Nationwide retrospective study on the prevalence and prognostic factors of NUT carcinoma in the United States.

Abstract

Background: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and exceedingly aggressive neoplasm, typically presenting as midline tumors such as mediastinal, thoracic, or sinonasal tumors, but can also be seen in other locations like the kidneys, liver, and pancreas. NC is frequently underdiagnosed or misdiagnosed due to its rarity, nonspecific clinical and histopathological presentation, lack of clinically trialed standard-of-care therapy, and, ultimately, a lack of awareness about the condition.

Methods: We retrospectively reviewed 45 cases of NC from the Surveillance, Epidemiology, and End Results (SEER) database.

Results: The patient pool was predominantly male, with the most common tumor sites being the lungs and bronchus (51.1%), followed by the nasal cavity and paranasal sinuses (28.9%).73.3% of patients received systemic therapy, 62.2% received radiotherapy, and only one-quarter of patients underwent surgery. The median survival time was 8 months (95% CI: 4.09-11.91). Systemic therapy (aHR = 0.268, P = 0.009) and radiotherapy (aHR = 0.335, P = 0.02) were the only independent prognostic factors in this cohort.

Conclusion: Our study reiterates the rarity of NC in the United States. We also reveal that when faced with the rare NC, systemic therapy and radiotherapy have been found to relatively prolong NC patient survival. Future research is needed to further guide NC treatment and improve the poor survival associated with the diagnosis.