ROSAI-DORFMAN'S DISEASE IN THE GREATER SALIVARY GLAND: A CASE REPORT

Abstract

Rosai-Dorfman Disease (DRD), also known as sinus histiocytosis with massive lymphadenopathy, is a pathology of uncertain, rare, and challenging biological potential, characterized by the proliferation of S100-positive histiocytes and the phenomenon of emperipolesis, where intact cells, such as lymphocytes, are found in the cytoplasm of these histiocytes. Although classically described as a nodal disease, DKD can manifest in extranodal sites, and the involvement of the salivary glands, especially in isolation, is extremely uncommon. In many cases, the disease is self-limiting, resolving spontaneously without the need for specific treatment. This report describes a rare case of DKD with exclusive involvement of the left submandibular gland in a 41-year-old female patient who presented with a submandibular mass with no other symptoms. After a surgical excision procedure, the diagnosis was confirmed by histopathological and immunohistochemical examinations, which revealed histiocytosis with emperipolesis and positivity for S100, CD68 and HAM56. The details of this case contribute to the understanding of DKD in extranodal sites, expanding the clinical-pathological spectrum of this rare entity.