CLINICAL CASES   

Otorinolaringologia 1998 March;48(1):35-9

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Sinus histiocytosis with massive cervical lymphadenopathy (Rosai-Dorfman disease)

Ferrario F., Macchi A., Spriano G., Piantanida R., Bonato M.*

Ospedale di Circolo e Fondazione Macchi - Varese Divisione ORL (Primario Inc.: Dott. G. Spriano) * Servizio di Anatomia ed Istologia Patologica (Primario: Prof. C. Capella)

Sinus histiocytosis with massive lymphadenopathy (SHML) was firstly described by Rosai and Dorfman in 1969. It is considered an idiopathic disease which usually occurs with a slowly growing and asymptomatic lymphnodal mass, appearing in the cervical area in the 90% of the cases. From the histological standpoint it is characterized by a remarkable dilatation of the lymphatic sinuses due to the proliferation of some peculiar histiocytes. The Authors report a case of SHML involving all the neck lymph nodes and the nasopharynx, whose clinical patterns showed some difficulties in differentiating it from a lymphoma. A definitive lymphadenectomy was necessary for the final diagnosis while repeated biopsies of the mucosal localization did not allow a certain interpretation.