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Meta-Analysis
. 2012 Oct 15;18(20):5773-9.
doi: 10.1158/1078-0432.CCR-12-1153. Epub 2012 Aug 15.

Clinicopathologic features and long-term outcomes of NUT midline carcinoma

Daniel E Bauer  1 , Chelsey M Mitchell, Kelly M Strait, Christopher S Lathan, Edward B Stelow, Sonja C Lüer, Somala Muhammed, Andrew G Evans, Lynette M Sholl, Juan Rosai, Eugenia Giraldi, Richard P Oakley, Carlos Rodriguez-Galindo, Wendy B London, Stephen E Sallan, James E Bradner, Christopher A French
Affiliations
Meta-Analysis

Clinicopathologic features and long-term outcomes of NUT midline carcinoma

Daniel E Bauer et al. Clin Cancer Res. .

Abstract

Purpose: NUT midline carcinoma (NMC) is a poorly differentiated squamous cancer characterized by rearrangement of the NUT gene. Research advances have provided opportunities for targeted therapy in NMC, yet the clinical features of this rare disease have not been systematically characterized. We report on a large population of such patients to identify the disease characteristics and treatments, correlate them with outcome, and to consider clinical recommendations.

Experimental design: A clinical database was established using retrospective demographic and outcomes data available on all known cases of NMC. Questionnaires were completed by treating physicians. Pathologic, demographic, and clinical variables were assessed for 63 patients, the largest cohort of patients with NMC studied to date. Outcome data from 54 patients were available for survival analyses.

Results: The diagnosis of NMC has increased annually since 2007. Since 2009, there has been an observed increase in the age at diagnosis (P < 0.05). Geographic distribution of patients with NMC has been concentrated in the United States (n = 41, 65%). The median overall survival for patients with NMC was 6.7 months. The 2-year progression-free survival (PFS) was 9% with a 95% confidence interval (CI) of 1% to 17% [1-year PFS 15% (5-24%) and 2-year overall survival (OS) was 19% with a 95% CI of 7%-31% (1-year OS: 30% (27-34%)]. Multivariate analysis suggested that extent of surgical resection and initial radiotherapy were independent predictors of PFS and OS. Notably, no chemotherapeutic regimen was associated with improved outcome.

Conclusions: NMC portends a poor prognosis among all squamous cell neoplasms and seems to be frequently unrecognized. The finding that conventional chemotherapy has been inadequate indicates a pressing need for the development of targeted therapeutics. Intensive local therapies such as gross total resection and radiotherapy might be associated with enhanced survival.

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Conflict of interest statement

Disclosures:

The authors report no conflicts of interest.

Figures

Figure 1
Figure 1. Cases of NUT midline carcinoma in adult (n=18) and pediatric (n=22) patients per year of diagnosis
Cases are limited to those diagnosed in “real-time”, that is, at time of initial presentation.
Figure 2
Figure 2. Survival of patients with NMC
The probability of overall survival (OS) and progression free survival (PFS) is presented for (A) pediatric (age<18; n=29) and (B) adult (age≥18; n=25) NUT midline carcinoma patients.
Figure 3
Figure 3. Influence of therapeutic intervention on survival of patients with NMC
The probability of overall survival is presented for NMC patients, as influenced by use of (A) radiotherapy; and (B) surgical resection.
See this image and copyright information in PMC

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