Interdigitating dendritic cell sarcoma of the supraglottic larynx

Abstract Background Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm with sparse published literature, limited to case reports. This neoplasm arises from dendritic cells mainly within lymph nodes, however extranodal localization has been noted in the head and neck. Methods Herein we present the first report of IDCS of the supraglottic larynx. A 35-year-old woman presented with odynophagia and dysphagia and was found to have an epiglottic primary lesion with bilateral regional lymphadenopathy. She was treated with transoral laser supraglottic laryngectomy, bilateral neck dissections, and postoperative radiation therapy. Results Patient has no evidence of disease with 4 years of follow-up. Conclusion Surgical resection with postoperative radiation therapy is a reasonable approach for management of loco-regionally advanced extranodal IDCS of the head and neck.