Intensive treatment and survival outcomes in NUT midline carcinoma of the head and neck.
Intensive treatment and survival outcomes in NUT midline carcinoma of the head and neck.
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DOI：
10.1002/cncr.30242
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发表时间：
2016-12-01
期刊：
影响因子：
6.2
通讯作者：
French CA
中科院分区：
文献类型：
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作者：
Chau NG;Hurwitz S;Mitchell CM;Aserlind A;Grunfeld N;Kaplan L;Hsi P;Bauer DE;Lathan CS;Rodriguez-Galindo C;Tishler RB;Haddad RI;Sallan SE;Bradner JE;French CA
NUT中线癌（NMC）是一种罕见的侵袭性鳞状细胞癌亚型，常发生于头颈部（HN）。HNNMC的特点和最佳管理尚不清楚。我们对国际NMC登记研究中所有已知的HNNMC病例进行了回顾性分析，数据截至2014年12月31日。在1993-2014年接受治疗的48例连续患者中，40例患者的临床病理变量和结局可用于分析，这是迄今为止研究的最大的HN NMC队列。根据患者特征和治疗分析总生存期（OS）和无进展生存期（PFS）。我们发现，从2011年到2014年，HNNMC的诊断增加了五倍。中位年龄为21.9岁（范围0.1-81.7岁），男性：女性为40%：60%，86%接受BRD 4-NUT融合。初始治疗为初始手术（S）+/−辅助放化疗（CRT）或辅助放疗（RT）（56%）、初始RT +/−化疗（C）（15%）或初始C +/− S或RT（28%）。中位PFS为6.6个月（范围4.7-8.4）。中位OS为9.7个月（范围6.6-15.6）。2年PFS为26%（95% CI，13%-40%）。2年OS为30%（95% CI，16%-46%）。初始S +/−术后CRT或RT（p=0.04）和切缘阴性的完全切除（p=0.01）是OS改善的重要预测因素，即使在调整年龄、肿瘤大小和颈部淋巴结肿大后。初始RT或C和NUT易位类型与结果无关。HNNMC预示预后不良。积极的初始手术切除+/−术后CRT或RT与生存率显著提高相关。C或RT本身往往是不够的。
NUT midline carcinoma (NMC) is a rare and aggressive genetically characterized subtype of squamous cell carcinoma frequently arising from the head and neck (HN). HNNMC characteristics and optimal management are unclear. We performed a retrospective review of all known cases of HNNMC in the International NMC Registry, data as of December 31, 2014. Of 48 consecutive patients treated from 1993–2014, clinicopathologic variables and outcomes from 40 patients were available for analyses, the largest cohort of HN NMC studied to date. Overall survival (OS) and progression-free survival (PFS) according to patient characteristics and treatment were analyzed. We identified a five-fold increase in diagnosis of HNNMC from 2011 to 2014. Median age was 21.9 years (range 0.1–81.7), male:female was 40%:60%, and 86% had BRD4-NUT fusion. Initial treatment was initial surgery (S) +/− adjuvant chemoradiation (CRT) or adjuvant radiation (RT) (56%), initial RT +/− chemotherapy (C) (15%), or initial C +/− S or RT (28%). Median PFS was 6.6 months (range 4.7–8.4). Median OS was 9.7 months (range 6.6–15.6). Two-year PFS was 26% (95% CI, 13%–40%). Two-year OS was 30% (95% CI, 16%–46%). Initial S +/− post-operative CRT or RT (p=0.04), and complete resection with negative margins (p=0.01) were significant predictors of improved OS even after adjustment for age, tumor size and neck lymphadenopathy. Initial RT or C, and NUT translocation type were not associated with outcome. HNNMC portends a poor prognosis. Aggressive initial surgical resection +/− post-operative CRT or RT was associated with significantly enhanced survival. C or RT alone is often inadequate.