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Right ventricular myxoma with heart failure.

Cardiac myxomas are histologically benign tumors but they may be lethal because of their strategic position. These are mostly located in the left atrium and uncommonly in the right atrium. We report a case of a large right ventricular myxoma presenting with heart failure.

Adult↗

Cardiac metastatic liposarcoma.

Metastatic cardiac liposarcoma is extremely rare, with only 2 cases previously reported, to our knowledge; of those, only 5 involved surgical resection of right ventricular liposarcoma. The first such case in Japan involved a 61-year-old woman with metastatic liposarcoma of the right ventricle. Despite emergency resection, the patient died of severe congestive heart failure 6 days after operation. Her history included surgery for liposarcoma in the right knee 11 years previously, although it is very difficult to predict that metastasis would proceed thereafter to the cardiac cavity. This rare case suggests, however, that follow-up including examination for cardiac lesions is necessary long after resection of the primary lesion.

Female↗

A recurrent left atrium leiomyosarcoma.

Cardiac leiomyosarcoma is a rare tumour which can grow either intramurally or extramurally. Its prognosis is dismal. Surgery lengthens survival, but recurrences usually occur in the short term. Gated MRI is useful for the assessment of cardiac masses since it provides fine spatial and contrast resolution. We present the case of a left atrial leiomyosarcoma whose recurrence was diagnosed by gated MRI. Our patient underwent a second resection but died soon after.

Echocardiography, Transesophageal↗

Right intraventricular metastasis of squamous cell carcinoma of the uterine cervix: an autopsy case and literature review.

An autopsy case of squamous cell carcinoma of the uterine cervix, which developed cardiac intracavitary metastasis in a 28-year-old Filipina, is reported. At autopsy, the right ventricle contained a soft, red-purple, cauliflower-like mass. Histologically, this mass was made up of sheets of malignant squamous cells similar to the primary uterine foci. The metastases were extensive and associated with multiple organ involvement. Although this case was stage Ib at operation, vascular invasion at the primary site was characteristic, and the intracavitary tumor of the right ventricle developed without myocardial involvement. The carcinoma of the primary site extended along the inferior vena cava and settled as an intracardiac obstructive mass. A literature review (including the present case) disclosed only 14 uterine cervical carcinomas with right intracavitary metastasis. The mean age of these patients was 46 years of age (range, 28-77 years). The clinical stage was Ib in two cases, IIa in one case, IIb in six cases, and IIIb in two cases. The prognosis of these cases was poor; 13 of the patients died at an average of 19.1 months after diagnosis.

Adult↗

Right ventricular metastasis from a primary cervical carcinoma.

A 49-year-old female presented with a 1-month history of cough and low-grade fever. Echocardiography showed a large mass in the right ventricle and percutaneous right ventricular endomyocardial biopsy provided the histologic diagnosis. Despite radiotherapy and chemotherapy, the patient died. At autopsy, the metastatic deposit in the heart was larger than the primary cervical carcinoma.

Carcinoma, Squamous Cell↗

Left ventricular metastasis from uterine leiomyosarcoma.

Cardiac metastases are uncommon but seem to be increasing in incidence, possibly in relation to prolonged survival of cancer patients. Leiomyosarcoma metastatic to the heart is extremely rare. We report the case of a 57-year-old woman previously treated for uterine leiomyosarcoma who presented with dyspnea, electrocardiographic changes mimicking myocardial infarction, and normal enzymes. A left intraventricular mass, suspected as cardiac metastasis, was revealed by echocardiography. The patient died 1 week later. At autopsy the mass proved to be histologically a metastasis of the uterine tumor.

Fatal Outcome↗

[Primary cardiac rhabdomyosarcoma involving the mitral valve].

Primary cardiac rhabdomyosarcoma is rare and its extension to the mitral valve even rarer. We report a case of left atrial rhabdomyosarcoma involving the mitral valve. The patient was a 62-year-old man who complained of recurrent pre-syncopal episodes, dyspnoea often sudden in onset, asthenia and major weight loss (10 kg in one month). 2-D echocardiography revealed a 4.9 cm2 wide mass attached to the atrial side of the anterior mitral leaflet and to the adjacent inferior interatrial septum, where it seemed to have origin. CT scan and scintigraphy revealed bone, kidney and spleen metastases. The patient underwent emergency cardiac surgery because of increasing pre-syncopal and dyspnoeic episodes due to obstruction by the intracardiac mass. At surgery a tumor was found infiltrating the left atrial wall, the interatrial septum, the mitral anulus and the anterior mitral leaflet up to its tip. Invasion of mitral anulus did not allow mitral valve replacement, so that an excision of the intracardiac mass was performed as extensively as possible. Histology revealed a rhabdomyosarcoma. A post-operative chemotherapy cycle had to be stopped due to onset of atrial fibrillation and dyspnoea. 2-D echo monitoring revealed rapid new growth of the tumor across the basal portion of mitral valve leaflet to the atrioventricular orifice. After several episodes of increasing dyspnoea, the patient had a pulmonary oedema and died.

Dyspnea↗

[Diagnosis of heart tumors--contribution of 2-dimensional and Doppler echocardiography].

The advantages of the modern two-dimensional echocardiography in comparison to the M-mode one are investigated on the basis of findings in 7 patients with heart tumors. Also the possibilities of Doppler echocardiography for comprehensive information about the cardiac situation in these patients are demonstrated. It will be shown that in some patients operation can be performed entirely on the basis of echocardiographic findings.

Adult↗

[Surgical treatment of leiomyosarcoma of the left atrium Report of a case and review of the literature].

The authors report a case of left atrial leiomyosarcoma presenting with systemic disturbances and retinal emboli diagnosed by echocardiography and surgery in a 59 year old woman. Surgical ablation was completed by a course of chemotherapy. Histopathological examination confirmed the diagnosis of sarcoma; although the patient remained generally well, severe mitral regurgitation appeared 21 months after surgery. Transoesophageal echocardiography revealed an abnormal, hyper-mobile, intra-atrial echo suggesting a ruptured chordae tendinae and the mitral valves appeared very thickened and retracted. The patient was reoperated and the mitral valve replaced with a bioprosthesis. After a total follow-up of 29 months, the patients is still alive and asymptomatic. The authors underline the importance of echocardiography in the diagnosis of intra-cardiac tumours in general and, in particular, of intra-cardiac sarcomas.

Echocardiography, Transesophageal↗

Cardiac myxomas: surgical treatment, long-term results and recurrence.

Long-term results of the surgical treatment of cardiac myxomas are not completely understood due to its recurrence. We review our experience in 27 operated cases with a follow-up to 22 years (mean 6.5 years), in order to throw light on results and review the problem of recurrence. One patient had a previous recurrent myxoma. At surgery we resect all the tumors and their attachment bases. We prefer a biatrial approach, ample resection and exploration of all cardiac chambers. Hospital mortality was 3.7% (1 case of associated aortic valve replacement) and late mortality 7%. Long-term results were satisfactory, without recurrences. The postoperative life expectancy of these patients seems similar to that of the normal population, except in cases of recurrence or associated valve replacement. Recurrence is very low (4.7% in 526 cases reported in the literature), except in the case of young patients and recurrent, familial, multiple or complex myxomas. The multigrowth potential of the tumor seems more important than an inadequate surgical resection.

Adult↗

[Cerebral embolism as the first manifestation of left ventricular metastatic bronchial carcinoma].

Commonly the diagnosis of cardiac metastases in extracardiac cancer is difficult because of predominant symptoms of the underlying disease. In the reported case, a cerebral embolization was the first manifestation of a bronchogenic carcinoma with left ventricular metastasis. A 58-year-old male patient was admitted to the hospital with symptoms of an incomplete left-sided hemiparesis that occurred without prodromi. Other clinical abnormalities were not found. The two-dimensional echocardiogram showed a small space-occupying lesion in the left ventricle. Clinical symptoms completely disappeared within 3 days of heparin therapy. In order to prevent repeated embolization (after angiographic exclusion of coronary stenoses), the tumor was excised by cardiac surgery. In the histologic examination, malignant tumor cells were found. As primary origin, a small peripheral bronchogenic carcinoma with (clinically silent) additional metastases to the left adrenal gland and the right pelvic bone was found. The patient died 7 months later because of progressive metastatic cancer disease. Thus (on one hand) the reported case underlines the value of echocardiographic examination in suspected heart manifestation of cancer disease. On the other hand, in patients with heart tumors prior to curative therapy, additional diagnostic procedures (such as CT or MRI) should be performed in order to detect extracardiac tumor manifestations.

Carcinoma, Bronchogenic↗

Left atrial myxoma with severe inflammatory response.

A 59-year-old man was admitted for intermittent fever. His temperature was 38 degrees C, white blood cell counts 19,800/mm3, C-reactive protein 17.9 mg/dL. Interleukin 6 (IL-6) serum level was 31.0 pg/mL. Transesophageal echocardiography showed a 6 x 4 cm left atrial tumor arising from the atrial septum. We strongly suspected that left atrial myxoma caused the fever. The tumor was excised with the aid of cardiopulmonary bypass. Tumor histology was typical of a cardiac myxoma. The serum IL-6 level decreased rapidly in postoperative two weeks (5.3 pg/mL). In this patient, IL-6 plasma level might be used as a marker of recurrence.

Biomarkers, Tumor↗

Spontaneous resolution of obstructive valvular tumors in infants.

BACKGROUND: Myxomas, the most common cardiac tumor in adults, occur in the atria and are prone to embolization. Obstructive valvular tumors in infants occur in pulmonic and aortic valves and generally have a more benign course. METHODS: We conducted a review of our experience in infants with tumors obstructing the right or left ventricular outflow tract, originating from pulmonic or aortic valve tissue. Tissue diagnosis was available from only the index case; 4 additional infants survived with spontaneous regression, and only echocardiographic characterizations were available. RESULTS: Five cases were found with obstructing valvular tumors. In the index case, the obstruction resulted in heart failure, required surgical extirpation, and revealed histology diagnostic of a myxoma. In the subsequent 4 infants with conditions diagnosed with echocardiography, the appearance was suggestive of myxomas (small, compact, ovoid, with no fronds and no embolization). All 4 cases remained mildly to moderately obstructive and then showed spontaneous regression during a period of months to years. CONCLUSION: None of our cases deteriorated clinically, except the first, and were therefore different from other tumors, such as adult atrial myxomas, papillary fibroelastomas, fibromas, and rhabdomyomas. Only the latter are known to spontaneously regress in infancy. The prevalence of valvular tumors in infants may have previously been underestimated, given the absence of symptoms in 4 of our cases and the spontaneous regression of the tumor. An alternate diagnosis for these 4 cases could be a tissue "myxomatous rest" as opposed to a neoplastic myxoma. In either case, surgical intervention may not be necessary.

Aortic Valve↗

Occurrence of a second primary papillary fibroelastoma.

Papillary fibroelastomas are rare, benign cardiac tumours that most commonly arise from cardiac valves, and often present with signs and symptoms of embolization. A patient presented initially with transient ischemic attacks. He was found to have a papillary fibroelastoma on his tricuspid valve, which was successfully resected. Nine years later, he presented again with cerebral embolic symptoms and was found to have a new fibroelastoma on his mitral valve. The presence of a new lesion should be considered if signs or symptoms recur.

Fibroma↗