Primary left atrial leiomyosarcoma: literature review and lessons of a case

Primary cardiac sarcoma is an uncommon disease. In particular, leiomyosarcoma of cardiovascular origin is extremely rare. Half of all cardiac leiomyosarcomas are located in the left atrium. Due to the extreme rarity of left atrial leiomyosarcoma, there is no great experience in its management. This review includes a report of a case of left atrial leiomyosarcoma followed up over 45 months. The literature review examines the distribution of left atrial leiomyosarcoma, the physiological reasons for the tendency of cardiac leiomyosarcoma to be localized to the left atrial cavity, the clinical and physical appearance of this disease, and the key differences between left atrial leiomyosarcoma and the most common left atrial tumor, myxoma. The morphological features, using light and electron microscopy and immunochemical staining, are discussed. Treatment modalities including adjuvant therapy and surgical resection are examined and their effectiveness compared. Opinions regarding the results and optimal treatment of leiomyosarcoma are not always in agreement. This highlights the need for inter-hospital comparison to determine the optimal treatment regimen.     

Leiomyosarcoma of the left atrium

A 41-year-old woman underwent successful excision of a leiomyosarcoma of the left atrium. The preferential left atrial location and frequent myxoid appearance of leiomyosarcoma of the heart make it particularly difficult to distinguish preoperatively from atrial myxoma. Early diagnosis and aggressive surgical intervention with chemotherapy may improve the prognosis.     

Familial recurring cardiac myxoma

A 34-year-old woman presented with her fourth occurrence of an atrial myxoma and a right lower lung field mass. Her surgical history was extensive. In addition to resection of three previous atrial myxomas, she had undergone resection of her adrenal glands as a teenager because of Cushing's syndrome, and a hysterectomy at age 26 revealed a myxoid leiomyoma. Family history was remarkable as a maternal uncle and daughter died from embolic complications of left atrial myxomas and her sister previously had a left atrial myxoma resected. The patient underwent uncomplicated removal of the myxoma, and resection of the lung mass revealed a granuloma. A review of typical and atypical aspects of cardiac myxomas is provided including a rare and recently described syndrome of familial cardiac myxoma associated with Cushing's syndrome, spotty skin pigmentation and other myxoid tumors.     

Asymptomatic recurrence of a left auricular myxoma. Apropos of a case

A case of asymptomatic recurrence, in a 23-year old female patient, of a left atrial myxoma surgically removed 7 years previously is reported. The initial tumour, revealed by lipothymias and progressive heart failure, had been diagnosed by TM-mode echocardiography. The myxoma had been entirely removed, together with part of the atrial septum around its pedicle. The recurrent tumour was discovered by chance during routine control echocardiography. Post-surgical recurrent cardiac myxomas are uncommon (5% of the cases), and they predominate in the left atrium, like the initial tumours. They develop more rapidly and may recur several times in succession, becoming increasingly aggressive and multiple, though most of them remain benign. They are thought to result from the proliferation of myxomatous cells in clusters disseminated within the cardiac walls. Echocardiography is the key to the diagnosis; it is also used for post-surgical follow-up.     

Leiomyosarcoma of the right ventricle extending into the pulmonary trunk

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. A 70 year old woman presenting with the symptoms of progressive exertional dyspnoea and left sided pleuritic pain is presented. A leiomyosarcoma was diagnosed that originated in the right lateral ventricle wall, causing pulmonary outflow obstruction. Pathological analysis showed a neoplasm with a myxoid stroma, high mitotic activity, and nuclei expressing atypia. Immunohistochemical staining was found positive for vimentin and desmin. Seven months after complete surgical resection the tumour relapsed. This case illustrates the poor outcome, high relapse rate, and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding incidence, diagnostic techniques, treatment strategies, and survival rates of this rare but terminal disease is reviewed.


Keywords: cardiac neoplasm; leiomyosarcoma; right ventricle     

Left atrial myxoma in transplanted heart

Primary cardiac tumours are discovered in about 1 in 2000 necropsies. Cardiac myxomas make up half of all benign intracardiac tumours. Seventy five per cent are located in the left atrium. The first left atrial myxoma arising from the left atrium of a transplanted heart is reported.     

Atrial myxoma related myocardial infarction: Case report and review of the literature

Atrial myxomas are the commonest primary cardiac tumors and usually affect the left atrium. Patients with atrial myxomas present with intracardiac obstruction, embolization to the pulmonary and systemic circulation, or constitutional symptoms.The coronary arteries’ involvement in myxomatous embolization, although rare, has been described to cause acute myocardial infarction (AMI). We report a case of atrial myxoma associated MI and present the clinical and echocardiographic features of this presentation followed by review of the English literature for the association of atrial myxomas and acute myocardial infarctions (AMI).     

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.     

Neoplastic heart disease -- the Muenster experience with 108 patients

BACKGROUND: Tumours of the heart are rare. Different histological subtypes are known. The most common tumour entity is benign cardiac myxoma. Malignant heart tumours are less common. Tumours originating in other organs such as the kidney may also affect the heart by tumour progression via the inferior caval vein. A large experience with surgical treatment of different types of heart tumours is presented. METHODS: Between January 1989 and April 2004, 108 patients with a heart tumour were included in a database. All patients underwent radical surgical resection, except for 2 patients who had malignant lymphoma of the heart. RESULTS: Histological findings included 78 myxomas (72.2 %), and 6 other benign cardiac tumours in 5.6 % of the patients. Primary malignant heart tumours were seen in 10 (9.2 %) and renal cell carcinoma with cardiac involvement in 6 (5.6 %) patients. Eight patients presented with tumour metastases inside the heart (7.4 %). Mean overall survival was 12.7 years for myxoma patients and 5.6 years for patients with other benign heart tumours. Patients with primary malignant heart tumours survived 5.5 years on average. CONCLUSIONS: Heart tumours are rare, but usually life-threatening. Radical surgical resection is the therapy of choice and may offer excellent long-term survival, even in cases with malignant heart tumours.     

Malignant histiocytofibroma of the heart simulating myxoma of the left atrium

The authors report a case of malignant fibrous histiocytoma of the left atrium which was excised but rapidly recurred in a 47-year-old woman. Clinical examination, echocardiography and the absence of biochemical abnormalities were in favour of the diagnosis of left atrial myxoma. This report illustrates the clinical similarity between myxoma and sarcoma of the left atrium. Very careful anatomopathological examination of multiple sections of the tumour are necessary to distinguish it from benign tumours, especially myxomas.     

Primary cardiac tumours: eighteen years of surgical experience on 21 patients

BACKGROUND: The literature on primary cardiac tumours is relatively limited because of their rare occurrence. OBJECTIVE: To report the authors' experience with primary cardiac tumours at McGill University teaching hospitals, Montreal, Quebec. In addition, four exceedingly rare primary cardiac tumours are presented--three valve myxomas and the seventh reported case of a primary synovial sarcoma. METHODS: Twenty-one patients resected for primary cardiac tumours were retrospectively reviewed in terms of their clinical presentation, surgical treatment, histopathological findings and outcome. Postoperative success was defined by disease-free survival after resection. A survival analysis was performed using the Kaplan-Meier life-table method. RESULTS: The mean age at the time of resection was 45.5 years (range birth to 74 years). The most common clinical presentation in adults was dyspnea (38%) or central nervous system/embolic phenomena (24%), and in the pediatric group, it was hypoxia (50%). All but one of the tumours were visualized using transthoracic echocardiography. Coronary angiography was performed in 11 patients, with a diagnosis of coronary artery disease in five patients (45%), all of whom underwent coronary artery bypass grafting. The surgical approach was uniatrial in 10 patients (48%)--seven (33%) via the left atrium and three (14%) via the right atrium. Eight patients (38%) required a biatrial approach; the remaining three patients (14%) with ventricular tumours required a ventricular approach. Of the 21 cases, 17 were adults and four were pediatric cases. There were 16 myxomas (76%), two rhabdomyomas (10%), one fibroma (5%), one angiosarcoma (5%) and one synovial sarcoma (5%). In adults, myxoma was the most common primary cardiac tumour (88%), whereas rhabdomyoma occurred most frequently in the pediatric population (50%). Three valve myxomas, two involving the mitral valve and one involving the tricuspid valve, were encountered; all underwent conservative leaflet resection and valve repair. The probability of disease-free survival for all patients was 80.7% (95% CI 63.6 to 97.7) at one year, and 75.6% (95% CI 57.0 to 94.3) at both five and 10 years after resection. For adult benign tumours, the probability of disease-free survival was 93% (95% CI 79.4 to 100.0) at one, five and 10 years. There was a total of five deaths. CONCLUSIONS: The results suggest that complete surgical resection of primary cardiac tumours prolongs symptom-free survival and is associated with a low recurrence rate. Benign primary cardiac tumours have an excellent prognosis in the adult population compared with in the pediatric population (93% versus 50% survival, respectively, in the present cohort). Furthermore, conservative leaflet resection with valve repair of valve myxomas, rather than radical excision and valve replacement, should be strongly considered. Early identification and diagnosis of primary cardiac tumours are necessary for expeditious surgical resection if a cure is desired, especially when encountering malignant primary cardiac sarcomas.     

Revelation of left atrial myxoma during acute myocardial infarction

Primary tumors of the heart are rare. The majority of these tumors are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumors are sarcomas and occur preferentially in the right side of the heart. Malignant tumors are also described in the left atrium. Large left atrial myxoma size was closely related with constitutional symptoms, congestive heart failure, with syncope and auscultatory findings suggestive of mitral valve disease, whereas smaller myxoma size and irregular surface were associated with embolization. We present the revelation of an asymptomatic left atrial myxoma in a 64-year-old Italian woman during an acute myocardial infarction.     

Left hemiparesis from atrial myxoma emboli

Atrial Myxomas are benign primary tumors of the heart, arising mainly from the left atrium. Clinical signs and symptoms produced by atrial myxomas may be non-specific or result in mechanical obstruction of cardiac function, arrhythmias, and embolization. The authors present a case of a 60-year-old woman who developed total left hemiparesis resulting from left atrial myxoma embolization causing complete occlusion of the right middle cerebral artery.     

Left atrial leiomyosarcoma: A rare cause of mitral valve (pseudo)stenosis

A 54-year-old lady presented with a mass in the left atrium causing an acute mitral valve pseudo-stenosis. The tumour mass was assumed to be a myxoma. The patient was scheduled for tumour mass extirpation to clear the obstruction of the mitral orifice. Histological finding from the acquired specimen showed a leiomyosarcoma, as a rare primary malignant cardiac tumour.     

Secondary tumors of the right heart. Echocardiographic aspects. Apropos of 6 cases in the adult

Secondary cardiac tumours are rare but but are now more frequently diagnosed by echocardiography. We report 6 cases of intracardiac metastases affecting the right heart which were diagnosed by 2D echocardiography. In 3 cases, a very mobile, oval-shaped tumour was visualised within the right atrium prolapsing into the tricuspid orifice in diastole like a myxoma but associated in 2 cases with signs of invasion of the inferior vena cava. Two other non-mobile tumours were observed causing massive invasion of the right atrium and the last case was of an infiltrating tumour of the right ventricle resulting in pulmonary infundibular obstruction. In the light of our experience and a review of the literature, it is difficult to distinguish secondary tumours of the right atrium from myxomas especially when the tumours are mobile and when it is impossible to visualise a pedicle inserted on the interatrial septum or tumoral invasion of the inferior vena cava. At the ventricular level, the diagnostic signs differ according to whether there is tumoral invasion of the cavity or infiltration of the muscular wall. These cases illustrate the value of 2D echocardiography in the diagnosis of intracardiac metastases, sometimes even in the absence of clinical signs.     

Bi-auricular myxoma associated with atrioventricular dissociation in an 18-year-old boy: a case report

Primary cardiac tumours are rarely found and have an incidence of 0.3% in all open-heart surgeries. Among those, approximately 70% are myxomas, most of them in the left atrium. The reported incidence of cardiac tumours in autopsy series is 0.001-0.28%. Right atrial myxomas are uncommon, but when present they often originate from the interatrial septum, and conduction disturbances are rarely noted as an accompanying feature in this condition. We report the case of an 18-year-old boy with a myxoma in both left and right atrium associated with atrioventricular dissociation.     

Ventricular outflow tract obstruction secondary to leiomyosarcoma of the right ventricle

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.     

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.     

Primary cardiac tumours--operative treatment of 20 patients

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.     

A Japanese case of familial cardiac myxoma associated with a mutation of the PRKAR1alpha gene

Familial cardiac myxoma is inherited as an autosomal dominant syndrome. Here, we report a Japanese case of familial cardiac myxoma identified as a genetic abnormality. The mother experienced multiple recurrence of tumors in the left atrium and left ventricle 40 months after surgical resection of a left atrial myxoma. All recurrent tumors were successfully resected. Her daughter also had a solitary myxoma in the left atrium, but she had no recurrence after the operation. Both patients had lentigines in their face but no endocrine abnormality. Molecular genetic analysis demonstrated involvement of a mutation in the PRKAR1alpha gene.