作者: Nicole G. Chau , Shelley Hurwitz , Chelsey M. Mitchell , Alexandra Aserlind , Noam Grunfeld
DOI: 10.1002/CNCR.30242
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摘要: BACKGROUND NUT midline carcinoma is a rare and aggressive genetically characterized subtype of squamous cell frequently arising from the head neck. The characteristics optimal management neck NUT (HNNMC) are unclear. METHODS A retrospective review all known cases HNNMC in International Midline Carcinoma Registry as December 31, 2014, was performed. Forty-eight consecutive patients were treated 1993 to clinicopathologic variables outcomes for 40 available analyses; they composed largest cohort studied date. Overall survival (OS) progression-free (PFS) according patient treatment analyzed. RESULTS This study identified 5-fold increase diagnosis 2011 2014. median age 21.9 years (range, 0.1-81.7 years); male female proportions 40% 60%, respectively; 86% had bromodomain containing 4–nuclear protein testis (BRD4-NUT) fusion. initial surgery with or without adjuvant chemoradiation radiation (56%), chemotherapy (15%), (28%). PFS 6.6 months 4.7-8.4 months). OS 9.7 6.6-15.6 2-year rate 26% (95% confidence interval [CI], 13%-40%). 30% CI, 16%-46%). Initial postoperative (P = .04) complete resection negative margins (P = .01) significant predictors improved even after adjustments age, tumor size, lymphadenopathy. translocation type not associated outcomes. CONCLUSIONS HNNMC portends poor prognosis. Aggressive surgical significantly enhanced survival. Chemotherapy alone often inadequate. Cancer 2016;122:3632-40. © 2016 American Society.