atrial myxoma

心房粘液瘤
  • 文章类型: Case Reports
    心房粘液瘤的症状通常被视为心脏的三联征,栓塞,和体质症状。皮肤病表现被视为体质症状,并没有受到太多关注。
    案例介绍和文献综述。
    我收治了一名28岁的女性患者,她被转移到综合卒中中心,患有缺血性卒中,接受了静脉溶栓治疗。她的手/手指和脚/脚趾/脚底有两年的皮肤损伤史。皮肤活检显示血管周围中性粒细胞炎性皮炎,无皮肤血管炎。入院体检值得注意的是,她的手和脚的慢性皮肤变化以及右颈外动脉分布中面部右侧的急性红斑斑疹。MRI显示脑缺血累及右侧颈内动脉区域。经胸超声心动图确定了2.4厘米的心房粘液瘤,二尖瓣功能正常。她接受了简单的心脏手术,并确认了绒毛粘液瘤。在她为期8周的随访中,她已经解决了她的神经缺陷,她的皮疹已经清除了.文献综述在诊断为心房粘液瘤之前确定了另外17例前哨皮疹。皮肤变化通常在手和脚报告,可能涉及手指,棕榈,和鞋底。在8个案例中,皮肤活检发现真皮血管中的粘液瘤栓子。这些皮肤表现与1890年代首次描述的奥斯勒淋巴结和简威病变有相似之处,现在被理解为代表真皮的微栓子。这17例中有7例(41%)在最初出现皮肤变化后出现中风。我们的患者发生了主要影响右侧颈总动脉区域的栓塞事件,由于颈外动脉区域的栓塞和右侧颈内动脉区域的脑缺血而导致面部皮肤变化。
    我建议将心房粘液瘤的皮肤征象重新分类为栓塞(至真皮微脉管系统),而不是体质症状。由于皮肤活检通常无法显示粘液瘤栓子,对于游走性皮疹优先影响远端四肢的患者,医师应高度怀疑近端栓塞来源.当前哨皮疹首次出现时,心房粘液瘤的早期诊断为患者中风或周围栓塞之前的心脏手术提供了宝贵的机会窗口。
    UNASSIGNED: Presenting symptoms of atrial myxoma are classically viewed as a triad of cardiac, embolic, and constitutional symptoms. Dermatologic manifestations are viewed as constitutional symptoms and have not received much attention.
    UNASSIGNED: Illustrative case presentation and literature review.
    UNASSIGNED: I admitted a 28 y woman transferred to the comprehensive stroke center with an ischemic stroke treated with intravenous thrombolysis. She had a two year history of skin lesions in the hands/fingers and feet/toes/sole. Skin biopsies showed perivascular neutrophilic inflammatory dermatitis without skin vasculitis. Admission physical exam was notable for chronic skin changes on her hand and foot and an acute erythematous petechial rash over the right side of her face in the distribution of the right external carotid artery. MRI brain showed ischemia involving the right internal carotid artery territory. Transthoracic echocardiogram identified a 2.4 cm atrial myxoma with normal mitral valve function. She underwent uncomplicated cardiac surgery with confirmation of a villous myxoma. At her 8 week follow up visit, she had resolution of her neurologic deficits, and her skin rashes had cleared.Literature review identified 17 additional published cases of sentinel rashes preceding diagnosis of atrial myxoma. Skin changes were often reported in the hand and feet and may involve the digits, palm, and sole. In 8 cases, skin biopsy identified myxomatous emboli in dermal vessels. These cutaneous manifestations share similarities with Osler nodes and Janeway lesions first described in the 1890\'s, now understood to represent microemboli to the dermis. Seven of these 17 cases (41 %) developed strokes after the initial presentation of cutaneous changes. Our patient had an embolic event primarily affecting the right common carotid artery territory with facial cutaneous changes due to emboli to the external carotid artery territory and cerebral ischemia in the right internal carotid artery territory.
    UNASSIGNED: I recommend reclassification of the cutaneous signs of atrial myxoma as embolic (to the dermis microvasculature) rather than a constitutional symptom. As the skin biopsy often fails to show myxomatous emboli, physicians should have a high suspicion for a proximal embolic source for patients with a migratory rash preferentially affecting distal extremities. Earlier diagnosis of atrial myxoma when the sentinel rash first appears provides a valuable window of opportunity for cardiac surgery before the patient experiences stroke or peripheral emboli.
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  • 文章类型: Journal Article
    一般来说,在常规超声心动图中最初怀疑有心脏肿块。进一步进行心脏磁共振(CMR)成像以区分肿瘤和假肿瘤,并根据其在T1/T2加权图像上的外观来表征心脏质量。在早期和晚期钆增强图像上检测灌注和显示基于钆的造影剂摄取。CMR对心脏质量的进一步评估至关重要,因为可以通过更好的组织表征来避免不必要的手术。不同的心脏组织有不同的T1和T2弛豫时间,主要是由于质子周围的内部生化环境不同。在CMR中,来自特定组织的信号强度取决于其T1和T2弛豫时间及其质子密度。CMR使用该原理通过基于其T1或T2弛豫时间对图像进行加权来区分各种组织类型。一般来说,肿瘤细胞更大,水肿,并有相关的炎症反应。肿瘤细胞的较高游离水含量和组织组成的其他变化导致T1/T2弛豫时间延长,因此在肿瘤和正常组织之间存在固有的对比。总的来说,这些生化变化创造了一个环境,不同的心脏质量在其T1加权和T2加权图像上产生不同的信号强度,这有助于区分它们.在这篇评论文章中,我们提供了用于评估心脏肿块的核心CMR成像方案的详细描述.我们还讨论了良性心脏肿瘤的基本特征以及CMR在评估和进一步组织表征这些肿瘤中的作用。
    Generally, cardiac masses are initially suspected on routine echocardiography. Cardiac magnetic resonance (CMR) imaging is further performed to differentiate tumors from pseudo-tumors and to characterize the cardiac masses based on their appearance on T1/T2-weighted images, detection of perfusion and demonstration of gadolinium-based contrast agent uptake on early and late gadolinium enhancement images. Further evaluation of cardiac masses by CMR is critical because unnecessary surgery can be avoided by better tissue characterization. Different cardiac tissues have different T1 and T2 relaxation times, principally owing to different internal biochemical environments surrounding the protons. In CMR, the signal intensity from a particular tissue depends on its T1 and T2 relaxation times and its proton density. CMR uses this principle to differentiate between various tissue types by weighting images based on their T1 or T2 relaxation times. Generally, tumor cells are larger, edematous, and have associated inflammatory reactions. Higher free water content of the neoplastic cells and other changes in tissue composition lead to prolonged T1/T2 relaxation times and thus an inherent contrast between tumors and normal tissue exists. Overall, these biochemical changes create an environment where different cardiac masses produce different signal intensity on their T1- weighted and T2- weighted images that help to discriminate between them. In this review article, we have provided a detailed description of the core CMR imaging protocol for evaluation of cardiac masses. We have also discussed the basic features of benign cardiac tumors as well as the role of CMR in evaluation and further tissue characterization of these tumors.
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  • 文章类型: Case Reports
    成人最常见的原发性心脏肿瘤是心房粘液瘤,青少年发病并不常见。在这个案例报告中,一名15岁女性因脑血管栓塞住院,后来被诊断为左心房粘液瘤.她以前有远端血管微血栓形成的迹象,包括反复出现的双侧下肢皮疹,这对心房黏液性肿瘤的早期诊断和鉴别诊断至关重要。我们回顾了各种临床症状和诊断方法来识别左心房粘液性肿瘤。该患者还患有内分泌相关疾病。我们回顾了卡尼复合体(CNC)的诊断方法,并讨论了甲状腺疾病在诊断CNC中的作用。
    The most common primary cardiac tumors in adults are atrial myxomas, with adolescent-onset being uncommon. In this case report, a 15-year-old female was hospitalized with cerebrovascular embolism and later diagnosed with a left atrial myxoma. She had previously shown signs of distal vascular micro thrombosis, including recurring bilateral lower extremity rash, which are crucial for the early diagnosis and differential diagnosis of atrial mucinous neoplasm. We reviewed the various clinical symptoms and diagnostic approaches to identify left atrial mucinous neoplasm. This patient also had a combination of endocrine-related diseases. We reviewed the diagnostic approach for the Carney Complex (CNC) and discussed the role of thyroid disease in diagnosing CNC.
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  • 文章类型: Journal Article
    背景:脑动脉瘤和左心房粘液瘤之间的关联是已知的,但很少见。我们描述了它的发病机制,临床表现,使用文献系统综述的诊断结果和治疗。方法:通过PubMed搜索MEDLINE,使用关键字“心房粘液瘤”搜索直到2022年8月发表的文章,“心脏粘液瘤”和“脑动脉瘤”。结果:在这篇综述中,55例多发性粘液瘤动脉瘤患者进行了分析,65%是女性。诊断动脉瘤的平均年龄为42.5±15.81;大多数患者年龄小于60岁(86%)。动脉瘤可以在诊断前发现,与心脏粘液瘤同时发生,甚至在切除心房肿块后25年。在我们的审查中,平均诊断时间为4.5年.我们的综述估计,最常见的症状是血管事件(25%)和癫痫发作(14.3%)。在15个案例中,报告了可变的头痛。关于管理策略,57%的病例以保守治疗为主要选择。结论:尽管由心房粘液瘤引起的脑动脉瘤很少见,长期后果可能很严重,应该对患者进行监测。
    Background: The association between cerebral aneurysms and left atrial myxoma is known but rare. We described its pathogenesis, clinical presentation, diagnostic findings and treatment using a systemic review of the literature. Methods: MEDLINE via PubMed was searched for articles published until August 2022 using the keywords \"atrial myxoma\", \"cardiac myxoma\" and \"cerebral aneurysm\". Results: In this review, 55 patients with multiple myxomas aneurysms were analyzed, and 65% were women. The average age when aneurysms were diagnosed was 42.5 ± 15.81; most patients were less than 60 years old (86%). Aneurysms could be found before the diagnosis, at the same time as cardiac myxoma, or even 25 years after resection of the atrial mass. In our review, the mean time to diagnoses was 4.5 years. Our review estimates that the most common symptoms were vascular incidents (25%) and seizures (14.3%). In 15 cases, variable headaches were reported. Regarding management strategies, 57% cases were managed conservatively as the primary choice. Conclusions: Although cerebral aneurysms caused by atrial myxoma are rare, the long-term consequences can be serious and patients should be monitored.
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  • 文章类型: Journal Article
    UNASSIGNED: Cardiac myxomas are considered the most common benign heart tumours. The clinical manifestations mainly depend on the size of the tumour. They usually vary from asymptomatic, mild non-specific symptoms, to severe obstructive cardiac and systemic findings. We describe herein a significantly large left atrial myxoma in a patient misdiagnosed with respiratory asthma.
    UNASSIGNED: A 54-year-old lady, was diagnosed previously with asthma, presented with a history of dyspnoea on exertion, palpitations, and mild peripheral oedema. Chest X-ray suggested pulmonary congestion. Due to high suspicion of cardiac issues, transthoracic echocardiography was done revealing giant left atrial mass. Consequently, the mass was approached and excised surgically through the inverted T biatrial incision. Grossly, the mass measured 10 × 8 × 6 cm, and it had a smooth surface and was filled with gelatinous material. The histopathology confirmed benign myxoma without malignant features.
    UNASSIGNED: Our article mainly focuses on the diagnostic challenges of a patient with atrial myxoma. The major discrepancy between the tumour size and the severity of the patient\'s symptoms should draw physicians\' attention to consider atrial myxoma over a long list of differentials, in order to take immediate action to reduce the mortality and improve the overall prognosis.
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  • 文章类型: Journal Article
    UNASSIGNED: \"Carney Complex (CNC) is a familial lentiginosis syndrome, caused by PRKAR1A mutations that lead to cyclic AMP-dependent protein kinase (PKA) signaling pathway abnormalities, predisposing to a variety of skin tumors, myxomas and endocrine tumors.
    UNASSIGNED: We describe a Greek family diagnosed with CNC after recurrent embolic strokes, secondary to left-sided atrial myxomas. There are limited cases in the literature describing this type of presentation for CNC; typically, most cases present with an endocrine syndrome. Our case serves as a reminder of this rare, underdiagnosed syndrome and its wide phenotypic spectrum. It is followed by a review of the current literature on cases with cerebrovascular disease as a manifestation of CNC.
    UNASSIGNED: The co-occurrence of emboligenic cardiac myxomas and skin lesions should be an indication for screening for CNC.
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  • 文章类型: Case Reports
    Cardiac myxoma is a rare benign neoplasm of the heart. Historically myxomas were incidental findings during autopsies, however improved imaging techniques made these diagnosis possible in living patients, making the surgical treatment of these neoplasms achievable. Cardiac myxomas may occur both sporadically and in a familial context, often in the clinico-pathological picture of the Carney complex. While familial myxomas occur in the context of well-known genetic mutations, the molecular etiology of sporadically occurring myxomas is still not completely clear. We must note however that many of the patients affected by myxomas are asymptomatic; when symptoms are present they are often nonspecific and hard to decipher, especially when referring to sporadically occurring heart myxomas. In this paper we describe a case of sudden death from the massive embolization of a left atrial cardiac myxoma. We also reviewed all the cases in the literature of sudden death from heart myxoma embolism. An accurate epidemiology of heart myxomas would be the key to outline the best treatment practices and the etiology of sporadic myxomas, nevertheless this target could only be pursued with a deep revaluation of the clinical autopsy as a fundamental diagnostic tool.
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  • 文章类型: Case Reports
    栓塞性卒中是心房粘液瘤的常见并发症,而与心房粘液瘤相关的多发性脑动脉瘤很少见。与心房粘液瘤相关的脑血管疾病的发病机制尚不清楚,并且没有指南指导此类患者的治疗和麻醉管理。
    在本报告中,我们介绍了一名38岁的女性,偶尔出现头晕和头痛,被诊断为多发性脑梭形动脉瘤,其中经胸超声心动图显示有肿块附着在左心房的房间隔上。在快速心脏手术路径下进行粘液瘤切除术,无神经系统并发症,没有对脑动脉瘤进行干预。手术后6天,她出院回家接受随访。此外,我们回顾并分析了PubMed和GoogleScholar数据库中的文献,以得出此类病例的最佳治疗方案.
    心房粘液瘤相关的脑动脉瘤在大多数情况下总是呈多发性和梭形。早期切除粘液瘤和保守治疗动脉瘤是最佳治疗方法。TEE和PbtO2监测在麻醉管理中起着至关重要的作用。快通道心脏麻醉对早期评估神经功能是安全有效的。建议对“粘液瘤动脉瘤”进行长期随访。大多数患者的结果是极好的。
    Embolic stroke is a common complication of atrial myxoma, whereas multiple cerebral aneurysms associated with atrial myxoma is rare. The pathogenesis of the cerebral vascular disease related to an atrial myxoma is still not well known, and there are no guidelines to guide treatment and anesthesia management in such patients.
    In this report, we present a 38-year-old woman with occasional dizziness and headache diagnosed as multiple cerebral fusiform aneurysms, in whom transthoracic echocardiography revealed a mass attached to the interatrial septum in the left atrium. Myxoma resection was performed in fast track cardiac surgery pathway without neurological complications, and no intervention was carried out on the cerebral aneurysms. She was discharged home 6 days after the procedure for followed-up. Furthermore, we reviewed and analyzed the literature in the PubMed and Google Scholar databases in order to conclude the optimal treatment in such cases.
    Atrial myxoma-related cerebral aneurysms are always multiple and in a fusiform shape in most occasions. Early resection of myxoma and conservative therapy of aneurysm is an optimal treatment. TEE and PbtO2 monitoring play an essential role in anesthesia management. Fast track cardiac anesthesia is safe and effective to early evaluate neurological function. Long term follow-up for \"myxomatous aneurysms\" is recommended. And outcome of most patients is excellent.
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  • 文章类型: Case Reports
    尽管在过去的几十年中,心血管护理取得了巨大的进步,心血管死亡人数也有所下降,心源性猝死(SCD)仍然是一个全国性的健康问题,占所有心血管疾病死亡的一半以上.这些病例中大多数与冠状动脉疾病和心律失常有关,然而,这些病例中只有极少数是继发于心脏肿瘤。这些肿瘤通常表现为传导异常或继发于瓣膜疾病的症状,如呼吸困难,端坐呼吸,咳嗽和/或水肿。这是一个罕见的病例,一位63岁的绅士因心脏粘液瘤继发心脏猝死。
    Despite the huge improvement in cardiovascular care over the past several decades and the decline in cardiovascular deaths, sudden cardiac death (SCD) continues to present a nationwide health problem accounting to more than half of all deaths from cardiovascular disease. Majority of these cases are related to coronary artery disease and arrhythmias, however, a very small number of these cases are secondary to cardiac neoplasms. These neoplasms commonly present with conduction abnormalities or symptoms secondary to valvular disease such as dyspnea, orthopnea, cough and/or edema. This is a rare case of a 63-year-old gentleman who suffered sudden cardiac death secondary to a cardiac myxoma.
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  • 文章类型: Case Reports
    Background  Cardiac myxomas, the most common primary cardiac tumors, are generally benign neoplasms. Primary cardiac lymphoma is a rare cardiac malignancy with a very poor prognosis. Here we present a case of a cardiac myxoma with cerebral metastases and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) arising within the cerebral metastases. Case description  A 62-year-old man, who presented with symptoms of multiple transient ischemic attacks, was found to have a left atrial myxoma. Twelve months after excision of the myxoma, the patient experienced a recurrence of neurologic symptoms. Brain magnetic resonance imaging revealed multiple hemorrhagic masses. Craniotomy was performed to resect the lesions. Histopathologic examination confirmed cardiac myxoma metastases and a small lymphocytic infiltrate within the tumor consistent with CLL/SLL. Conclusion  Including the present case, there are 27 cases of cardiac myxoma cerebral metastases and 22 cases of lymphomas arising within myxomas. The present case is the first known instance of both entities in the same patient. There is no standard management for either cardiac myxoma metastases or lymphoma within a myxoma. For both diseases, surgical excision is the primary treatment modality, but postoperative chemotherapy and/or radiation have been attempted. Myxomas may create a chronic inflammatory state that could lead to the development of CLL/SLL.
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