怎么样尽早发掘神经母细胞瘤:症状和体征
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤发病初期,<span style="color: black;">孩儿</span><span style="color: black;">通常</span><span style="color: black;">无</span><span style="color: black;">尤其</span><span style="color: black;">显著</span>的症状。家长可能<span style="color: black;">发掘</span><span style="color: black;">孩儿</span>的肚子鼓了一点,<span style="color: black;">或</span>皮肤上有<span style="color: black;">有些</span>瘀青——<span style="color: black;">一般</span><span style="color: black;">来讲</span>,这些都是成长中很<span style="color: black;">平常</span>的。正因如此,<span style="color: black;">非常多</span>家长直到症状非常<span style="color: black;">显著</span>的时候才带<span style="color: black;">孩儿</span>去医院<span style="color: black;">检测</span>,<span style="color: black;">错失</span>了治疗的最佳<span style="color: black;">机会</span>。<span style="color: black;">因为</span>神经母细胞瘤发病率低,<span style="color: black;">初期</span>症状又不<span style="color: black;">显著</span>,<span style="color: black;">因此</span>很难在发病初期<span style="color: black;">发掘</span>。但<span style="color: black;">倘若</span>家长能仔细观察,还是有机会<span style="color: black;">发掘</span>可疑体征,尽早到医院去<span style="color: black;">检测</span>,从而<span style="color: black;">把握</span>更有利的治疗<span style="color: black;">机会</span>。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的原发部位</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的症状取决于肿瘤侵犯的部位,可能表<span style="color: black;">此刻</span>原发部位,<span style="color: black;">亦</span>可能表<span style="color: black;">此刻</span>转移部位。神经母细胞瘤最典型的原发部位是肾上腺。在确诊时未<span style="color: black;">出现</span>转移的病例中,大<span style="color: black;">大概</span>40%原发于肾上腺,而在确诊时<span style="color: black;">已然</span><span style="color: black;">出现</span>转移的病例中,大<span style="color: black;">大概</span>60%原发于肾上腺。总体<span style="color: black;">来讲</span>,<span style="color: black;">大概</span>一半的病例原发于单侧肾上腺髓质,双侧肾上腺原发的病例少于1%。</p>
<div style="color: black; text-align: left; margin-bottom: 10px;"><img src="https://pic3.zhimg.com/80/v2-a311811bd759f47071ce16d3ec9932b6_720w.webp" style="width: 50%; margin-bottom: 20px;"></div>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">除了肾上腺,肿瘤<span style="color: black;">亦</span>可能沿着脊柱,原发于从颈部到骨盆的交感神经链上的任何<span style="color: black;">地区</span>,如颈部(1%)、胸部(19%)、腹部非肾上腺的部位(30%)和骨盆(1%)。另有文献指出,神经母细胞瘤的原发部位包括:肾上腺(50%)、椎旁神经节(25%)、胸椎和纵膈后(20%)、颈部(1%)和骨盆(4%)。<span style="color: black;">通常</span><span style="color: black;">来讲</span>,神经母细胞瘤<span style="color: black;">都数</span>原发于肾上腺和脊柱<span style="color: black;">周边</span>。</p>
<div style="color: black; text-align: left; margin-bottom: 10px;"><img src="https://pic2.zhimg.com/80/v2-77059296eb8f190bfe14f57d49bd92f9_720w.webp" style="width: 50%; margin-bottom: 20px;"></div>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤原发于多个部位的病例(即多灶病例)较为少见,<span style="color: black;">病人</span><span style="color: black;">一般</span>是婴儿,预后(即治疗后的<span style="color: black;">存活</span><span style="color: black;">状况</span>)<span style="color: black;">亦</span><span style="color: black;">一般</span>较好。有文献<span style="color: black;">报告</span>,日本某医院的108个神经母细胞瘤病例中,<span style="color: black;">仅有</span>8个多灶病例。这篇论文还<span style="color: black;">科研</span>了53个神经母细胞瘤的多灶病例,肿瘤的病理生物学特征<span style="color: black;">显示</span>,这些病例<span style="color: black;">都数</span>为神经节瘤(神经母细胞瘤的一种),并且MYCN基因不扩增,这些都是对预后有利的<span style="color: black;">原因</span>。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的原发部位和预后</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的原发部位和预后<span style="color: black;">关联</span>。相<span style="color: black;">针对</span>其他病例,原发于肾上腺的病例的总体<span style="color: black;">存活</span><span style="color: black;">情况</span>要差<span style="color: black;">有些</span>,而原发于胸部的预后较好。国际神经母细胞瘤<span style="color: black;">危害</span>组项目(International Neuroblastoma Risk Group Project)的<span style="color: black;">研究</span>人员对8369个神经母细胞瘤病例进行了统计,<span style="color: black;">她们</span><span style="color: black;">发掘</span>:</p>原发部位在肾上腺的神经母细胞瘤和不良的预后特征密切<span style="color: black;">关联</span>。同其他病例相比,原发于肾上腺的病例分期为4期的比例更大,<span style="color: black;">病人</span>发病年龄<span style="color: black;">少于</span>18个月,有染色体畸变的比例<span style="color: black;">亦</span>更高。<span style="color: black;">倘若</span>神经母细胞瘤原发于肾上腺,其MYCN扩增的<span style="color: black;">危害</span>要大于原发在腹部的其他部位,而<span style="color: black;">倘若</span>原发于脊柱<span style="color: black;">周边</span>,MYCN扩增的<span style="color: black;">危害</span>会<span style="color: black;">明显</span>降低。原发于胸部的病例比其他病例预后稍好,其MYCN扩增的<span style="color: black;">危害</span><span style="color: black;">亦</span>更低。<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">经过</span>对143个病例的跟踪比较,韩国的一项<span style="color: black;">科研</span><span style="color: black;">亦</span><span style="color: black;">显示</span>,同其他病例相比,原发于腹部的神经母细胞瘤预后更差。对原发于骨盆的神经母细胞瘤,不同的<span style="color: black;">科研</span>(样本为数十到上百)有不同的结论。有的<span style="color: black;">科研</span>者认为<span style="color: black;">由于</span>初期症状不<span style="color: black;">显著</span>,原发于骨盆的病例<span style="color: black;">更易</span>在晚期才被<span style="color: black;">发掘</span>和确诊,但<span style="color: black;">亦</span>有<span style="color: black;">科研</span>者认为原发于骨盆的病例预后较好。<span style="color: black;">针对</span>原发于胸部的病例,有<span style="color: black;">科研</span>认为其发病年龄相对更早,确诊时肿瘤未<span style="color: black;">出现</span>转移的比例更大,预后<span style="color: black;">亦</span>相对更好。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的转移部位</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">大<span style="color: black;">大概</span>50–60%的神经母细胞瘤<span style="color: black;">病人</span>,在确诊时肿瘤<span style="color: black;">已然</span><span style="color: black;">出现</span>转移。<span style="color: black;">平常</span>的转移部位<span style="color: black;">包含</span>局部淋巴结、骨髓和骨骼,转移到肺部和中枢神经系统的则比较少见。<span style="color: black;">针对</span>18个月以下的<span style="color: black;">病人</span>,转移到肝和皮肤<span style="color: black;">状况</span>相对<span style="color: black;">平常</span>。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">针对</span>4期患者<span style="color: black;">来讲</span>,最<span style="color: black;">平常</span>的转移部位<span style="color: black;">包含</span>骨髓(87%)、骨骼(66%)、淋巴结(19%)和肝脏(17%),<span style="color: black;">另一</span><span style="color: black;">亦</span>可能转移到肺(5%)或脑。<span style="color: black;">针对</span>4S期的<span style="color: black;">病人</span>,<span style="color: black;">平常</span>的转移部位有肝脏(76%)、骨髓(61%)和皮肤(12%)。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的淋巴结转移和预后</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">在国际神经母细胞瘤分级系统(International Neuroblastoma Staging System, INSS)中,原发部位同侧和对侧的淋巴结转移对应着不同的肿瘤分期。原发部位同侧的淋巴结转移对预后的影响较小,但<span style="color: black;">倘若</span>淋巴结转移越过身体中线,到达了对侧体腔,<span style="color: black;">病人</span>的预后会<span style="color: black;">显著</span>变差。<span style="color: black;">因此</span>,<span style="color: black;">针对</span><span style="color: black;">出现</span>淋巴结转移但<span style="color: black;">无</span>扩散到身体对侧的病例,其肿瘤分期<span style="color: black;">能够</span>为2B;<span style="color: black;">倘若</span>肿瘤<span style="color: black;">已然</span>跨过中线,转移到原发部位的对侧,分期就要升为3期<span style="color: black;">乃至</span>更高。有<span style="color: black;">科研</span>认为局部淋巴结转移<span style="color: black;">重点</span>影响一岁以上的<span style="color: black;">病人</span>,但<span style="color: black;">亦</span>有<span style="color: black;">科研</span>对此持不同意见。</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">神经母细胞瘤的症状和体征</p>
<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">因为</span>腹部肿瘤的病例最为<span style="color: black;">平常</span>,神经母细胞瘤的临床表现多为腹部肿块;当肿瘤侵犯其他部位时,<span style="color: black;">病人</span><span style="color: black;">亦</span>会<span style="color: black;">显现</span>其他的症状和体征。</p>腹部或骨盆内的肿瘤可能会<span style="color: black;">导致</span>腹部膨隆,但<span style="color: black;">一般</span><span style="color: black;">没</span>触痛。<span style="color: black;">孩儿</span>可能会抱怨腹痛、不想吃饭,并<span style="color: black;">显现</span>体重下降。<span style="color: black;">孩儿</span><span style="color: black;">亦</span>可能<span style="color: black;">显现</span>便秘或腹泻,排便习惯<span style="color: black;">亦</span>可能<span style="color: black;">出现</span>改变;在<span style="color: black;">少许</span><span style="color: black;">状况</span>下,可<span style="color: black;">显现</span>高血压。胸部和颈部的肿瘤可能表现为体表硬块,<span style="color: black;">一般</span><span style="color: black;">没</span>触痛。<span style="color: black;">倘若</span>肿瘤压迫到大静脉,会<span style="color: black;">导致</span>面部、颈部、手臂和上胸部肿胀,皮肤可<span style="color: black;">显现</span>蓝色或红色瘀斑。<span style="color: black;">倘若</span>肿瘤压迫到颈部交感神经,<span style="color: black;">孩儿</span>可能<span style="color: black;">显现</span>霍纳<span style="color: black;">综合症</span>,表现为眼睑下垂和单侧瞳孔缩小(两个瞳孔一大一小,见下图)。<span style="color: black;">倘若</span>肿瘤挤压到喉咙或气管,可能<span style="color: black;">引起</span>咳嗽、<span style="color: black;">呼气</span>和吞咽困难。<div style="color: black; text-align: left; margin-bottom: 10px;"><img src="https://pic4.zhimg.com/80/v2-f3208dab626edfb3b127205decbf5e93_720w.webp" style="width: 50%; margin-bottom: 20px;"></div>头部的肿瘤可能会压迫脑部的神经和血管,<span style="color: black;">导致</span>头痛、<span style="color: black;">头昏</span>或<span style="color: black;">认识</span>改变。脊柱<span style="color: black;">周边</span>的肿瘤可能会压迫神经,影响感觉和行动能力,<span style="color: black;">孩儿</span>在站立、走路或爬行时会受到影响。眼眶<span style="color: black;">周边</span>的肿瘤可能会<span style="color: black;">导致</span>眼周瘀青、肿胀、眼球<span style="color: black;">明显</span>。骨髓转移会<span style="color: black;">引起</span>贫血,表现为面色苍白,<span style="color: black;">亦</span>可能<span style="color: black;">引起</span>血小板减少,表现为<span style="color: black;">很难</span>止血。骨骼转移会<span style="color: black;">引起</span>骨骼<span style="color: black;">病痛</span>。<span style="color: black;">倘若</span>肿瘤浸润到肝脏,肝脏会<span style="color: black;">出现</span>肿大,并可能<span style="color: black;">导致</span><span style="color: black;">呼气</span>窘迫、凝血<span style="color: black;">错乱</span>、胃肠和肾脏功能损害,这些<span style="color: black;">状况</span>多见于三个月以内的婴儿<span style="color: black;">病人</span>。当<span style="color: black;">病人</span><span style="color: black;">出现</span>皮肤或皮下转移时,皮肤可能会变成青色,这种<span style="color: black;">状况</span><span style="color: black;">亦</span>多见于婴儿<span style="color: black;">病人</span>。<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">参考文献</p><a style="color: black;"><span style="color: black;">http://www.</span><span style="color: black;">cncfhope.org/cms_images</span><span style="color: black;">/file_200.pdf</span></a>Prognostic factors. Neuroblastoma Treatment. National Cancer Institute. Retrieved on 24 Feb 2017.<a style="color: black;"><span style="color: black;">https://www.</span><span style="color: black;">cancer.gov/types/neurob</span><span style="color: black;">lastoma/hp/neuroblastoma-treatment-pdq#link/_571</span></a>Signs and Symptoms of Neuroblastoma. American Cancer Society. Retrieived on 24 Feb 2017.<a style="color: black;"><span style="color: black;">https://www.</span><span style="color: black;">cancer.org/cancer/neuro</span><span style="color: black;">blastoma/detection-diagnosis-staging/signs-and-symptoms.html</span></a>Symptoms and causes. Neuroblastoma. Mayo Clinic.<a style="color: black;"><span style="color: black;">http://www.</span><span style="color: black;">mayoclinic.org/diseases</span><span style="color: black;">-conditions/neuroblastoma/symptoms-causes/dxc-20164445</span></a><a style="color: black;"><span style="color: black;">http://www.</span><span style="color: black;">macmillan.o</span></a>rg.uk/cancer<span style="color: black;">information/cancertypes/childrenscancers/typesofchildrenscancers/neuroblastoma.aspx</span><a style="color: black;"><span style="color: black;">http://</span><span style="color: black;">clinicalgate.com/neurob</span><span style="color: black;">lastoma-2/</span></a>Vo KT, Matthay KK, Neuhaus J, et al.: Clinical, biologic, and prognostic differences on the basis of primary tumor site in neuroblastoma: A report from the international neuroblastoma risk group project. J Clin Oncol 32(28): 3169–76, 2014.Hiyama E, Yokoyama T, Hiyama K, et al.: Multifocal neuroblastoma: Biologic behavior and surgical aspects. Cancer 88(8): 1955–63, 2000.Davidoff AM. Neuroblastoma. In: Oldham KT, Colombani PM, Foglia RP, et al, editors. Principles and Practice of Pediatric Surgery. Philadelphia: Lippincott Williams & Wilkins; 2005.Sung KW, Yoo KH, Koo HH, et al. Neuroblastoma originating from extra-abdominal sites: Association with favorable clinical and biological features. J Korean Med Sci. 24: 461–467, 2009.Adams GA, Shochat SJ, Smith EI, et al. Thoracic neuroblastoma: A Pediatric Oncology Group study. J Pediatr Surg. 28: 372–378, 1993.<p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;">本文系对文献的翻译和总结,不承担任何因信息不实而产生的后果。转载须注明出处;未经许可,不得转载。</p>
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对于这个问题,我有不同的看法...
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