Post-treatment sequelae and management of primary laryngeal NK/T-cell lymphoma: A case report

METHODS: Case Report, April 2016.

A 27-year-old female presented with a three-week history of flu-like symptoms including sore throat, myalgia and insidious nasal congestion. Her hospital course was complicated by pancytopenia, extensive GI bleed of unknown origin and fever. Radiographic studies revealed multifocal centrilobular ground-glass lung nodules, splenomegaly, frontal and paranasal sinus opacification and mucosal thickening and inflammatory process of false and true vocal cords with concentric multi-level narrowing. Extensive rheumatologic, hematologic and infectious workup failed to demonstrate an etiology. Flexible laryngoscopic and bronchoscopic examinations with biopsies exhibited a diffuse supraglottic and glottic exophytic, "heaped-up" infiltration; limited vocal cord motion; and airway narrowing to 3 mm. Pathology yielded NK/T-Cell lymphoma with PET/CT confirming nasopharyngeal and laryngeal involvement sparing the brain and other distant structures. During treatment, the patient's airway became increasingly compromised requiring tracheostomy. Flexible laryngoscopic examination after four rounds of SMILE (dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide) chemotherapy and subsequent radiotherapy with 50 Gy in 25 fractions revealed complete glottic stenosis with alteration of the laryngeal anatomy and a pin-hole aperture seen posteriorly through the tracheostomy. Two dilation procedures performed one month apart, improved her airway considerably which allowed for successful decannulation. She continues to be disease-free post treatment but has persistent supraglottic and glottic scar and has developed tracheal stenosis at her tracheostomal site.

NK/T-Cell lymphoma of the larynx has been rarely described. Herein, we describe the sequalae of treatment and the requisite airway management.