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儿童初等心脏肿瘤:艺术状况审查

标题、摘要和全文为机器翻译。 英文原文

  • Kevan English1
  • Elfreda Dzukey2
  • Reicelle Garcia3
  • Kritikka Ajit Kumar4
  1. Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA ROR
  2. Department of Pharmacy, Campbell University College of Pharmacy & Health Sciences, Buies Creek, NC, USA ROR
  3. Department of Internal Medicine, Creighton University Medical Center-Bergan Mercy, Omaha, NE, USA ROR
  4. Department of Pediatrics, University of South Florida College of Medicine, Tampa, FL, USA ROR
下载 PDF (0.9 MB) 引用方式 doi:10.5455/umj.20260718040933

摘要

心肌瘤是指由心内膜,心肌,或周围的心肌引起的良性或恶性肿瘤. 它们可以是初级的或元的. 原发性肿瘤罕见,据报解剖频率高达0.30%. 这些肿瘤可能发生在新生儿期或胎儿期,后者的发病率为0.14%. 大多数儿科心脏瘤都是良性的,大约10%是恶性肿瘤. Rhabdomyoma是儿童中最常见的良性肿瘤,占这些人群中所有初级心脏肿瘤的50%以上. 在胎儿或新生儿时期观察到的多颗心脏瘤通常与管状硬化有关。 其他风险因素包括家族史和其他遗传倾向,如卡尼综合体. 在临床怀疑时,回声心电图、计算成像和磁共振成像有助于诊断。 治疗包括手术、化疗和有针对性的治疗。 文章对儿童的心脏肿瘤、其流行病学、组织规律、诊断和治疗进行了范围审查.

关键词

全文

全文为机器翻译；以英文原文为准。 英文原文

导言

儿童体内的心肌瘤很少见,最常见的是心内膜,心肌,或周围的心外膜. (1, 2)这些肿瘤可以是初级肿瘤,也可以是元肿瘤,在解剖系列中,初级肿瘤的流行率在0.0017%至0.28%之间。 (3)在胎儿期,心脏肿瘤的发病率据报高达0.14%。 (3, 4)绝大多数儿童的主要心脏肿瘤是良性的,高达10%是恶性肿瘤。 (5)二级恶性肿瘤比初级恶性肿瘤普遍得多,成年人和儿童心脏肿瘤的类型和频率不同。 (3–5)。 。 。

在成人中,75%的肿瘤是良性的,25%是恶性的。 (3–6)肌瘤是成年人中最常见的一级肿瘤,沙子瘤占恶性肿瘤的75% (7–9)rhabdomyoma是童年和胎儿生活中最常见的心脏肿瘤,其次是纤维瘤和致畸瘤(图1)。 (10, 11)心肌积聚的病人可能无症状,或出现心力衰竭、心律迟钝、心律突然死亡或心律失常。 (12)心电图是主要的诊断方式,心脏磁共振成像(CMRI)进一步定性肿瘤和邻近心脏结构的范围以支持治疗规划 (13, 14). 管理方式从保守到重新剖析或移植各不相同. 本条对儿童心脏肿瘤进行了范围审查,包括诊断和治疗。

流行病学

儿科人口中主要心脏肿瘤(PCTs)很少,在各种尸检序列中发病率高达0.28% (3)此外,据报告,胎儿生命期心血管肿瘤的发病率约为0.14%。 (3, 4)大多数多氯三联苯是良性的,估计有10%的恶性 (3, 4, 15)儿童与成年人的心脏病类型和频率不同。 在成年人中,良性初级肿瘤占心脏肿瘤的75%,恶性肿瘤约占25%。 (16, 17)。 。 。

此外,肌瘤是成年人中最常见的主要肿瘤,占良性肿瘤的40%左右,与新生儿和胎儿相比,后者极为罕见。 (18)rhabdomyoma是儿童和胎儿生活中最常见的PCT,占初级肿瘤的近70%,其次是teratoma、纤维瘤和肝细胞瘤。 (19, 20)与肌瘤类似,rhabdomyomas在成人中极为罕见,二级恶性心脏瘤的发病率高达20x 高于初级恶性肿瘤 (19–21)在过去几年中,多氯三联苯的发病率明显增加。 (22)这种趋势的部分原因可能是成像技术的进步,更先进的成像易于获取。

图1:显示儿科心脏瘤流行病学和位置分布的巴图。 LV = 左心房;RV = 右心房;LA = 左心房;RA = 右心房.
Figure 1. 显示儿科心脏瘤流行病学和位置分布的条形图. LV = 左心房;RV = 右心房;LA = 左心房;RA = 右心房.

临床表现

一般来说,心脏肿瘤可能在胎儿或产后生活中出现. 一些肿瘤可能具有症状,或者在评估时偶然发现,原因有别. 症状通常与肿瘤的心脏位置有关,虽然有些症状可能引起宪定症状. 胎儿生命中心脏肿瘤的临床表现可能包括凝血性心衰竭(CHF),心律失常(hythmias),以及水分胎儿(hydops fetalis). (23, 24)在产后生活中,由于内流或外流障碍,可能出现心肌功能障碍、呼吸困难、杂音、氰化物、心律失常、肝脏不足和性病。 (23–25)更常见的阴道肿瘤可能妨碍左流或右流道,可能表现为胸痛或同步。 大型地心瘤可以阻断心血管(AV)的阀流和模仿性地心血管激素.

图2:显示儿童主要心脏肿瘤最常见的位置的图示图。
Figure 2. 说明性图表,显示儿童主要心脏肿瘤最常见的位置。

从心律失常的角度看,肠内或心内肿瘤可以通过心肌刺激或导体组织直接渗入影响心律节奏,这可能导致AV阻塞和心室心肌炎. 但是,列报也可能是直接的SCD. 出现心律失常症应始终导致寻找结构性心脏病,有时偶然发现心脏肿瘤。 (26, 27)患者还可能患有肿瘤产生的肺和/或系统血栓 (28). 多个微小胚胎可能模仿心内膜炎或血管炎,而较大的碎片在涉及左侧肿瘤时可能导致缺血性中风 (28, 29)右侧肿瘤经典地动员到肺上,可能会产生胸膜症状和可能的右心衰竭.

最后,患者可能出现发烧、动脉瘤、体重下降和疲劳等系统性症状,以及偏头痛综合症。 (30, 31)血液异常,包括多细胞贫血、贫血、白细胞病、血栓细胞病和高发炎标记(即C-反应蛋白和红细胞沉积率),也可能发生。 (30–32)。 。 。

诊断工作

心脏肿瘤患者的临床表现和预测因损伤位置和组织类型而异(图2)。 此外,环境还提供了诊断线索,有助于确定损伤或肿瘤的病理。 例如,在出现细菌性贫血和高发炎标志的假肢或原生阀门疾病的病人中,可能观察到植被。 相比之下,心肌梗塞更可能与心律失常有关,如心肌纤维化,或左心肌梗塞与下皮炎或心肌梗塞. 如果最终发现心脏质量,则通过考虑四个因素确定其病原学:患者的年龄、肿瘤的位置、基于CMRI的组织特征以及肿瘤的流行病学可能性或临床概率(表1-2) (33)。 。 。

Table 1.
表1 按区域开列的所需资源

初级心肺肿瘤

狂犬病

心脏病是儿童中最常见的一次心脏肿瘤,占多氯三联苯的60%以上。 (10, 11, 34)它最常与管状硬化综合症有关,这是一种自发性的主要障碍,其特征是大脑和其他地区的无癌肿瘤或损伤。 (35)通常,Rhabdomyomas位于通风口内。 在回声心电图上,它们出现在心肌的心肌中,是同质的,环绕良好的回声原生体。 (36). 计算成型或磁共振可以更好地划分质量与涉及的心肌和相邻结构的数量之间的关系。 这些患者还应进行全身成像,排除其他良性肿瘤和恶性肿瘤,如肾细胞癌、乳腺癌和甲状腺癌等。 (37–39)Rhabdomyomas 显示具有中央位置细胞瘤的病原体蜘蛛细胞,其核和肌膜辐射到细胞壁(图3A) (40). 临床表现因肿瘤的位置、数量和大小而异。 在AV交叉口的出现有时可以使肿瘤起到从属作用,这会造成心律不适 (41)心脏病可能导致共同人道主义基金和婴儿呼吸困难 (10, 11, 40, 41). 鉴于其出生后自发回归率高,可通过回声心电图和心电图监测进行保守管理 (42)手术干预应只针对血动力妥协、难以克服的心律失常或严重障碍。

图3:(A) 电显微镜显示由具有丰富的甘化物(脊髓灰质细胞)的大型空心细胞组成的心律瘤。 (B) 心肌瘤的高功率显微镜显示极少的细胞性。 散落的脊柱细胞 微小的粉红色细胞质
Figure 3. (A) 电显微镜显示由具有丰富的甘油(平板细胞)的大型排卵细胞组成的心律瘤。 (B) 心肌瘤的高功率显微镜显示极少的细胞性。 在松散的 myxoid stroma中,有分布的脊髓细胞,很少出现粉红色细胞瘤. (C)有时在心肌纤维瘤(Hematoxylin和Eosin 污物,20x)中出现分泌性钙化. (D)低功率的视差是位于损伤深处的有突出的,厚厚的,大口径的喂养容器的圆柱形肝脏瘤.

肌瘤

心肌瘤是常见的良性多氯三联苯,最常出现在左原子(图3B)中. (43). 肌瘤是零星的;然而,在所有心肌瘤中,有多达7%的家族病症被描述为家族病症,有时与卡尼复合体有关,是PRKAR1A基因突变导致的罕见遗传紊乱,导致色素变化,良性肿瘤,内分泌异常. (33, 44, 45)肿瘤以短而广泛的附着在具有胶质一致性的试墙上。 在回声心电图上, myxomas 以移动质量的形式出现,通过一个支架附着在心内膜表面,通常起源于fossa ovalis. (46)超过50%的左心肌瘤患者出现症状,可能包括脊髓质瓣膜激素化或阻塞导致的痢疾和矫形。 (47)肿瘤的分解可能使脑血管机能栓塞并引起脑血管事件。 外科切除手术仍然是治疗的支柱,并导致极佳的长期存活,大多数患者都有良好的预后. (48)。 。 。

纤维

心肌纤维瘤(英語:Cardiac fibromas)是连接组织纤维拉片衍生的单独肿瘤,主要分布于心肌囊内. (49)它们通常在童年时期出现,从1厘米到10厘米不等。 (49, 50)在宏观层面,纤维瘤与心肌明显分界线。 从微观角度来说,心肌周围的数位化,肿瘤中心部分的钙化是病态的,反映了肿瘤的血液供应不良(图3C) (51)在回声心电图上,它们作为大型的、周密的单独物质出现在通风自由墙或塞普图中。 (52)以通风口为中心的单独、大型、同质心肌质量在CT或核磁共振上通常很明显。 (52, 53)。 。 。

纤维瘤可以侵入心肌,取代功能性心肌,并可能造成抗硬性氰化症或CHF (49–51)偶尔,肿瘤可能延伸至通风传导系统,导致AV阻断或心律失常。 (50–54)这些肿瘤通常处于休眠状态,自发性回归很少发生。 因此,通常建议进行外科手术 (55). 功能性心肌纤维的逐渐丧失和心力衰竭不会在重新剖析后得到解决,这可能导致考虑心脏移植.

帕皮氏纤维素瘤

帕皮纤维素瘤是最常见的多氯三联苯 (56)随着影像质量和技术多年来的改进,人们现在越来越认识到它们。 在粗略检查时,它们看起来是小的洞内质团,通常是坚固的,有一个短的柱子和多条帕彼利面 (57)这些肿瘤通常很小,临床上很少出现有阻碍的迹象。 与脑缺血有关的症状患者的手术病例 (58)。 。 。

赫曼吉欧马

血管瘤是良性肿瘤,主要由血管组成 (59)它们最常出现在右侧的阁楼和通风塞普图中,最常偶然发现。 (59, 60)新生儿的多次皮肤肝脏瘤应促进心脏肝脏瘤的修复 (60, 61)在组织学上,它们表现为由内皮细胞排列的血管扩散,这些血管可能是大而薄的血管(如穴状的肝脏瘤,最常见的类型),小毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛细毛 (62)肝脏瘤是良性肿瘤,通常会自发退缩,预后良好 (60–62)。 。 。

利波玛

唇瘤是良性肿瘤,主要由成熟脂肪细胞组成 (63)它们可以在任何年龄出现,在男性和女性中频率相同。 利波马在心脏的任何地方都可能发生,并且根据肿瘤的位置在临床上出现. 核磁共振通常用于诊断脂瘤,这明显表明脂肪组织 (64)。这些肿瘤通常无声,尽管有时会导致心律失常和AV区块。 (63, 64)。 。 。

初级恶性心脏病肿瘤

昂焦萨尔科马

Angiosarcoma是一种具有攻击性的肿瘤,它经常起源于右侧的阁楼,在陈述时极有可能发生元化. (65). 组织病理学通常显示一种肿瘤,其血管化程度高,有心肌渗透、多发性、坏死和结膜炎。 (66). 心电图通常在右侧靠近下等藤木的阁楼显示一个点点或隆起的质量 (67). CT或核磁共振显示动脉相增强,从而提高诊断准确性 (68, 69). 临床表述往往不具体,可能包括心腹旁塞或右侧心脏衰竭的症状 (66, 67)地方肺、胸膜和中枢元瘤也很常见。 (66–69)治疗通常包括手术和辐射以及化疗 (68–70)。 。 。

莱奥米约萨科马

Leiomyosarcoma是平滑肌肉细胞的原生物,一般起源于两个地点。 (71)它可以在左边的阁楼里形成, 模仿左心肌瘤及其症状, 或者肺内膜和动脉,模仿肺栓塞 (72, 73). Dyspnea是主要的抱怨,但患者有时可能会出现胸膜胸痛,肝炎,以及庭外纤维化等心律不适的症状. 治疗包括外科切除术 (74). 化学辐射疗法可提供治疗 (72–74)。 。 。

拉博多米约萨尔科马

心脏硬骨瘤是儿童最常见的恶性心脏肿瘤,经常影响阀门 (75)这些是斑纹肌肉的恶性肿瘤,约占心脏骨骼的4%至7%,男性发病率很高。 (75, 76)在粗略的检查中,它们看起来有粘液或胶质。 从史学上看,胚胎型显示了含丰富的甘油脂和脱氧核糖核酸、血红蛋白和蛋白素的表达。 (75)患者可能出现心腹病、胸膜输精或胚胎现象的症状,但也可能出现系统性疾病,包括发烧、体重下降和疾病。 与血管癌(angiosarcoma)相比,分散的心肌参与是少见的,肿瘤很少侵入心肌外. 预后一般很差 (77)。 。 。

菲布罗萨尔科马

纤维链瘤(Fibrosarcomas)是中枢瘤,发源于纤维瘤,一般可见于左心室或右心室内. (78). 临床表现因肿瘤位置和大小而异。 在左室参与下,乳头硬化、肺拥塞和肺静脉阻塞的症状很常见。 (79)心肌纤维瘤的预后一般较差,中位存活时间不到一年。 (80)。 。 。

无差别的全形沙拉科马

无差别的心脏骨骼具有入侵性,没有特定的组织形态 (81, 82). 它们最常出现在左侧的阁楼中,大多数患者出现肺拥塞. 尽管手术和缓和化疗,预后普遍不佳 (81–83)术后平均存活期为5至18个月 (81–84)。 。 。

Table 2. 儿童、婴儿和成人主要恶性心脏肿瘤的临床特征、发病率、性别分布和组织病理学,从Tyebally修改 et al (中文(简体) ).页:1
表2:从Tyebally等人(33人)改编而来的主要恶性心脏肿瘤的临床特征、发病率、性别分布以及儿童、婴儿和成年人的病理学。

审查的限制

关于儿科心脏瘤的现有文献受到其罕见且样本规模小的限制,大多数公布的证据包括单中心病例系列,评论,以及多中心协作,这些合作仍然招收数量不大的人群. 初级心脏肿瘤在儿科解剖系列中大约为0.0017-0.28%,占儿科心脏手术的不到0.1%。 (3, 85)迄今最大的多中心心血管磁共振研究包括了31个国际中心的213个经证实的儿科心脏群,但即使是这一重大努力也反映了多年来罕见的病例总数 (86)单机构外科手术系列通常报告2-3年累计的16-120名病人,全世界不到20个病例记录了恶性组织型,如初心Ewing sarcoma。 (85, 87–89). 这一数据匮乏限制了循证管理准则的制定;2022心血管磁共振学会/美国回声心学学会的共识指出,出版的心血管磁共振经验主要包括"案例报告和相对较小的案例系列".(90)因此,诊断算法依赖专家的共识而不是预期的验证,外科手术策略是从良性肿瘤经验中推断出来的,结果——特别是罕见的恶性变体的结果——仍然缺乏特征。 (86, 91)肿瘤异质性(rhabdomyoma、纤维瘤、 myxoma、teratoma、hemangioma和各种sarcomas)进一步分散了现有证据,使建立特定组织型预测模型或标准化治疗协议的努力复杂化。

结论

简而言之,心脏肿瘤是罕见的,可能造成严重并发症,如致命的心律失常和心内障碍,即使在良性时也是如此. 磁共振和CT扫描等成像技术在诊断中发挥着关键作用,有助于区分肿瘤类型,并最终缩短从诊断到治疗的时间. 这些肿瘤可以通过手术治疗,其发病率和死亡率是可以接受的。 死亡率最强的预测因素与病人的体征,展示时的血动力状态,以及心肌组织参与的百分比有关. 对儿科心脏瘤患者的评价需要多学科评价和个性化管理. 总体而言,良性肿瘤的存活率很高,在重新解剖后应监测患者的复发情况。 竞争利益声明:作者报告没有相关的竞争利益。

声明

作者贡献（CRediT）
所有作者对全部 14 项 CRediT 角色贡献均等（Conceptualization, Data curation, Formal analysis, Funding acquisition, Investigation, Methodology, Project administration, Resources, Software, Supervision, Validation, Visualization, Writing – original draft, Writing – review & editing）。
资助

This article received no specific grant or support from any public or private agencies.

利益冲突
The authors report no relevant competing interests.
伦理批准
Ethics committee approval was not required for this article, as it is based on previously published literature and does not involve human participants, patient data or animals.
数据可用性
Data sharing is not applicable to this article as no new data were created or analysed in this study.

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English K, Dzukey E, Garcia R, Kumar KA. Primary Cardiac Tumors in Children: A State-of-the-Art Review. Ulutas Med J. 2026;12(3):29-40. doi:10.5455/umj.20260718040933

English, K., Dzukey, E., Garcia, R., & Kumar, K. A. (2026). Primary Cardiac Tumors in Children: A State-of-the-Art Review. The Ulutas Medical Journal, 12(3), 29–40. https://doi.org/10.5455/umj.20260718040933

English, K. et al. (2026) 'Primary Cardiac Tumors in Children: A State-of-the-Art Review', The Ulutas Medical Journal, 12(3), pp. 29–40. doi: 10.5455/umj.20260718040933.

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