Primary pulmonary nuclear protein of the testis(NUT)midline carcinoma(NMC)is a rare and highly aggressive thoracic malignancy that poses significant diagnostic and therapeutic challenges in clinical practice.This tumo...Primary pulmonary nuclear protein of the testis(NUT)midline carcinoma(NMC)is a rare and highly aggressive thoracic malignancy that poses significant diagnostic and therapeutic challenges in clinical practice.This tumor is characterized by its heterogeneous clinical presentations and poor prognosis,often evading accurate initial diagnosis.In this study,we present two cases of primary pulmonary NMC treated with an integrated therapeutic approach combining anti-angiogenic agents,platinum-based chemotherapy,and radiotherapy.This multimodal strategy achieved survival durations of 32 and 13 months,respectively,surpassing the currently reported median survival of advanced NMC.Through a systematic literature review of reported cases,we have summarized the currently used diagnostic methods and treatment modalities for NMC.Our findings suggest that multimodal therapy incorporating anti-angiogenic treatment may offer superior clinical outcomes compared to conventional monotherapy regimens,particularly for patients who are not eligible for surgery.This comprehensive investigation enhances our understanding of NMC management by elucidating diagnostic pitfalls through histopathological correlation and proposing an effective therapeutic combination that demonstrates improved survival outcomes.By providing valuable insights into the diagnosis and treatment of primary pulmonary NMC,we hope to contribute to the development of more effective strategies for managing this rare and aggressive malignancy.展开更多
1文献来源FRENCH C A,KUTOK J L,FAQUIN W C,et al.Midline carcinoma of children and young adults with NUT rearrangement[J].J Clin Oncol,2004,22(20):4135-4139.doi:10.1200/JCO.2004.02.107.2研究背景既往病例报道描述了一种罕...1文献来源FRENCH C A,KUTOK J L,FAQUIN W C,et al.Midline carcinoma of children and young adults with NUT rearrangement[J].J Clin Oncol,2004,22(20):4135-4139.doi:10.1200/JCO.2004.02.107.2研究背景既往病例报道描述了一种罕见的癌症--NUT癌,它常发生在年轻人中并具有t(15;19)染色体易位的特征,该特征似乎与高致死性的临床进程相关。特殊的染色体易位是其独特标识,但大多数癌具有高度的同源性及多重核型,诊断性染色体平衡易位较缺乏。就细胞遗传学特征而言,NUT癌更类似于淋巴瘤及肉瘤。仅有的文献表明,该癌肿属于主要起源于胸腺或呼吸道上皮的实体瘤,对放化疗无明显效应,具有高度致命性,但这种疾病的临床病理特征仍不明确,需要进一步研究探索。展开更多
NUT癌是一种极为罕见的恶性肿瘤,与15q14号染色体上的NUT癌家族成员1(NUT midline carcinoma family member 1,NUTM1)基因重排密切相关,其中以BRD4::NUTM1融合基因多见[1]。NUT癌侵袭性高、分化程度低,往往具有较差的生存及预后。由于...NUT癌是一种极为罕见的恶性肿瘤,与15q14号染色体上的NUT癌家族成员1(NUT midline carcinoma family member 1,NUTM1)基因重排密切相关,其中以BRD4::NUTM1融合基因多见[1]。NUT癌侵袭性高、分化程度低,往往具有较差的生存及预后。由于其与鳞状细胞癌的同源相似性,存在较高的误诊率。诸多患者在诊断时已发现疾病处于晚期阶段[2]。目前,以免疫组化(immunohistochemistry,IHC)初筛为主,荧光原位杂交(fluorescence in situ hybridization,FISH)、二代测序(next-generation sequencing,NGS)等为辅的检测手段大大提高了NUT癌的检出率[3]。展开更多
1文献来源LEWIN J,SORIA J C,STATHIS A,et al.PhaseⅠb trial with birabresib,a small-molecule inhibitor of bromodomain and extraterminal proteins,in patients with selected advanced solid tumors[J].J Clin Oncol,2018,36(30...1文献来源LEWIN J,SORIA J C,STATHIS A,et al.PhaseⅠb trial with birabresib,a small-molecule inhibitor of bromodomain and extraterminal proteins,in patients with selected advanced solid tumors[J].J Clin Oncol,2018,36(30):3007−3014.doi:10.1200/JCO.2018.78.2292.2证据水平1b。3研究背景伴睾丸核蛋白(nuclear protein in testis,NUT)基因重排的中线癌又称NUT癌,是一种罕见的具有高度侵袭性的鳞状癌。它的发病与位于15号染色体长臂的NUT中线癌家族成员1基因(NUT midline carcinoma family member 1,NUTM1)重排有关。展开更多
1文献来源PIHA-PAUL S A,HANN C L,FRENCH C A,et al.Phase 1 study of molibresib(GSK525762),a bromodomain and extra-terminal domain protein inhibitor,in NUT carcinoma and other solid tumors[J].JNCI Cancer Spectr,2019,4(2)...1文献来源PIHA-PAUL S A,HANN C L,FRENCH C A,et al.Phase 1 study of molibresib(GSK525762),a bromodomain and extra-terminal domain protein inhibitor,in NUT carcinoma and other solid tumors[J].JNCI Cancer Spectr,2019,4(2):pkz093.doi:10.1093/jncics/pkz093.2研究背景溴结构域和超末端结构域(bromodomain and extraterminal domain,BET)家族是表观基因组的调节因子,也是肿瘤细胞生存所依赖的肿瘤相关基因表达的关键驱动因子,一些重要的原癌基因如MYC、BCL2、CDK6均受到BET蛋白的调控,BET蛋白的异常激活与包括癌症在内的各种人类疾病密切相关,包括罕见且具有高度侵袭性的NUT癌。展开更多
Activated carbons (ACs) calcined at 400˚C, 500˚C, and 600˚C (AC-400, AC-500, and AC-600) were prepared using palm nut shells from Gabon as raw material and zinc chloride (ZnCl2) as a chemical activating agent. Prepare...Activated carbons (ACs) calcined at 400˚C, 500˚C, and 600˚C (AC-400, AC-500, and AC-600) were prepared using palm nut shells from Gabon as raw material and zinc chloride (ZnCl2) as a chemical activating agent. Prepared ACs were characterized by physisorption of nitrogen (N2), determination of diode and methylene blue numbers for studies of porosity and by quantification and determination of surface functional groups and pH at point of zero charge (pHpzc) respectively, for studies of chemical properties of prepared ACs. Then, effects of calcination temperature (Tcal) on porosity and chemical properties of prepared ACs were studied. The results obtained showed that when the calcination temperature increases from 500˚C to 600˚C, the porosity and chemical properties of prepared ACs are modified. Indeed, the methylene blue and iodine numbers determined for activated carbons AC-400 (460 and 7.94 mg·g−1, respectively) and AC-500 (680 and 8.90 mg·g−1, respectively) are higher than those obtained for AC-600 (360 and 5.75 mg·g−1, respectively). Compared to the AC-500 adsorbent, specific surface areas (SBET) and microporous volume losses for AC-600 were estimated to 44.7% and 45.8%, respectively. Moreover, in our experimental conditions, the effect of Tcal on the quantities of acidic and basic functional groups on the surface of the ACs appears negligible. In addition, results of the pHpzc of prepared ACs showed that as Tcal increases, the pH of the adsorbents increases and tends towards neutrality. Indeed, a stronger acidity was determined on AC-400 (pHpzc = 5.60) compared to those on AC-500 and AC-600 (pHpzc = 6.85 and 6.70, respectively). Also according to the results of porosity and chemical characterizations, adsorption being a surface phenomenon, 500˚C appears to be the optimal calcination temperature for the preparation of activated carbons from palm nut shells in our experimental conditions.展开更多
文摘Primary pulmonary nuclear protein of the testis(NUT)midline carcinoma(NMC)is a rare and highly aggressive thoracic malignancy that poses significant diagnostic and therapeutic challenges in clinical practice.This tumor is characterized by its heterogeneous clinical presentations and poor prognosis,often evading accurate initial diagnosis.In this study,we present two cases of primary pulmonary NMC treated with an integrated therapeutic approach combining anti-angiogenic agents,platinum-based chemotherapy,and radiotherapy.This multimodal strategy achieved survival durations of 32 and 13 months,respectively,surpassing the currently reported median survival of advanced NMC.Through a systematic literature review of reported cases,we have summarized the currently used diagnostic methods and treatment modalities for NMC.Our findings suggest that multimodal therapy incorporating anti-angiogenic treatment may offer superior clinical outcomes compared to conventional monotherapy regimens,particularly for patients who are not eligible for surgery.This comprehensive investigation enhances our understanding of NMC management by elucidating diagnostic pitfalls through histopathological correlation and proposing an effective therapeutic combination that demonstrates improved survival outcomes.By providing valuable insights into the diagnosis and treatment of primary pulmonary NMC,we hope to contribute to the development of more effective strategies for managing this rare and aggressive malignancy.
文摘1文献来源FRENCH C A,KUTOK J L,FAQUIN W C,et al.Midline carcinoma of children and young adults with NUT rearrangement[J].J Clin Oncol,2004,22(20):4135-4139.doi:10.1200/JCO.2004.02.107.2研究背景既往病例报道描述了一种罕见的癌症--NUT癌,它常发生在年轻人中并具有t(15;19)染色体易位的特征,该特征似乎与高致死性的临床进程相关。特殊的染色体易位是其独特标识,但大多数癌具有高度的同源性及多重核型,诊断性染色体平衡易位较缺乏。就细胞遗传学特征而言,NUT癌更类似于淋巴瘤及肉瘤。仅有的文献表明,该癌肿属于主要起源于胸腺或呼吸道上皮的实体瘤,对放化疗无明显效应,具有高度致命性,但这种疾病的临床病理特征仍不明确,需要进一步研究探索。
文摘NUT癌是一种极为罕见的恶性肿瘤,与15q14号染色体上的NUT癌家族成员1(NUT midline carcinoma family member 1,NUTM1)基因重排密切相关,其中以BRD4::NUTM1融合基因多见[1]。NUT癌侵袭性高、分化程度低,往往具有较差的生存及预后。由于其与鳞状细胞癌的同源相似性,存在较高的误诊率。诸多患者在诊断时已发现疾病处于晚期阶段[2]。目前,以免疫组化(immunohistochemistry,IHC)初筛为主,荧光原位杂交(fluorescence in situ hybridization,FISH)、二代测序(next-generation sequencing,NGS)等为辅的检测手段大大提高了NUT癌的检出率[3]。
文摘1文献来源LEWIN J,SORIA J C,STATHIS A,et al.PhaseⅠb trial with birabresib,a small-molecule inhibitor of bromodomain and extraterminal proteins,in patients with selected advanced solid tumors[J].J Clin Oncol,2018,36(30):3007−3014.doi:10.1200/JCO.2018.78.2292.2证据水平1b。3研究背景伴睾丸核蛋白(nuclear protein in testis,NUT)基因重排的中线癌又称NUT癌,是一种罕见的具有高度侵袭性的鳞状癌。它的发病与位于15号染色体长臂的NUT中线癌家族成员1基因(NUT midline carcinoma family member 1,NUTM1)重排有关。
文摘1文献来源PIHA-PAUL S A,HANN C L,FRENCH C A,et al.Phase 1 study of molibresib(GSK525762),a bromodomain and extra-terminal domain protein inhibitor,in NUT carcinoma and other solid tumors[J].JNCI Cancer Spectr,2019,4(2):pkz093.doi:10.1093/jncics/pkz093.2研究背景溴结构域和超末端结构域(bromodomain and extraterminal domain,BET)家族是表观基因组的调节因子,也是肿瘤细胞生存所依赖的肿瘤相关基因表达的关键驱动因子,一些重要的原癌基因如MYC、BCL2、CDK6均受到BET蛋白的调控,BET蛋白的异常激活与包括癌症在内的各种人类疾病密切相关,包括罕见且具有高度侵袭性的NUT癌。
文摘Activated carbons (ACs) calcined at 400˚C, 500˚C, and 600˚C (AC-400, AC-500, and AC-600) were prepared using palm nut shells from Gabon as raw material and zinc chloride (ZnCl2) as a chemical activating agent. Prepared ACs were characterized by physisorption of nitrogen (N2), determination of diode and methylene blue numbers for studies of porosity and by quantification and determination of surface functional groups and pH at point of zero charge (pHpzc) respectively, for studies of chemical properties of prepared ACs. Then, effects of calcination temperature (Tcal) on porosity and chemical properties of prepared ACs were studied. The results obtained showed that when the calcination temperature increases from 500˚C to 600˚C, the porosity and chemical properties of prepared ACs are modified. Indeed, the methylene blue and iodine numbers determined for activated carbons AC-400 (460 and 7.94 mg·g−1, respectively) and AC-500 (680 and 8.90 mg·g−1, respectively) are higher than those obtained for AC-600 (360 and 5.75 mg·g−1, respectively). Compared to the AC-500 adsorbent, specific surface areas (SBET) and microporous volume losses for AC-600 were estimated to 44.7% and 45.8%, respectively. Moreover, in our experimental conditions, the effect of Tcal on the quantities of acidic and basic functional groups on the surface of the ACs appears negligible. In addition, results of the pHpzc of prepared ACs showed that as Tcal increases, the pH of the adsorbents increases and tends towards neutrality. Indeed, a stronger acidity was determined on AC-400 (pHpzc = 5.60) compared to those on AC-500 and AC-600 (pHpzc = 6.85 and 6.70, respectively). Also according to the results of porosity and chemical characterizations, adsorption being a surface phenomenon, 500˚C appears to be the optimal calcination temperature for the preparation of activated carbons from palm nut shells in our experimental conditions.
