Extracardiac manifestations of atrial myxomas

Primary cardiac tumors are extremely rare and constitute only about 5% of all cardiac tumors. Cardiac myxomas are noncancerous primary tumors of the heart and constitute about of 50% of all primary heart tumors. Left-sided atrial myxomas are more common than right-sided atrial myxomas. Atrial myxomas can lead to a triad of complications. The most common symptoms are associated with obstruction due to the size and location of the tumor. The next most common symptoms are associated with pulmonary and systemic embolization. Patients may also present with constitutional symptoms. Diagnosis is made via means of transesophageal echocardiography and magnetic resonance imaging. Early diagnosis and surgical resection remain the treatment of choice to prevent complications. Patients usually have a good prognosis after resection.     

Diagnosis of mitral valve myxoma by transesophageal echocardiography

Mitral valve myxomas are rare. We report a patient with a mitralmyxoma arising from the posterior mitral leaflet in whom transthoracicechocardiography revealed equivocal findings. However, transoesophagealechocardiography provided accurate relevant anatomical informationincluding the size, morphological characteristics, and tumourattachment point.     

Bilateral atrial myxomas. Surgical correction after echocardiographic diagnosis

A case is reported of bilateral atrial myxomas diagnosed noninvasivelyby echocardiography and successfully removed. The excised tumormass consisted of a mobile right atrial myxoma prolapsing intothe right ventricle and a less mobile, not prolapsing myxomain the left atrium. The operation was performed entirely onthe basis of echocardiographic findings, which correlated wellwith the operative results. Further investigation by computertomography verified the diagnosis, but added no extra informationof importance for therapy. Diagnostic aspects of the combinedapplication of M-mode and cross-sectional echocardiography withDoppler echocardiography are emphasized. Postoperative follow-upechocardiography showed the complete removal of the myxomasand improvement of cardiac function.     

Bilateral atrial myxomas. Surgical correction after echocardiographic diagnosis

A case is reported of bilateral atrial myxomas diagnosed noninvasivelyby echocardiography and successfully removed. The excised tumormass consisted of a mobile right atrial myxoma prolapsing intothe right ventricle and a less mobile, not prolapsing myxomain the left atrium. The operation was performed entirely onthe basis of echocardiographic findings, which correlated wellwith the operative results. Further investigation by computertomography verified the diagnosis, but added no extra informationof importance for therapy. Diagnostic aspects of the combinedapplication of M-mode and cross-sectional echocardiography withDoppler echocardiography are emphasized. Postoperative follow-upechocardiography showed the complete removal of the myxomasand improvement of cardiac function.     

Echocardiographic aspects of multiple myxoma in Carney's syndrome

In Carney's syndrome, the association of cardiac myxomas, spotty pigmentation and endocrine over activity, the myxomas are usually multiple and have atypical locations. The authors report a case in which an accurate diagnosis of these multiple myxomas was made by transoesophageal echocardiography, although transthoracic echocardiography had missed the diagnosis.     

Atrial myxoma: case report and a review of the literature

Atrial myxomas are the most common benign primary tumor of the heart and occur in as many as 3 in 1000 patients. These tumors are a major cause of patient morbidity and mortality. Although the majority of atrial myxomas occur in the left atrium, 3 separate familial myxoma syndromes can result in multiple myxomas in atypical locations. Approximately 50% of patients with myxomas may experience symptoms due to central or peripheral embolism or intracardiac obstruction, but 10% of patients may be completely asymptomatic. Screening for myxomas should involve a thorough history and physical examination and a transthoracic and/or transesophageal echocardiogram. Transthoracic echocardiography is approximately 95% sensitive for the detection of cardiac myxomas, and transesophageal echocardiography approaches 100% sensitivity. Though the majority of atrial myxomas are sporadic, it is imperative that first-degree relatives of patients with documented myxomas undergo screening for occult myxomas. Surgical removal of the myxoma is the treatment of choice and usually curative; however, myxoma recurrence does occur and is most frequently associated with a familial syndrome.     

心脏粘液瘤的诊断与外科治疗（附71例报告）

共收治71例心脏粘液瘤患者。左房粘液瘤62例,其中多发瘤3例、合并室间隔缺损2例、二尖瓣关闭不全1例,右房粘液瘤9例、均由超声心动图确诊,并在全麻体外循环下手术摘除粘液瘤,同期行二尖瓣成形和二尖瓣替换术各1例,室缺修补术2例。术后2例死亡（2．8％）。随访9个月－16年,2例复发（2．8％）。认为超声心动图对心脏粘液瘤的诊断具有特殊价值,心脏粘液瘤一经诊断应立即手术,彻底切除是预防复发 的关键。     

Familial recurrent atrial myxoma: Carney's complex

We report on a family of 4 members, all of whom have had multifocal, recurrent atrial myxomas associated with skin pigmentation, melanotic schwannomas, mucocutaneous myxomas, and tumors of the ovary and pituitary, adrenal, and thyroid glands. Immunochemistry of the myxoma cells is positive for calretinin, confirming their neuroendocrine origin. Genetic studies confirmed mutations in the gene coding protein kinase A, regulatory subunit 1-α (PRKAR1α). This is Carney's complex, characterized by multiple, mucocutaneous myxomas; pigmented lesions over the lips, conjunctiva, and genitalia; adenomas of the breast and thyroid; schwannomas; and endocrinal abnormalities including Cushing syndrome and acromegaly. Members of the family require vigorous screening, including urinary free cortisol, plasma transforming growth factor-β(1) and thyrotropin-releasing hormone, testicular ultrasound, routine echocardiographic screening, searches for cardiac and mucocutaneous myxomas in multiple locations, and genetic studies for the PRKAR1α gene sequence.     

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.     

Recurrent cardiac myxoma: the surgical implications of two distinct groups of patients

15 consecutive cases where patients (11 female, 4 male) had undergone surgical excision of cardiac myxoma were reviewed. The 11 survivors were re-assessed by trans-oesophageal echocardiography and recurrent myxoma was detected in 2 patients. These two were the only patients who had originally presented with multiple myxomas. Analysis of these cases in conjunction with 26 previously reported cases of recurrence identifies two distinct groups of patients. In one group (12 cases, mean age 48.7 years) recurrence occurred at the site of previous excision, typically following the removal of a single septal myxoma by partial thickness excision. In the second group, which includes our two cases, (16 cases, mean age 30.6 years) recurrent myxoma was found distant to the site of previous excision, typically in younger patients who had multiple myxomas at original operation. These results suggest that 1) single myxomas should be removed by full thickness excision 2) in younger patients with multiple myxomas surgery should be as radical as possible.     

A rare association of pheochromocytoma and thoracic paraganglioma: utility of 2D contrast enhanced echocardiography

Primary tumours of the heart are rare. Myxomas are the most common primary cardiac tumours with an estimated incidence of 0.5 per million population per year. Myxomas are most commonly found in left atrium and are solitary in more than 90% cases. Familial myxomas are rarer and tend to be multiple. We report a rare case of multiple myxomas in the right heart which occurred sporadically, presenting with an episode of pre-syncope.     

Multiple biatrial myxomas demonstrated by two-dimensional echocardiography

The two-dimensional and M-mode echocardiographic detection of multiple atrial myxomas is described in an asymptomatic patient with a family history of myxomas. Surgery was subsequently performed without resort to cardiac catheterization studies. Echocardiographic techniques correctly delineated the number and size of the tumors, as well as their site of attachment, mobility and degree of interference with cardiac function.     

Multiple recurrences of nonfamilial cardiac myxomas: a report of two cases

Cardiac myxomas are the most common primary cardiac tumors. Surgical resection usually provides definitive treatment; however, postoperative tumor recurrence has been reported, especially when myxomas occur as part of a familial pattern. Only a few cases of 2nd recurrence of nonfamilial cardiac myxoma have been reported. We report 2 cases of nonfamilial cardiac myxoma, with multiple recurrences after surgical resection. The possibility of repeated recurrence of cardiac myxomas demonstrates the importance of regular echocardiography after surgical resection in order to detect such recurrence. Future studies, including genetic analysis of patients with recurrent cardiac myxomas, are warranted to investigate the nature of these tumors.     

Bilateral cardiac myxomas and peripheral myxomas in a patient with recent myocardial infarction.

Coronary arteriography, performed for recent anterior wall myocardial infarction in a 29 year old man, showed multiple aneurysms of the distal branches of the left coronary artery without evidence of obstructions in the major branches, and two clusters of abnormal vessels, supplying tumors in the right ventricle and the left atrium. At operation and histologic examination both tumors appeared to be myxomas. In addition, two myxomas, located elswhere in the body, could be proven histologically.     

Multicystic/cavitated giant left atrial myxomas: a matter of technology?

We present the case of a rare echocardiographic image of a giantcavitated myxoma and the pathologic findings of the cystic mass.The new echocardiographic equipment not only has improved thesensitivity for diagnosis of different pathologies but alsohas redefined its visual and morphologic characteristics. Althoughmost myxomas are solid masses and some cystic myxomas have beenreported, the presence of multiple cavities on echocardiographicexam has exceptionally been described. While cystic changeshave been described at autopsy in 14% of cardiac myxomas, itsidentification with echocardiography is rare. Nowadays, thenew echocardiographic equipment has improved the quality andthe accuracy to detect and describe intracardiac masses, showingmyxomas with cystic cavities in vivo that in the past was apathologic finding.     

Angiocardiographic symptomatology and hemodynamic disorders in cardiac myxoma

Hemodynamic and angiocardiographic findings are analysed in 10 patients with intracardiac myxomas of varying localization: right-atrial myxoma in 1, right-ventricular myxoma in 2, left-atrial in 6, and multiple right-atrial and right-ventricular myxomas in 1. The pattern of intracardiac hemodynamic disorder depends on the site of tumour and resembles patterns associated with various valvular defects. Selective angiocardiography combined with roentgenocinematography can yield the most objective information for myxoma diagnosis.     

Rezidivierende familiäre kardiale Myxome und Lentiginose

The case of a 41-year-old woman with recurrent cardiac myxomas and widespread lentiginosis is reported. The diagnosis of a Carney complex was established 7 and 25 years, respectively, after first manifestation of the cardiac myxomas in both the patient and her brother. This peculiar hereditary disease is commonly associated with multiple neoplasms and an endocrine overactivity, requiring a thorough examination of the patients and their relatives to detect additional typical manifestations.     

THE COMPLEX OF MYXOMAS, SPOTTY PIGMENTATION AND ENDOCRINE OVERACTIVITY

We describe a 40-year-old male with acromegaly, multiple and recurrent cutaneous myxomas, spotty cutaneous pigmentation, a mammary myxoma, a large-cell calcifying Sertoli cell tumour of the testis, and an unusual calcifying pigmented neuroectodermal tumour. He presented a combination of clinical and histological features not previously documented within the complex of myxomas, spotty pigmentation and endocrine overactivity, and therefore the findings broaden the spectrum of the syndrome.     

When catastrophe strikes - a case of atrial myxoma with distal embolization

Primary tumors of the heart are rare, with an incidence between 0.0017 and 0.19 percent in unselected patients at autopsy. About three-quarters of these tumors are benign, and nearly half of these benign tumors are myxomas. First described in 1845, myxomas were diagnosed posthumously until the 1950s. The clinical features are determined by their size, locations and mobility. Embolism occurs in one-third of patients, but it is rare for patients to initially present with multiple, simultaneous widespread emboli.     

Association of cutaneous myxoma, recurrent cardiac myxoma and Cushing's syndrome (Carney's complex). Description of a case and review of the literature

The authors report the case of a rare clinical syndrome which has recently been called Carney's complex or "myxomas, spotty pigmentation and endocrine overactivity". Three components of this complex are described: cutaneous myxomas, Cushing's syndrome of unpredictable evolution treated by bilateral adrenalectomy for multiple adrenal adenoma and left atrial myxoma which recurred twice. The authors review the literature and discuss the practical implications of this new syndrome which may be familial. The role of echocardiography, the key investigation for the detection of the myxoma and follow-up of these patients who have a high risk of recurrence, is underlined.