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Case Study: March 2008
History
A 12-year-old-boy presents with a mass in the nasal cavity, maxillary sinus and orbit.
The orbit component is biopsied.
Figure 1-H and E low power of mass showing ‘dark’ and ‘light’ areas.
Figure 2-Higher power of ‘dark’ area.
Figure 3-Higher power of ‘light’ area.
Figure 4- CD68 immuno on ‘light’ area
Figure 5-S100 immuno on ‘light’ area
The ‘light’ area cells were negative for CD1a, Melan A and cytokeratins.
Questions
1. What are the cells in the ‘light’ areas?
2. What is the predominant cell type in the ‘dark’ areas?
3. What is the diagnosis?
4. Whilst not fully developed in this case, which other histological feature in this entity would you look for?
1. Histiocytes (monocyte-macrophages).
2. Plasma cells
3. The anatomical location, histological features and immunophenotype all add up to a diagnosis of extranodal, orbital Rosai-Dorfman disease.
4. Emperipolesis.
References
Foucar E, Rosai J, Dorfman RF.The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol. 1979 Mar;87(3):354-67.
Lee-Wing M, Oryschak A, Attariwala G, Ashenhurst M.Rosai-Dorfman disease presenting as bilateral lacrimal gland enlargement. Am J Ophthalmol. 2001 May;131(5):677-8.
Khan R, Moriarty P, Kennedy S.Rosai Dorfman disease or sinus histiocytosis with massive lymphadenopathy of the orbit.
Br J Ophthalmol. 2003 Aug;87(8):1054.
de Silva D, Joshi N. Rosai-Dorfman disease recurrence with bilateral orbital masses following immunosuppressant therapy. Orbit. 2005 Mar;24(1):51
Malur PR, Bannur HB, Kodkany SB.Orbital Rosai-Dorfman disease: report of a case with fine needle aspiration cytology and histopathology. Acta Cytol. 2007 Jul-Aug;51(4):581-2.
Goldberg S, Mahadevia P, Lipton M, Rosenbaum PS.Sinus histiocytosis with massive lymphadenopathy involving the orbit: reversal of compressive optic neuropathy after chemotherapy. J Neuroophthalmol. 1998 Dec;18(4):270-5.
Marion JR, Geisinger KR.Sinus histiocytosis with massive lymphadenopathy: bilateral orbital involvement spanning 17 years. Ann Ophthalmol. 1989 Feb;21(2):55-8.
Friendly DS, Font RL, Rao NA. Orbital involvement in ‘sinus’ histiocytosis. A report of four cases. Arch Ophthalmol. 1977 Nov;95(11):2006-11.
