Massive right atrial myxoma

Right atrial myxomas are uncommon and are often detected incidentally in asymptomatic individuals. We describe a case of a massive right atrial myxoma that was suspected following an abnormal right heart border on a chest X-ray and an abnormal 12 lead electrocardiogram.     

心房黏液瘤合并动脉栓塞的CT表现与临床治疗

目的 探讨心房黏液瘤合并动脉栓塞的CT表现、临床治疗及疗效。方法 收集6例左心房黏液瘤合并动脉栓塞患者,均于完善包括冠状动脉及外周动脉CTA检查在内的相关检查后行手术治疗;对其CTA表现及临床资料进行回顾性分析。结果 6例心房黏液瘤均发生于左心房,CT平扫呈低密度,3例动脉期强化,蒂宽窄不等,1例伴有钙化;6例均伴有动脉栓塞,栓塞部位为髂动脉2例、 肱动脉1例、 股动脉2例和腹主动脉伴两侧髂动脉多发栓塞1例。采用同期或分次心房黏液瘤切除联合外周动脉Fogarty导管取栓术进行治疗,无死亡病例,术后患者肢体疼痛消失。随访6个月~2年,均无心房黏液瘤复发或取栓部位黏液瘤再发种植转移,受累肢体血运良好。结论 CTA可对心房黏液瘤合并动脉栓塞作出较全面的诊断;采用心房黏液瘤切除辅以栓塞动脉Fogarty导管取栓术进行联合治疗可获得满意疗效。     

Cushing''s syndrome due to primary pigmented nodular adrenocortical disease with cardiac myxomas and mucocutaneous lentigines

A young Japanese female demonstrated unusual features of Cushing's syndrome, cardiac myxomas and mucocutaneous lentigines. At the age of 12 years she presented with growth failure and obesity. The dexamethasone suppression test, the metyrapone test and low corticotropin concentrations indicated a primary adrenal disorder. At surgery, the adrenal glands were not enlarged (the right, 4.0 g; the left; 4.5 g) but had numerous small dark brown nodules. The pathological findings showed multiple small black cortical nodules containing large cells with eosinophilic cytoplasm and lipofuscin, and internodular cortical atrophy. These abnormalities were consistent with primary pigmented nodular adrenocortical disease. At age 22 years she complained of fatigue and palpitations associated with mid-chest pain. Four cardiac myxomas, suspected from the echocardiogram, were surgically removed. Because Cushing's syndrome and cardiac myxomas are life-threatening conditions, an awareness of the complex is important.     

Cellular myxoma of soft tissue: a clinicopathological study of 38 cases confirming indolent clinical behaviour

AIMS: To characterize the clinicopathological features and biological potential of a group of soft tissue lesions with morphology intermediate between intramuscular myxoma and low-grade myxofibrosarcoma. METHODS AND RESULTS: Thirty-eight lesions in 37 patients were retrieved from the authors' consultation files. Clinical and follow-up data were obtained and the lesions were also studied immunohistochemically. Tumours occurred in adults aged 25-83 years (mean 51.9 years) with a slight predominance in females. All cases, except two, were solitary. The extremities were preferentially involved (18 lower limb; nine upper limb), with seven lesions arising around the upper (2/7) and lower limb (5/7) girdles and four lesions occurring at other locations. Twenty-nine of 31 of the tumours, for which the depth was known, were situated deep to the superficial fascia, although only 19 were strictly intramuscular. Histologically these lesions were both more cellular and more vascular than intramuscular myxoma, while lacking the cytological pleomorphism, nuclear atypia and curvilinear vascular pattern characteristic of low-grade myxofibrosarcoma. CD34 positivity in lesional cells was identified in 17/30 (57%) cases, probably reflecting their fibroblastic nature. Staining for alpha-smooth muscle actin was focally positive in 3/30 (10%) cases, while desmin and S100 protein staining were consistently negative. Clinical follow-up data (available in 22 cases; median duration 30 months) demonstrate that these lesions behave in a benign fashion with only a small risk of local recurrence if not excised completely; in this study only two tumours recurred, both of which originally had been incompletely excised. None metastasized. CONCLUSIONS: The risk of recurrence in this group of lesions which we have designated 'cellular myxoma' appears to be low. Consequently simple complete local excision is most often adequate treatment. Longer follow-up (5-10 years or more) in a larger number of cases will be important in more definitively confirming the natural history of these lesions.     

Infected cardiac myxoma

Infected cardiac myxomas are extremely rare with only forty cases described in the literature. We report a case of an infected cardiac myxoma that presented in a manner similar to bacterial endocarditis. Our case is the first to be diagnosed using previously defined criteria, and is unusual in that transesophageal echocardiography was required to make the diagnosis.     

提高心腔粘液瘤手术疗效的临床探讨

自１９８１年１１月至１９９２年６月共手术治疗心腔粘液瘤２７例；其中左房瘤２５例，右房、右室瘤各１例。手术死亡１例。随访８个月至１０年，无晚期死亡或并发症。我们认为：（１）本病潜在危险大，决定手术时不应过分拘泥于常规禁忌；（２）应用保守的小部分房间隔切除术，远期效果一样良好；（３）术中重视对受累瓣膜的检查和处理，有利于提高疗效。     

Carney complex: report of a Japanese case associated with cutaneous superficial angiomyxomas,labial lentigines,and a pituitary adenoma

We report the case of a 12-year-old female patient who manifested multiple cutaneous angiomyxomas and labial pigmented lesions. Although the familial history was not confirmed in the present case, autosomal dominant inheritance has been reported to be involved in the pathogenesis of this condition. In addition to the cutaneous complications, magnetic resonance (MR) images revealed the presence of a pituitary adenoma, which provoked an elevation of serum growth hormone (GH) level. On the other hand, no significant symptoms such as cardiac myxoma, myxoid fibroadenoma of the breast, or adrenocortical complaints suggesting Cushing syndrome, were detected. In the Japanese literature, only a few cases of this disorder have been described in the form of brief reports. There have been only a few similar cases described in the dermatological field, except for one report diagnosed as Carney complex in 1990. Therefore, the present case seems to be the first Japanese case of typical Carney complex manifesting major clinical complications, including angiomyxomas, lentigines, and a pituitary adenoma, which induced endocrine overactivity.     

Multiple cerebral aneurysms as delayed complication of left cardiac myxoma: a case report and review

Left cardiac myxoma and also consecutive embolization into the brain is well documented, whereas the association of myxomas with multiple fusiform cerebral aneurysms is rare. We analyze 33 previously reported patients and present a case of a 43-year-old woman with multiple cerebral infarctions 2 years after resection of a recurrent myxoma in the left atrium. Cerebral angiography displayed multiple fusiform aneurysms of several cerebral arteries, including a giant aneurysm of the basilar artery. Serum level of interleukin-6 (IL-6) was highly elevated. The clinical, radiological and pathological features of these aneurysms are summarized. The pathogenesis, including the role of IL-6 in the formation of myxomatous aneurysms, is discussed.     

Multiple Local Recurrent Myxoma

Cardiac myxomas, usually considered benign tumors, occasionally pursue an aggressive clinical course. The recurrence of sporadic myxomas is much less frequent than that of complex or familial myxomas (1–3% vs. 22% vs. 12%, respectively). We report a case of sporadic myxoma with multiple local recurrence in the left atrium diagnosed by transesophageal two-dimensional echocardiography 4 years after the first excision.