巨大的右室粘液瘤一例报告——附电镜观察

心脏粘液瘤是最常见的原发性、良性心脏肿瘤,四个心腔均可发生,但见于心房者占95%以上。发生于心室者,尤以右心室者极为罕见。M 型和二维超声心动图问世后,对心脏肿瘤诊断有了极大的改观,目前已公认为这一无创性的检查是心脏粘液瘤可靠的诊断方法。右室粘液瘤国内报道三例,1982年 Bactolotti 复习文献,收集世界文献中右室粘液瘤病例34例,     

Left atrial myxoma presenting with synchronous carotid and femoral embolism

Summary Synchronous embolism of the right femoral and the left internal carotid arteries, occurring in a 39-year-old teacher upon physical effort, was found to be due to a left atrial myxoma. Clinical findings, electrocardiogram and chest radiographs had suggested myocarditis. The patient died of cerebral infarction. In cases of suspected myocarditis, left atrial myxoma has to be considered in the differential diagnosis. Histological examination is recommended of any material obtained by embolectomy.

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Zusammenfassung Ausgehend von einem Myxom des linken Vorhofes, traten bei einem 39jährigen Lehrer unter körperlicher Belastung gleichzeitig embolische Verschlüsse der rechten Arteria femoralis und der linken Arteria carotis interna auf. Der Tumor wurde erst post mortem diagnostiziert. Der klinische Herzbefund ließ im vorliegenden Fall am ehesten an eine Myocarditis denken. In seltenen Fällen ist bei bestehendem Verdacht auf Myocarditis differentialdiagnostisch auch ein Vorhofmyxom in Betracht zu ziehen. Durch Embolektomie gewonnenes Material sollte immer histologisch untersucht werden.

     

Cellular ‘neurothekeoma’: an epithelioid variant of pilar eiomyoma? Morphological and immunohistochemical analysis of a series

Cellular neurothekeoma is a recently recognized benign cutaneous neoplasm, which is currently regarded as being of nerve sheath origin and is thought to represent a variant of conventional neurothekeoma (dermal nerve sheath myxoma). Nine new cases presenting predominantly in adolescents or young adults are described. Morphologically they were characterized by short fascicles or small nests of palely eosinophilic epithelioid or spindle-shaped cells which ramified in an ill-defined manner between dermal collagen bundles. Myxoid matrix was absent or sparse. Scattered normal mitoses and multinucleate giant cells were often present. Immunohistochemically all nine cases were strongly NK1/C3 positive, seven were weakly NSE positive and three were smooth muscle actin positive. Staining for S-100 protein, PGP 9.5, epithelial membrane antigen and desmin was negative in all cases. In view of its distinctive architecture and immunophenotype, both of which are totally different from conventional neurothekeoma, it is proposed that cellular 'neurothekeoma' is a separate discrete entity which may represent an epithelioid variant of pilar leiomyoma.     

Left atrial myxoma and systemic AL-amyloidosis.

A case of left atrial myxoma with systemic AL-amyloidosis is described. Localized myxomas have the peculiar property of giving rise to constitutional symptoms, indicating a systemic disease process. The coexistence of AL-amyloidosis and an atrial myxoma in the same patient makes it tempting to suggest that an immunological defect is involved in the development of both diseases.     

Erythematous Papules in a Patient with Cardiac Myxoma: A Case Report and Review of the Literature

A 35-year-old woman was followed up by a physician for one and a half years as a case of transient ischemic attack (TIA) due to thrombosis. She had a few erythematous papules on the dorsal side of her right forearm and on the right side of her neck. From the history of TIA and the transient erythematous papules, we suggested the possibility of cardiac myxoma. Echocardiogram demonstrated a large mass in her left atrium. An immediate operation was carried out, and the cardiac myxoma was excised surgically. Histological examination of the erythematous papule revealed myxoma emboli.     

Right atrial myxoma mistaken for recurrent pulmonary thromboembolism

A 69 year old man was admitted for investigation of right sided pleuritic chest pain and dyspnoea, both of which began suddenly four days before admission. Acute pulmonary embolism was diagnosed. Six months after discharge while on warfarin he died. Necropsy found a 50 mm diameter myxoid tumour arising on the right atrial side of the interatrial septum. This lesion may have been discovered earlier by echocardiography although there were no clear indications for this investigation. Presentation was that of recurrent pulmonary embolism with no obvious source or cause of thrombosis. Patients who are thought to have idiopathic pulmonary embolism should undergo early echocardiography to exclude the rare but treatable diseases of the right heart that may be responsible

Keywords: atrial myxoma     

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.     

Left atrial thrombus masquerading as a myxoma in a patient with mitral stenosis

The preoperative differentiation of a thrombus from a myxoma is important but not always easy. Clinical and echocardiographic characteristics of myxoma and thrombi are not diverse enough to always reliably distinguish the two. We report the case of a patient who was found to have a left atrial appendage thrombus that was misdiagnosed as a myxoma on the basis of its imaging characteristics on cardiac magnetic resonance in addition to the detection of dense neovascularization on coronary angiography. This case highlights the unusual features of an organized thrombus that can mimic many of the distinguishing characteristics of a myxoma.     

The Variations in the Immunologic Features and Interleukin-6 Levels for the Surgical Treatment of Cardiac Myxomas

Purpose In this study, we propose the existence of a relationship between cardiac myxomas and the immunologic features or interleukin-6 (IL-6), while also considering the optimal treatment of cardiac myxoma, especially “familial myxoma.” Methods In a 19-year period at our hospital, 20 patients underwent 21 operations for cardiac myxomas. The immunologic features and the IL-6 levels were measured pre-operatively in 13 cases and post-operatively in 10 cases. A case of “familial myxoma” was diagnosed based on molecular genetic analyses. Results No patients died in the hospital. The tumor size correlated with the preoperative IL-6 and/or α1-globulin values (P < 0.05). In addition, all of the immunologic features and IL-6 levels normalized by 4 weeks after surgery. “Familial myxoma” demonstrated recurrence without showing increases in either the immunologic features, inflammatory signs, or serum IL-6 levels. Conclusions Patients with cardiac myxoma should therefore be operated on immediately because the possibility that the tumor size might be large when IL-6 and/or α1-globulin values are high. In addition, cases of “familial myxoma” require careful observation and periodic echocardiography after surgery to identify any possible recurrence. Recently, molecular genetic analyses are therefore considered to be an important diagnostic tool for cardiac myxoma, especially “familial myxoma.” Our “familial myxoma” case demonstrated a C769T PRKAR1a mutation, which has also been observed in other cases of “familial myxoma.”     

Intraoperative real time three-dimensional transesophageal echocardiographic evaluation of right atrial tumor

Right atrial myxomas are uncommon heart tumors that can simulate nonspecific symptoms, such as fever, paroxysmal palpitations, chronic anemia, weight loss, and may escape timely diagnosis until the development of severe complications due to embolism. We present a patient with a history of palpitations. In search for the source of palpitations, a 2D transthoracic echocardiography was performed, showing a right atrial mass. Real time three-dimensional transesophageal echocardiography (RT3DTEE) was performed intraoperative and demonstrated very accurate information about the size and the morphology of the tumor. This is the first case report of a right atrial myxoma visualized intraoperatively by RT3DTEE .