Primary Natural Killer T Cell Lymphoma of the Supraglottis

Abstract

Objective: Describe an especially rare case, primary natural killer (NK) T Cell lymphoma of the supraglottis. Because of its unique presentation and infrequent occurrence, treatment in this case was delayed while awaiting a definitive diagnosis. Presentation of this case and the review of the literature should aid in the differential diagnosis. Method: The patient’s medical records including his clinical history, laboratory, radiologic, and pathologic data were reviewed. Additionally, a literature search was performed for cases of laryngeal lymphoma, with special attention to NK T-cell type. Results: A 22-year old male from South America presented with 2 months of odynophagia, hoarseness, fever, hemoptysis, night sweats, and a 70-pound weight loss. Work up for tuberculosis, HIV, and serologies for systemic granulomatous conditions were negative. Otolaryngologic evaluation demonstrated normal-appearing nasal mucosa and oral cavity. Laryngoscopy revealed a thickened epiglottis with polypoid mucosa of the vallecula and the supraglottis. Biopsy of the epiglottis demonstrated NK T cell lymphoma, nasal-type with CD3 and CD56 positivity and histologic features of an aggressive EBV-associated form. Imaging and bone marrow biopsy confirmed its confinement to the supraglottis. He is currently undergoing concurrent chemo-radiation therapy. Conclusion: Lymphoma of the larynx represents 1% of malignant laryngeal tumors. Primary laryngeal NK T cell lymphoma with disease isolated to the larynx is exceptionally rare and has not previously been reported. Medical work up to rule out infectious or inflammatory lesions along with directed biopsies should help in the diagnosis.