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

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.