Solitary extranodal Rosai-Dorfman disease of the mandible: an exceedingly rare presentation

Abstract

Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.

Keywords: Histiocytosis, NonLangerhan-cell; Histiocytosis, Sinus; Mandible.

Figure 1. CT scan demonstrating lytic lesion involving bilateral mandible (A – Coronal; B – Sagittal and C – Axial plane). Avid radio tracer uptake on scintigraphy (D).

Figure 2. Gross images of the specimen demonstrating entire specimen – A; anterolateral aspect – B; inner aspect – C and cystic portion – D.

Figure 3. Photomicrograph demonstrating diffuse proliferation of histiocytes (A – H&E, 100X) causing local bone destruction (B – H&E, 100X). Histiocytes exhibiting emperipolesis. (C – H&E, 200X, D – H&E, 400X).

Figure 4. Histiocytes exhibiting diffuse immunoreactivity in with CD68 (A – 200X) and S100 (B – 200X).