Large left atrial myxoma presented as fever of unknown origin: a challenging diagnosis and a review of the literature

Myxoma is the most common type of primary tumors of the heart in adults. The majority of patients with myxomas may experience symptoms due to central or peripheral embolism or intracardiac obstruction, while in some cases, they may be completely asymptomatic. Rarely, patients develop unusual symptoms that complicate the diagnostic evaluation. Herein, we describe the case of a 70-year-old patient with a long-lasting low-grade fever due to a large left atrial myxoma revealed during a transthoracic echocardiography.     

卡尼复合征心脏黏液瘤的临床特征及外科治疗结果

目的  比较卡尼复合征（Carney complex）心脏黏液瘤与散发心脏黏液瘤的临床特征及外科治疗结果,为Carney complex心脏黏液瘤提供诊疗经验。 方法 回顾性分析阜外医院2009年1月至2013年12月共372例接受心脏黏液瘤切除手术患者的临床资料,其中7例确诊为Carney complex心脏黏液瘤。7例中男2例,女5例;年龄7～50岁,平均（2857±1326）岁;均接受外科手术切除。术后随访54～94个月,平均（7686±1549）个月。比较Carney complex心脏黏液瘤和散发心脏黏液瘤在年龄、性别、术前动脉栓塞率、心脏黏液瘤多发性和心脏黏液瘤切除术后的复发率。 结果 手术切除Carney complex心脏黏液瘤在总体心脏黏液瘤中的发病率为188％,最常见的发生部位是左房。所有7例Carney complex心脏黏液瘤术后没有早期死亡,随访期间无死亡。Carney complex心脏黏液瘤与散发性心脏黏液瘤在年龄、术前动脉栓塞率、心脏黏液瘤多发率及心脏黏液瘤术后复发率的差异均有统计学意义（P＜005）,而在性别上差异无统计学意义（P＞005）。 结论  Carney complex心脏黏液瘤可取得良好的外科疗效。与散发心脏黏液瘤相比,Carney complex心脏黏液瘤发病年龄更早,术前动脉栓塞率更高,常见多发肿瘤,术后复发率更高。对于Carney complex心脏黏液瘤,外科手术应更积极,术后应更密切随访。     

A midface swelling in a child – A possible diagnostic dilemma

A lump on the midface of a child can pose as a diagnostic dilemma. There is a wide variety of possible differential diagnoses, ranging from simple benign conditions such as a sebaceous cyst, dermoid cyst, lipoma, neuroma and neurofibroma, to potentially devastating conditions such as odontogenic myxoma.A case of a child in which the formulation of a definite diagnosis was clinically and histologically challenging is presented.     

Cardiac myxoma with glandular component: case report

Cardiac myxoma has varying clinical presentation, uncertain histogenesis and debatable immunohis- tochemical profile. Glandular epithelial differentiation is a rare phenomenon, but glandular elements are known to be present in cardiac myxoma as an intrinsic component of the tumor. We present a case of cardiac myxoma having focal glandular differentiation, with special reference to the morphologic features and immunohistochemical profiles.     

心脏粘液瘤的外科治疗

目的总结我院10年来心脏粘液瘤的外科治疗经验,探讨心脏粘液瘤的临床特点及诊疗要素。方法对1992年10月至2002年12月手术治疗的15例心脏粘液瘤患者的临床资料进行回顾性分析,并复习有关文献。结果男6例,女9例,年龄21～63岁(平均45.08±12.40岁)。其中左房粘液瘤14例,右房粘液瘤1例,均在全麻中低温体外循环下行心脏粘液瘤切除术,2例同期行冠状动脉搭桥术,1例同期行二尖瓣置换术,2例同期行三尖瓣成形术。主动脉阻断时间38.58±21.56分钟,体外循环时间74.33±43.47分钟。全组无死亡。随访6个月～8年(平均4.5年),无复发、无远期并发症。结论对于心脏左房粘液瘤,及时手术是唯一有效的治疗方法。     

Cardiac myxoma is rich in factor XIIIa positive dendrophages: immunohistochemical study of four cases

Cardiac myxoma is an enigmatic tumour thought to arise from primitive cardiac mesenchymal cells. Factor XIIIa+ dendrophages are tissue histiocytes that are active in tissue repair and thrombosis. To explore whether factor XIIIa+ dendrophages play a role in cardiac myxoma morphogenesis, we stained four cases with an antiserum against coagulation factor XIIIa (FXIIIa). We also used antibodies recognizing CD34, CD31, and S-100 protein. Samples of valvular endocardium from 12 and 16 week fetuses and two adult autopsies were compared with the four myxomas. All cardiac myxomas had rounded and dendritic FXIIIa+ cells admixed with more numerous CD34+ spindle and stellate myxoma cells. The CD34+ cells formed multicellular syncytia and capillary sprouts. Many of these syncytial structures also expressed CD31 and, to a lesser extent, S-100 protein, strongly in two cases and more focally in two. Fetal subendocardium was composed of CD34+ stellate fibroblast-like cells invested with scattered FXIIIa+ histiocytes; no S-100+ cells were detected. Our findings confirm that cardiac myxomas are composed of CD34+ primitive subendocardial cells. These cells show a capacity for CD31+ endothelial differentiation. In cardiac myxoma, the CD34+ myxoma cells are accompanied by numerous FXIIIa+ dendrophages, the presence of which suggests abnormal organizing thrombus-like differentiation in cardiac myxoma morphogenesis.     

Immunohistochemical expression of endothelial markers in left atrial myxomas: a study of six cases

Vascular endothelial cells are antigenically heterogeneous and therefore it has been recommended that a range of immunohistochemical markers is employed to show the presence of cells of endothelial origin in surgical pathology. In this study we applied three monoclonal antibodies—to factor VIII-related antigen, JC70 (CD31), QBend 10 (CD34)—and Ulex europaeus agglutinin type 1 lectin (UEA-1), to six consecutive cases of left atrial myxoma. We found that JC70 and QBend 10 consistently stained myxoma cells in all their different growth patterns contrasting with factor VIII-related antigen expression and UEA-1 binding which were restricted to areas which showed morphological evidence of vascular differentiation. These findings suggest that the constituent cells of atrial myxomas show more widespread endothelial differentiation than has previously been recognized and that differences in immunohistochemical staining may reflect the maturation status of these cells.     

Intramuscular myxoma with fibrous dysplasia: A report of two cases with a review of the literature

Two cases are reported of a rare association of intramuscular myxoma with fibrous dysplasia in a 70 and 40 year old Japanese woman, respectively. One of them had a solitary intramuscular myxoma, and the other patient suffered from two intramuscular tumors that had been initially misdiag-nosed as myxoid liposarcoma. Only 24 cases of this kind of association have been recorded in the literature. This association should be taken into consideration to avoid inappropriate treatment, when cases of myxoid soft tissue tumor with a bone lesion are encountered.     

Demonstration of the inner structure of a right atrial myxoma by transoesophageal echocardiography

We report a case of a large right atrial myxoma. With the useof transoesophagal echocardiography, several cysts were idntifieldwithin the tumour and necrotic tissue was differentiated frommyxomatous tissue according to its echo-level. Transoesophagealechocardiography was very useful for the precise diagnosis ofcardiac tumour and demonstration of the inner structure.