BACKGROUND: Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare benign disease with nodal and extranodal involvement. METHODS AND RESULTS: Herein, we report 3 cases in which the patients presented with nasal masses. In addition, 2 of the 3 patients had subglottic lesions. Only 2 of the 3 patients had cervical lymphadenopathy, which is the commonest presentation of RDD. Histopathology via fine-needle aspiration cytology (FNAC) or open biopsy is required for diagnosis. Emperipolesis with S-100 immunohistochemical staining is characteristic. The disease runs a benign course and the literature reports that treatment can vary from steroids, to cytotoxic chemotherapy to radiotherapy with variable outcomes. Surgical intervention may be necessary for airway protection. CONCLUSION: RDD is self-limiting, but can sometimes be a life-threatening condition. Treatment should be tailored to the individual patient.
BACKGROUND: Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare benign disease with nodal and extranodal involvement. METHODS AND RESULTS: Herein, we report 3 cases in which the patients presented with nasal masses. In addition, 2 of the 3 patients had subglottic lesions. Only 2 of the 3 patients had cervical lymphadenopathy, which is the commonest presentation of RDD. Histopathology via fine-needle aspiration cytology (FNAC) or open biopsy is required for diagnosis. Emperipolesis with S-100 immunohistochemical staining is characteristic. The disease runs a benign course and the literature reports that treatment can vary from steroids, to cytotoxic chemotherapy to radiotherapy with variable outcomes. Surgical intervention may be necessary for airway protection. CONCLUSION: RDD is self-limiting, but can sometimes be a life-threatening condition. Treatment should be tailored to the individual patient.
Authors: Junu Ojha; Yeshwant B Rawal; Jason L Hornick; Kelly Magliocca; David R Montgomery; Robert D Foss; Kevin R Torske; Brent Accurso Journal: Head Neck Pathol Date: 2019-07-31
Authors: Nishanth S Iyengar; Danielle Golub; Michelle W McQuinn; Travis Hill; Karen Tang; Sharon L Gardner; David H Harter; Chandranath Sen; David A Staffenberg; Kristen Thomas; Zachary Elkin; Irina Belinsky; Christopher William Journal: Acta Neuropathol Commun Date: 2020-07-18 Impact factor: 7.801