Clinicopathologic Features and Long-term Outcomes of NUT Midline Carcinoma

摘要: Purpose: NUT midline carcinoma (NMC) is a poorly differentiated squamous cancer characterized by rearrangement of the gene. Research advances have provided opportunities for targeted therapy in NMC, yet clinical features this rare disease not been systematically characterized. We report on large population such patients to identify characteristics and treatments, correlate them with outcome, consider recommendations. Experimental Design: A database was established using retrospective demographic outcomes data available all known cases NMC. Questionnaires were completed treating physicians. Pathologic, demographic, variables assessed 63 patients, largest cohort NMC studied date. Outcome from 54 survival analyses. Results: The diagnosis has increased annually since 2007. Since 2009, there an observed increase age at ( P n = 41, 65%). median overall 6.7 months. 2-year progression-free (PFS) 9% 95% confidence interval (CI) 1% 17% [1-year PFS 15% (5–24%) (OS) 19% CI 7%–31% (1-year OS: 30% (27–34%)]. Multivariate analysis suggested that extent surgical resection initial radiotherapy independent predictors OS. Notably, no chemotherapeutic regimen associated improved outcome. Conclusions: portends poor prognosis among cell neoplasms seems be frequently unrecognized. finding conventional chemotherapy inadequate indicates pressing need development therapeutics. Intensive local therapies as gross total might enhanced survival. Clin Cancer Res; 18(20); 5773–9. ©2012 AACR .