DOI: 10.36347/SJMCR.2019.v07i12.005

Pages: 740-742

Introduction: Rosai-Dorfman disease (RDD) is a rare atypical proliferation of histiocytes. RDD revealed by laryngeal involvement, as in the present case, is even less common. The objective of this presentation is to illustrate the clinical, para-clinical and therapeutic aspects of this rare localization through this clinical case and a review of the literature. Case report: A 40 years old woman is presented with a dysphonia of 4 year of evolution, aggravated by laryngeal dyspnea requiring emergency tracheotomy. Physical examination showed multiple cervical adenopathies, with a subglottic lesion covered with healthy mucosa. Consistent with the CT scan findings, the lesion caused a 90% obstruction. Histopathologic findings of the lesion biopsy revealed the diagnosis of RDD confirmed by immunohistochemical complement (positivity of PS100 and negativity of CD1a, and of CD207). The patient was put on chemotherapy based on vincristine. The evolution was marked by a clear regression of the subglottic obstacle; hence the decision to remove the tracheotomy cannula was taken. With a decline of 6 months, no recurrence was noted. Conclusion: The diagnosis of RDD should be kept in mind when evaluating sub-glottic tumor with cervical lymphadenopathy. A high degree of suspicion and a thorough pathological review are necessary to diagnose this rare clinical entity.