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Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.

We analyzed a series of 112 consecutive cases of left atrial myxoma diagnosed in a single French hospital (72 women and 40 men; age range, 5-84 yr) over 40 years, from 1959 to 1998. Symptoms of mitral valve obstruction, the first arm of the classic triad of myxoma presentation, were present in 75 patients (67%), with mostly cardiac failure or malaise. Symptoms of embolism, the second frequent presentation in the classic triad, were observed in 33 cases (29%) with 1 or several locations, essentially cerebral emboli with stroke. Males are statistically at greater risk than females of developing embolic complications. The third arm of the classic triad consists of constitutional symptoms (34%) with fever, weight loss, or symptoms resembling connective tissue disease, due to cytokine (interleukin-6) secretion. Younger and male patients have more neurologic symptoms, and female patients have more systemic symptoms. Seventy-two patients (64%) had cardiac auscultation abnormalities, essentially pseudo-mitral valve disease (53.5%) and more rarely the suggestive tumor plop (15%). The most frequent electrocardiographic sign was left atrial hypertrophy (35%), whereas arrhythmias were uncommon. The greater number of myxoma patients (98) diagnosed preoperatively after 1977 reflects the introduction of echocardiography as a noninvasive diagnostic procedure. However, there was no significant reduction in the average time from onset of symptoms to operation between patients seen in the periods before and after 1977. The tumor diameter ranged from 1 to 15 cm with a weight of between 15 and 180 g (mean, 37 g). The myxoma surface was friable or villous in 35% of the cases, and smooth in the other 65% cases. Myxomas in patients presenting with embolism have a friable surface; those in patients with cardiac symptoms, pseudo-mitral auscultation signs, tumor plop, and electrocardiogram or radiologic signs of left atrium hypertrophy and dilatation are significantly the larger tumors. The long-term prognosis is excellent, and only 4 deaths occurred among our 112 cases over a median follow-up of 3 years. The recurrence rate is low (5%), but long-term follow-up and serial echocardiography are advisable especially for young patients.

Aims: Primary cardiac sarcomas are rare and aggressive tumours. The aims of this study were to precisely classify cardiac sarcomas according to their pathology, and to determine their clinicopathological features and prognosis., Methods and Results: Twenty-four primary cardiac sarcomas were studied. Clinical features and follow-up of all patients were collected. Histological diagnoses were obtained by combining both morphological features as described in soft tissue counterparts and the immunoprofile of the tumours. The 24 cases were classified as undifferentiated sarcoma (nine cases), angiosarcoma (six cases all located in the right atrium), leiomyosarcoma (six cases), malignant fibrous histiocytoma (one pleomorphic and one giant cell type) and synovial sarcoma (one case). Patients included 14 men and 10 women, with a mean age of 46 years. Clinical manifestations were protean, often delaying the diagnosis. Dyspnoea resulting from obstruction of the cardiac chambers was the most common symptom. Echocardiography and magnetic resonance imaging were useful respectively to detect and to evaluate tumour extension. Complete macroscopic resection was possible in only 33% of patients. The most common cause of death was local recurrence of the tumours (50%), even in the cases of complete macroscopic resection. Whatever the treatment, the prognosis was poor with a mean survival of 16.5 months after diagnosis., Conclusions: All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.

Papillary fibroelastoma is a seldom reported tumour. It usually occurs in adults and develops on the aortic and mitral valves. It is not different of giant Lambl excrescences and differential diagnostic can be difficult with the myxoma. Its systematic surgical ablation is justified by the important risk of embolic complications. It has rarely been reported in children. We report a case peculiar by fortuitous diagnostic, tricuspid site, large size and occurrence in a 3-year old child.

After a period during which cardiac papillary fibroelastomas were considered incidental autopsy findings cerebral and coronary arteries embolism proved their aggressiveness. Echocardiography is now able to identify them and surgical resection is rapidly required. Sea anemone like macroscopic pattern is characteristic with finely villous masses, each frond being at microscopical examination formed by a central fibroelastic core surrounded by a myxomatous layer overlied by endothelial cells. Histogenesis remains elusive and we tried to clarify it by immunohistochemical analysis of 8 of the 20 cases studied (10 autopsies, 10 surgical resections). Morphological and immunohistochemical data show that endothelial cells play the most important part in abnormal formations.

Objectives: The growing number of reports of surgery for papillary fibroelastomas of the heart led us to evaluate the diagnostic potential of ultrasonography in patients with cerebral or coronary signs and to assess the efficacy of anticoagulant therapy in preventing recurrent cerebral ischemia and disease progression after resection., Patients and Methods: Ten cases of echographically diagnosed fibroelastoma of the heart treated by surgery were analyzed together with cases reported in the literature., Results: Transesophageal echography has been shown to be the superior method. Surgical resection has given good results and the postoperative course is always excellent. Recurrent embolism occurred in two of our cases despite well-conducted anticoagulation., Discussion: Surgical resection should be performed as early as possible because anticoagulation does not appear to sufficiently protect against embolic events, particularly cerebral events.

Lambl's excrescences are filliform aortic or mitral valve tumours. They do not usually cause any clinical problems and are autopsy findings. The authors report a case of a 64 year old female with an invalidating angina in whom echo and angiographic investigations suggested obstruction of the right coronary ostium by a valvular tumour. This was confirmed at surgery and tumorectomy was followed by regression of the anginal syndrome.

One hundred patients operated for left atrial myxoma in the same surgical department underwent clinical and anatomical assessment at long-term from 1959 to July 1995 (66 women and 34 men, average age 52.2 years). The clinical presentation was related to mitral valve obstruction in half the cases (dyspnoea, cough, pulmonary oedema), the presentation in the other half of cases being very variable. The widespread use of echocardiography has relegated other investigations to a subsidiary role: auscultation, radiology, ECG (9 cases diagnosed by echocardiography performed for another indication). Serious complications of left atrial myxoma include systemic embolism : 37 cases out of the 100 in this series, including 10 plurifocal but mainly cerebral (19 cases including 11 isolated cerebral emboli). Surgical treatment is well established, should not be deferred and gives excellent results (2 early postoperative deaths out of 100 cases in the early years of the study). There were 6 cases of recurrences including 3 cases of Carney's syndrome. Clinico-pathological correlations showed that mitral stenotic effects occurred when the tumour diameter exceeded 5 cm and embolism was associated with tumours having multiple villositi. Histopathological analysis distinguished between active and inactive tumours, differentiated or not, and enabled the elaboration of hypotheses on the rate of growth of the tumour and on the absence of true metastases. Histopathological techniques also show the presence of lymphoplasmocytic infiltration, the sign of secretion of interleukin 6 by the myxoma, a cytokine involved in the general inflammatory process and which explains the unusual clinical presentation sometimes observed.

A 63-year-old man presented with unstable angina. The coronary angiogram revealed a proximal left anterior descending artery (LAD) stenosis and an irregularity on the anterior wall of the left ventricle. Intraoperatively, a malignant melanoma, independent of the coronary stenosis, was identified and resected, and an internal mammary graft was inserted. No primary tumor was found. The patient is alive 18 months after operation, with a normal magnetic resonance imaging (MRI), which seems to be the technique of choice for following-up heart melanomas.

A 31 year old right handed woman presented with acute onset of aphasia which cleared over two days. CT-scan showed a left middle cerebral artery infarct within Wernicke area. Initial transesophageal two-dimensional echocardiography disclosed a mitral valve lesion suggesting a thrombus. She was discharged on oral anticoagulant treatment. A second stroke occurred ten months later involving left lenticulo-striate arteries area. Echocardiography remain unchanged. Subsequently, giant Lambl's excrescences of mitral valve was confirmed by operation and pathologic examination. The majority of patients with Lambl's excrescences are asymptomatic. However surface thrombus is common with this tumors which reposant a potential us for cerebral embolization. These tumors should be operated since complete excision is the only definitive means of eliminating the source of recurrent embolization.

We report a patient with a papillary fibroelastoma arising from the left ventricular posterior wall. The tumor was detected incidentally during echocardiography undertaken to evaluate aortic stenosis. Possible complication from tumor embolization was avoided by surgical resection during aortic valve replacement.

Ten pericardial mesotheliomas (8 of which had associated unilateral pleural involvement) were observed over a 22 year period in subjects over 50 years of age. The diagnosis was only confirmed several months after the presenting symptoms (shortness of breath, chest pain), usually by histological studies of pericardial biopsies performed during construction of a pleuro-pericardial window because of tamponade or of pleural biopsy in cases of pleuro-pericardial disease. There is no specific diagnostic feature and even modern imaging methods are unable to distinguish mesothelioma from pericardial tuberculosis. In 7 cases, there were large haemorrhagic pericardial effusions. At present, there is no effective treatment for mesothelioma and the physician's goal is to make the patient's short survival time as comfortable as possible with respect to the severe pain and recurrent pleuro-pericardial effusions. The pericardial mesothelioma is rare (less than 1% compared with 96% pleural and 3% peritoneal localisations) and possibly related to exposure to asbestos, at least in those cases with associated pleural involvement. The authors underline the utility of histological analysis of the utility of histological analysis of the pericardium if only to establish the diagnosis of mesothelioma and to enable administration of curative treatment of other pathologies (tuberculosis, malignant lymphoma) with identical clinical presentations.

In a series of 100 patients with intracardiac myxoma, the lesion was in the right atrium in 10 cases. The authors attempt to define the particular features of right atrial myxoma in terms of clinical aspects, outcome and pathology. The diagnostic difficulty raised by complex clinical pictures (including right ventricular failure, pseudopericardial forms and forms with a predominance of systemic problems) has now been resolved by imaging and, above all echocardiography. Consequences for the right side of the heart may involve the pulmonary artery with myxomatous emboli causing multiple fusiform aneurysms and pulmonary hypertension, or, more rarely, the tricuspid (with need for valve replacement), vena cava (Budd-Chiari syndrome) or atrial septum (right/left shunt). Alongside systemic problems due to interleukin 6, erythrocyte abnormalities are a new feature. Frequent etiological inclusion in a Carney complex explains recurrences and multi-cavity forms. The dominant pathological feature is tumor calcifications, no doubt as a result of the long presymptomatic phase of myxoma.

Cardiologists rarely encounter tumoral pathology, the commonest being the myxoma which presents more often as cardiac rather than neoplastic disease. A few cases of "malignant or metastatic cardiac myxoma" have been reported in the literature. We have not observed this behaviour in the follow-up of 100 cases of myxoma between 1959 and 1989. However, during the same period, 14 intracardiac sarcomas (operated or not) were seen, which were all rapidly fatal. A critical review of the cases of metastatic cardiac myxomas reported in the literature enabled us to classify them into three groups: the first, 9 cases of false myxomas. They were cardiac sarcomas and therefore a histopathological diagnostic error had been made. The second group comprised false metastases. They concerned arterial embolism of fragments of myxoma causing ischemic infarction or infiltration of the arterial wall by the myxoma, damaging the artery and resulting in fusiform aneurysm. This cannot be accepted as a true metastasis: a number of examples are given as they are too many to be named individually. The cardiac and cutaneous myxomas observed in Carney's complex may be included in this category. The third group has to be discussed case by case: these "metastatic myxomas" can all be explained logically and malignancy can be excluded; they are few in number (5 cases!) but often published several times by different authors. In our opinion, a carcinologically "malignant" metastatic myxoma remains a questionable pathological entity. This does not mean that a myxoma cannot be lethal: its intracardiac position may cause fatal valvular obstruction, or it may give rise to fatal embolism.(ABSTRACT TRUNCATED AT 250 WORDS)

The histological features of eighty cardiac myxomas removed by surgery between 1959 and 1988 were evaluated by the usual staining techniques and also by identification of immunohistological markers (vimentin, desmin, factor VIII, actin). Myxoma cells present various forms (polygonal, round, spindle, stellate) when isolated but are characterized by their tubular angioid structures: cellular masses, elementary tubes, organoid formations with several parietal layers and abundant "myxoid" matrix, denser around the angioid structures. Sometimes they are located on the surface forming crypts and vegetations, replaced in old myxomas by hyalinization. Other constituents such as vascular spaces, fibrinous exudate, hemosiderosis pigments, necrobiotic areas, various calcifications, plasma cell infiltrates are also frequently found. The insertion on the endocardium is usually an interesting structure. The presence of mucous epithelial structures and clusters of smooth muscle cells is uncommon but constitutes an additional argument in favour of the present explanation concerning its histogenosis i.e. a tumor derived from vestigial cells, usually confined to the floor of the fossa ovalis. On the basis of these various characteristics, myxomas can be classified as being active or inactive (more or less mummified), poorly differentiated or mature, which may correspond to the duration of the lesion prior to its resection. A precise histological examination of myxoma avoids confusion with more malignant myxomatous cardiac tumors.

It was sought to determine whether recurrent cardiac myxomas had special etiological, surgical and/or pathological features differentiating them from usual forms. This involved study of 6 recurrences occurring in 85 patients treated surgically between 1959 and 1989, as well as 33 well documented recurrences in the literature. True recurrent myxomas in young adults have a definite familial nature, frequently occurring within the context of Craney's complex and are due to a multicentre onset. Closer postoperative monitoring is therefore required in such cases. Recurrence of sporadic myxomas is generally due to incomplete excision and can be avoided by precautions regarding operative technique.

Left ventricular myxomata are exceptional (12 published cases). In the case reported, the clinical picture included emboli (cerebral and coronary) and syncope. Auscultation, the electrocardiogram and the phonomechanocardiographic tracings suggested an impaired left ventricular ejection, while the left ventriculography demonstrated the tumour and the coronary arteriography its vascular relationship with the right coronary artery. Cure was obtained by surgical removal. In that respect, an attempt was made to describe the clinical and paraclinical bases for the diagnosis of the myxomata of the left ventricle.