Rosai‐Dorfman Disease with Isolated Laryngeal Involvement

Objective Rosai‐Dorfman (RD) disease is also known as sinus histiocytosis with massive lymphadenopathy (SHML) because of its characteristic lymph node involvement. It is exceedingly rare for RD to present with isolated, recurring laryngeal involvement without lymphadenopathy. This case presentation highlights this rare scenario, and difficulty in managing recidivistic laryngeal involvement. Method Case presentation with review of literature. Results A 45‐year‐old woman presented to our laryngology clinic with dysphonia from a recurring right hemilaryngeal mass. Prior removal and analysis showed inflammatory mass and work‐up for sarcoidosis and Wegner was negative. Videostroboscopy showed large right submucosal fullness of right true vocal fold and this was confirmed on imaging. She underwent removal of the mass and pathologic diagnosis remained inconclusive. After 6 months the mass recurred and repeat pathologic analysis was consistent with RD disease. After multiple recurrences requiring emergent intervention and limited response to steroids, radiation therapy was initiated. Conclusion Rosai‐Dorfman disease is a rare disease of massive lymphadenopathy and histiocytosis. The presentation with exclusive laryngeal involvement is rare, and due to extensive surrounding fibrosis and inflammation, histopathologic diagnosis was difficult. This case demonstrates that radiation therapy should be considered if surgical excisions and steroids are inadequate.