An unusual presentation of extranodal Rosai–Dorfman disease threatening the airway

A Case of Extranodal Rosai-Dorfman Disease Presenting as an Isolated Mass on the Base of the Tongue in a 57-Year-old Woman

“…Extranodal involvement may be the initial clinical presentation in approximately 25% of patients with RDD, which can progress to a chronic, relapsing clinical course that has a worse prognosis than regular RDD [ 6 , 10 , 15 – 18 ]. RDD can occur in any age group; however, it is most common in children and young adults, and is more frequent in males [ 5 , 7 , 18 ]. The disease usually presents as a large, bilateral, painless lymphadenopathy that is often accompanied by systemic symptoms, such as fever, night sweats, fatigue, and weight loss, the severity of which depends on the sites involved [ 1 ].…”

“…The etiology and pathogenesis of RDD is still poorly understood; however, several theories have been proposed [ 5 , 7 , 12 ]. One theory is that the stimulation received via the macrophage colony-stimulating factor (M-CSF) leads to histiocytic proliferation due to a disturbance of cell-mediated immunity, auto-immune mechanisms, and altered immune responses [ 5 , 18 ] Viral infections such as Epstein-Barr, varicella-zoster, and human herpesvirus, have also been proposed as possible causes of RDD [ 5 , 7 , 8 , 12 , 18 , 19 ].…”

“…Histopathologically, RDD typically demonstrates extensive inflammatory infiltration with numerous histiocytes, lymphocytes, plasma cells and a few neutrophils. Enlarged histocytes with phagocytosis of lymphocytes and neutrophils within their cytoplasm, which is known as emperipolesis, is a common pathognomonic finding [ 5 – 7 ].…”

“…The head and neck are the typically affected extranodal sites [ 7 , 8 , 10 , 17 ]. Although the pharynx and supraglottis are not considered as lymphoid organs, they have a large amount of lymphatic tissue in the submucosal region as part of the mucosa-associated lymphoid tissue (MALT) system [ 24 ], which makes them vulnerable to extranodal RDD involvement.…”

“…Good outcomes have been reported in the treatment of laryngeal and tracheal RDD by surgical excision [ 13 , 19 ]. Further, the mass on the BOT was required for differential diagnosis from malignant carcinoma, as well as benign masses, such as vallecular cyst, thyroglossal duct cyst, and hemangiomas [ 7 ]. In the current case, the early stage of extranodal RDD without lymphoadenopathy was confined to the BOT.…”