Sinonasal Nut Carcinoma: A Consecutive Case Series

 

Objective: NUT carcinoma is a rare and aggressive subtype of squamous cell carcinoma that can occur within the sinonasal tract. It is genetically characterized by a chromosomal rearrangement in the nuclear protein of testis (NUTM1). Patients with this tumor have poor outcomes, often with rapid progression and early metastasis. With less than 100 cases published, there is a paucity of literature on the presentation, management, and survival of these rare tumors. Previously reported overall survival is 7 to 9 months. The aim of this study is to inform clinical care for sinonasal NUT carcinoma.

Methods: A consecutive case series was performed of all sinonasal NUT carcinomas treated at a tertiary care hospital from 2010 to 2021. Patient demographics, risk factors, presenting symptoms, tumor characteristics, staging, and treatments are reported. The primary outcome is overall survival. The secondary outcomes include recurrence-free survival and factors associated with survival. The analysis is ongoing and will be fully reported in the presentation.

Results: The study included a total of 12 patients with sinonasal NUT carcinoma, who were 58.3% female and 41.6% White (25.0% Asian, 33.3% other) with a mean age at diagnosis of 40.2 years (median: 40.2, range: 12.4–57.6). 50% of patients initially received an alternative diagnosis. Primary treatment was induction chemotherapy with surgery and adjuvant radiation with or without chemotherapy (50.0%), surgery with adjuvant chemotherapy (16.7%), induction chemotherapy with radiation (8.0%), induction chemotherapy with chemoradiation (8.0%), and concurrent chemoradiation (8.0%). Overall survival was 13.5 months, and recurrence-free survival was 9.1 months. The median time-to-recurrence was 25.5 months (range, 3.5–79.9), and the median time to all-cause mortality was 45.5 months (range: 16.4–263). The 2-year rate of mortality was 58.3%, and the 2-year rate of recurrence was 75.0%.

Conclusion: This report is the largest single-institution case series of sinonasal NUT carcinoma. Half of the patients initially received an alternative diagnosis, and a significant majority of patients were treated with induction chemotherapy followed by surgery with adjuvant treatment or concomitant chemoradiation. Most patients experienced recurrence and mortality within two years. Overall survival was 13.5 months and recurrence-free survival was 9.1 months. Although analysis is ongoing, this overall survival is substantially greater than previous reports, suggesting that induction chemotherapy may improve outcomes for NUT carcinoma. Regardless, survival remains poor and targeted treatment is needed to improve outcomes ([Fig. 1]).

Publication History

Article published online:
15 February 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany