Nasal septal tumor as a sole presentation in the head and neck region in Rosai-Dorfman disease

Abstract

From the Departments of Otolaryngology (Drs. Sheen and Ko), Pathology (Drs. Chang and Wu), and Oncology (Dr. Yeh), National Taiwan University Hospital. Reprint requests: Yih-Leong Chang, MD, Department of Pathology, National Taiwan University Hospital, No. 7, Chung-Shan South Rd., Taipei, Taiwan. Otolaryngol Head Neck Surg 1998;118:408-11. Copyright © 1998 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/98/$5.00 + 0 23/78/77260 Sinus histiocytosis with massive lymphadenopathy was first reported by Rosai and Dorfman1, 2 in 1969. Until 1990, 423 patients were enrolled in the registry.3 These patients usually had bilateral, painless, and extensive cervical lymphadenopathy. The involved lymph nodes showed pericapsular fibrosis with abundant histiocytes. These histiocytes were large and contained vesicular nuclei, delicate nuclear membrane, distinct nucleoli, voluminous pale cytoplasm, and often with phagocytosed lymphocytes. This phenomenon was referred to as emperipolesis.4, 5 This disease was usually benign and was differentiated from some malignant diseases such as malignant lymphoma or Hodgkin’s disease. Approximately 40% of the patients also had extranodal involvement.3 Moreover, extranodal involvement was sometimes the initial and sole manifestation of the disorder. The condition was similar to nodal disease with typical histiocytes but without the nodal capsule or subcapsular sinusoid. A sole extranodal involvement in the head and neck region was occasionally reported in the literature.6-15 A 15-year-old boy was referred to us because of nasal obstruction and nasal bleeding. A nasal mass was found in his right nasal cavity. RosaiDorfman disease was proved after pathologic examination of this nasal mass was performed.