Advancing Education, Research, and Quality of Care for the Head and Neck oncology patient.
INTRODUCTION: Head and neck NUT carcinoma (HNNC) is an uncommon but devastating malignancy with a very poor prognosis. Unfortunately, there is no standardized treatment protocol for this disease, and many patients ultimately experience disease-specific mortality within a year of diagnosis. The aim of this study is to investigate the demographics, clinical presentation, treatment, outcomes, and potential prognostic indicators in patients with HNNC.
METHODS: A systematic literature search yielded 3042 articles, and a total of 70 published articles were included in this study. Demographic, clinical, diagnostic, treatment, and patient outcome data was collected, and prognostic variables were evaluated using Cox proportional hazards regression (CR) and Kaplan-Meier (KM) survival analyses.
RESULTS: A total of 106 cases of HNNC were included in this study. Sex was evenly distributed. The average age at presentation was 36 years ranging from 9 months to 82 years. The most common presenting symptoms were mass/swelling (47.2%), pain (35%), and nasal symptoms (24.5%). The most common primary sites of malignancy were sinonasal (49.1%) and salivary gland (17%). Of the cases with reported diagnostic testing, less than one-third (??27.6%) of cases were diagnosed using NUT IHC alone, while 70.5% of patients underwent both NUT IHC and FISH/NGS to identify the specific mutation. The majority of patients underwent surgery (70.8%), radiation (75.5%), and/or chemotherapy (64%), while only 9.4% of patients received some form of immunotherapy. Mean length of survival was 13.8 months ± 19.9, with a 1-year overall survival (OS) rate of 38% and a 2-year OS rate of 9.8%.
Females were almost twice as likely to experience death compared to males (OR=1.9, p=0.02; KM p=0.02). Those who developed distant metastasis after initial presentation also had a significantly lower likelihood of survival (OR=1.8, p=0.03; KM p=0.03). Surgical resection with negative margins improved survival by 67.7% (OR=0.3, p=0.02; KM p=0.01), and radiation therapy may also be a positive prognostic factor but did not reach statistical significance (p=0.08). Patients who were found to have a non BRD4-NUTM1 mutation also had significantly better outcomes compared to those with the BRD4-NUTM1 mutation (KM p=0.049). Analysis of the remaining variables, including age, cancer site, and staging had no significant impact on survival.
CONCLUSION: HNNC is an aggressive malignancy that is difficult to treat given the current gaps in the literature. Female sex and distant metastasis after diagnosis were found to be negative predictors of survival. Oncologic resection with negative margins was associated with improved survival. No other treatment modality or combination thereof demonstrated a significant impact on survival, highlighting the need for investigation into more optimal treatment approaches for these patients.