Department of Respiratory Medicine, Japanese Red Cross Medical Center, Tokyo, Japan.
Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan.
Jpn J Clin Oncol. 2022 May 31;52(6):531-538. doi: 10.1093/jjco/hyac033.
Nuclear protein of the testis (NUT) midline carcinoma (NMC) is a rare tumor that typically presents in the head, neck, and chest region. NMC is characterized by rearrangement of the NUTM1 gene. It mainly affects children and young adults and is rapidly progressive and lethal. Reportedly, the prognoses of NMCs of the head and neck improve following aggressive initial surgical resection +/- postoperative chemoradiotherapy (CRT) or radiotherapy (RT). However, as NMC of the thorax was identified later, treatments to improve its prognosis are yet to be identified. Our review reveals that NMC is an extremely rare cancer, and most patients remain undiagnosed. Furthermore, this review outlines the clinical characteristics of NMC of the thorax and the prospects for its treatment.
睾丸核蛋白(NUT)中线癌(NMC)是一种罕见的肿瘤,通常发生在头部、颈部和胸部区域。NMC 的特征是 NUTM1 基因的重排。它主要影响儿童和年轻人,且病情进展迅速,致命性高。据报道,头颈部 NMC 经积极的初始手术切除 +/-术后化疗放疗(CRT)或放疗(RT)后,预后改善。然而,由于胸内 NMC 的诊断较晚,改善其预后的治疗方法仍有待确定。我们的综述表明,NMC 是一种极其罕见的癌症,大多数患者仍未被诊断。此外,本综述概述了胸内 NMC 的临床特征及其治疗前景。