Sinonasal NUT Carcinoma: A Systematic Review With Pooled Analysis and Report of a Case of Extraordinary Survival.

Abstract

Introduction: NUT carcinoma (NC) is a rare and aggressive malignancy often occurring in the midline structures of the head and neck. In this study, we present a systematic review and pooled analysis on patients specifically with sinonasal NC (SNC), as well as report a case of a patient with extraordinary survival following presentation with an NC of the maxillary sinus.

Methods: Among 3042 articles retrieved, 27 were included in the final analysis. Clinical outcomes were described for a patient presenting with SNC at a tertiary academic medical center. Predictors of survival were evaluated with Kaplan-Meier (KM) and Cox regression analyses.

Results: There were 45 patients with a relatively even gender distribution. The most common presenting symptoms were nasal obstruction (54%) and headache/facial pain (49%). Common primary sites of malignancy were the nasal cavity (50%), maxillary sinus (22%), and ethmoid sinus (16%). In terms of treatment, 31% underwent oncologic resection, 64% received radiation therapy, and 71% received chemotherapy. The overall cohort survival rate was 40% with an average survival of 12.4 months. The presence of nerve palsies (OR = 16.6, p < 0.001) was a substantial negative predictor for survival. Oncological resection with negative margins was associated with improved survival on KM analysis (p < 0.05). We also report a case of a 47-year-old female who presented with a left maxillary SNC with extra-sinus extension. She underwent a combined endoscopic/open oncologic resection with negative margins followed by chemoradiation. The patient remains alive 52 months after presentation.

Conclusion: SNC remains an understudied clinical entity. Oncologic resection with negative margins is associated with improved survival. The presence of nerve palsies worsens overall survival. Further, large-scale studies are needed to determine optimal treatment protocols.