Extranodal Rosai-Dorfman Disease with Simultaneous Involvement of the Brain, Paranasal Sinus, and the Larynx: A Case Report

10.6966/EDMJ.202409_11(3).0003

Chia-Chi Chen；Chen-Chou Hsieh；Yu-Duan Tsai；Sung-Nan Pei；Ching-Feng Lien

Rosai-Dorfman disease ； non-neoplastic mass ； extranodal disease ； simultaneous multifocal involvement

E-Da Medical Journal

11卷3期（2024 / 09 / 01）

17 - 24

英文

Rosai-Dorfman disease (RDD) is a rare, idiopathic, and nonmalignant histiocytic proliferative disorder involving lymph nodes or extranodal organs. The most common organs involved are skin and subcutaneous tissue, followed by lymph nodes, bone, and head and neck. Some reports have suggested that extranodal involvement is present in approximately 40% of cases. The most frequently affected locations in the head and neck region are nasal cavity, pharynx, and paranasal sinuses. The diagnosis of extranodal RDD is difficult, because its clinical symptom is uncommon or mild. The treatment of symptomatic RDD patients can involve surgical resection, radiotherapy, high-dose steroid, and chemotherapy. Extranodal RDD with simultaneous multifocal involvement is rare and its treatment remains challenging. Therefore, we report a case of extranodal RDD with a simultaneous involvement of the brain, paranasal sinus, and the larynx because it is extremely rare. The clinical, histopathological and immunohistochemical features, and treatment modalities are discussed.