A 5 year old male of African origin presents with a right-sided massive axial proptosis with corneal exposure/ulceration. An intraconal and extraconla mass is seen on the scan and he under goes a biopsy.
1. Describe Fig 1
2. Which cells are seen in Fig 2?
3. Which cells are seen in Fig 3?
4. Name the stain used in Fig 4 and which pathological process is delineated?
5. What is the diagnosis?
1. Light and dark celular zones with sharp demarcation.
2. Eosinophilic histiocytes with slighly bubbly cytoplasm.
3. Mature plasma cells.
4. S100 staining the histiocytes with negative profile of ingested lymphocytes-this is emperipolesis.
5. Orbital extra-nodal Rosai-Dorfman disease.
Tan JJ, Narang S, Purewal B, Langer PD, Blaydon S, Schwarcz RM, McCormick SA, Kim JY, Walrath JD, Burt BO, Shinder R. Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases. Ophthal Plast Reconstr Surg. 2015
McClellan SF, Ainbinder DJ. Orbital Rosai-Dorfman disease: a literature review. Orbit. 2013 Oct;32(5):341-6