Rosai-Dorfman disease with infiltration of IgG4-bearing plasma cells presenting as laryngeal-nasal masses and cervical lymphadenopathy A case report

Abstract

Rationale: Rosai-Dorfman disease (RDD) is a rare and self-limiting condition caused by the non-neoplastic proliferation of histiocytes/phagocytes in the sinusoids of lymph nodes and in extranodal tissues. Of the extranodal involvement, laryngeal involvement is extremely rare. Because of its rarity and nonspecific clinicoradiologic features, RDD is often difficult to differentiate from other benign or malignant lymphoproliferative diseases. We present a case of RDD with infiltration of IgG4-bearing plasma cells manifesting laryngeal and nasal masses with cervical lymphadenopathy. Patient concerns: A 45-year-old male patient presented with recurrent epistaxis and airway disturbance. Diagnoses: On endoscopy, there were submucosal masses in both nasal cavities and both sides of subglottic larynx. On neck CT, there were well-defined, enhancing soft tissue masses in both nasal cavities and both sides of subglottic larynx, resulting in mild airway narrowing. In addition, multiple enlarged lymph nodes showing homogeneous enhancement were noted in both parotid glands and both internal jugular chains. All lesions demonstrated marked FDG-uptake on PET/CT. Therefore, the initial radiologic differential diagnoses included lymphoma and IgG4-related disease. Biopsy was performed on the nasal and laryngeal lesions, and they revealed RDD with infiltration of IgG4-bearing plasma cells. Intervention: The patient underwent surgical resection of the masses in the nasal cavity and larynx to relieve airway narrowing. Outcomes: After surgery, airway obstruction was much improved and the patient was asymptomatic. On outpatient follow-up, he exhibited a stable condition and had no dyspnea on exercise. Lessons: Clinical awareness and suspicion are important for the accurate diagnosis and management of patients with homogeneous masses in the larynx or nasal cavity, even if there is no combined cervical lymphadenopathy.