harnsberger HR, Ginsberg LE, Hamilton BE, Wiggins RH 3rd, Hudgins PA. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol. 2008 Dec;191(6):W299-306.
• Gupta P, Babyn P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease. Pediatr Radiol. 2008 Jul;38(7):721-8; quiz 821-2. Epub 2008 Feb 2.