Clinical Presentation of Left Atrial Cardiac Myxoma: A Series Of 112 Consecutive Cases
PINEDE, LAURENT; DUHAUT, PIERRE; LOIRE, ROBERT
From Service de Médecine Interne (LP, PD), Hôpital Edouard Herriot; and Service d’Anatomie pathologique (RL), Hôpital Cardio-pneumologique Louis Pradel, Lyon, France.
Address reprint requests to: Dr Laurent Pinède, Department of Internal Medicine, Edouard Herriot Hospital, 69437 Lyons Cedex 03, France. Fax: 33 4 72 11 75 67; e-mail: laurent.pinede@ chu-lyon.fr.
Cardiac myxoma is the most frequent “benign” tumor of the heart and presents an important diagnostic challenge (4,43,47). Myxomas may resemble many cardiovascular or systemic diseases, and can arise in any of the cardiac chambers, although 75% occur in the left atrium. Most cases of atrial myxomas are now diagnosed in living patients, allowing successful surgical extirpation (1,3,30,44,53).
Before the introduction of angiocardiography in 1951, cardiac myxoma diagnosis was made only at autopsy (22). The first successful surgical removal of an atrial myxoma was performed in 1954 (35,47). In 1959, the first M-mode echocardiogram of a left atrial myxoma was reported (16). Indeed, the introduction of echocardiography has provided an important noninvasive means of diagnosis for cardiac myxomas (55). Thus, the diagnosis of small myxomas (less than 5 cm in size and asymptomatic) is becoming easier.
We report here the experience of a French cardiovascular center in the clinical presentation of left atrial myxomas. The large number of patients in this series allowed us to perform an analytic, clinicopathologic study.
Patients and Methods
We reviewed the 112 cases of left atrial myxoma seen at 1 institution (Hôpital Louis Pradel, Lyons, France) over a 40-year period (1959–1998). In all cases the diagnosis was established before surgical removal. The patients’ medical records were reviewed, and data concerning clinical presentation, diagnostic methods, and postoperative course were collected.
Histopathologic slides were reviewed for all cases by the same pathologist (RL). Other locations where cardiac myxoma occurred during that time period were the right atrium (15 cases), left ventricle (2 cases), and right ventricle (1 case). Follow-up information was obtained by contacting either the patients or the attending physicians, and a standardized questionnaire was completed for each case.
A chi-square test, or the Fisher exact test when required, was performed for all qualitative variables, and odds ratios (OR) with 95% confidence intervals (95% CI) were computed. For ordinal variables a Wilcoxon rank sum test was used. Data analysis was performed using SAS (Statistical Analysis System, SAS Institute Inc., Cary, NC).
The variables studied in the statistical analysis were 1) sex and age, period of diagnosis (before or after echocardiography was available), delay to diagnosis (delay between the first symptoms and diagnosis), possible systematic (serendipitous) discovery; 2) size of the tumor (> or < 5 cm), tumor surface appearance (smooth or villous); 3) the main symptoms: cardiac (cardiac failure, malaise or syncope, chest pain or palpitations), embolic (cerebral, coronary, or peripheral artery), systemic (fever, weight loss, or pseudo-connective tissue disease signs), or neurologic (stroke, vertigo, epilepsy, coma, or other signs); 4) cardiac auscultation modifications (pseudo-mitral signs, “tumor plop”, nonspecific other signs); 5) possible chest X-ray abnormalities (left cardiac failure signs, cardiomegaly, pleural effusion, left atrium enlargement, visible myxoma when calcified), electrocardiogram (ECG) alterations (arrhythmias or conduction disturbances, ST-segment and T-wave shifts, left atrial hypertrophy, and nonspecific signs), or serologic test abnormalities (elevated erythrocyte sedimentation rate [ESR], anemia).
The study group included 72 women, (64% of the whole sample; median age, 53 yr; range, 23–84 yr) and 40 men (36% of the sample; median age, 58 yr, range, 5–77 yr). Forty-six patients (41% of the sample, 31 women, 15 men) were less than 50 years old, 47 (42%, 31 women, 16 men) were between 50 and 70 years, and 19 (17%, 10 women, 9 men) were older than 70. Age distribution was homogenous in men, but most female patients were between 40 and 60 years old (Figure 1).
The clinical and laboratory features of left atrial myxoma in our series are presented in Table 1. The different frequencies of clinical and laboratory signs according to sex and age are detailed in Table 2. Male patients were more at risk than females for embolic complications and for neurologic symptoms; females were more at risk than males to develop systemic symptoms such as fever; younger patients (age <50 yr) had more neurologic symptoms and cardiac auscultation abnormalities than older patients (age >50 yr).
We divided the patients into 2 groups according to the pre-and post-echocardiography periods: 1) between 1959 and 1977, when the only diagnostic methods were angiocardiography and cardiac cathe-terization, only 14 patients (12.5%) underwent surgery; 2) since 1977, with 2-dimensional echocardiography use (transthoracic, then transesophageal), about 5 patients per year have undergone surgery for left atrial myxoma (98 patients, 87.5%). The sex ratio and age ranges are similar in these 2 patient groups, but other differences can be identified (Table 3). Before 1977, most patients were diagnosed on the basis of serologic or blood test abnormalities (particularly elevated ESR) and radiologic abnormalities; chest X-ray evidence of cardiac failure or left atrium enlargement was particularly prevalent in patients diagnosed before 1977 compared with those diagnosed after 1977 (p = 0.006 and p = 0.02, respectively).
Delay before surgery
In our series, the time interval between symptoms onset and diagnosis (surgical removal) varied from 0 to 126 months (median, 4 mo). The median delay before surgery was 5.5 months before 1977, and 3 months after 1977; the difference is not significant. The median delay to diagnosis increased significantly when cardiac symptoms were present; there was no significant difference for the other symptoms (Table 4).
Initial symptoms (
For 11 patients, the left atrial myxoma diagnosis was a mere chance, or serendipitous, discovery (“systematic findings” in Figure 2). Among these patients, in 9 cases echocardiography (8 cases) or computed tomography (CT) (1 case) was performed for another indication and allowed a diagnosis of myxoma. For 2 patients, echocardiography had been systematically performed during follow-up for surgery on a right atrial myxoma.
The most common initial symptoms of left atrial myxoma in our series were related to mitral valve obstruction: dizziness and occasionally syncope, palpitations, dyspnea, cough, pulmonary edema, or congestive heart failure were observed in 58 patients. Eight patients showed atypical paroxysmal clinical manifestations: chest pain, hemoptysis, limb claudication, thoracic noise, shortness of breath, or syncope when reclining. Embolic manifestations suggested myxoma in 18 patients. Constitutional or general systemic signs appeared as initial manifestations for 17 patients: myalgia, muscle weakness, arthralgia, fever, weight loss, fatigue, and Raynaud syndrome.
Clinical features at diagnosis
All patients presented with at least 1 or more symptoms of the classic triad: 1) obstructive cardiac signs; 2) embolic signs; and 3) constitutional or systemic manifestations (See Table 1).
1) Intracardiac obstruction signs occurred in 75 patients. Pulmonary edema or progressive congestive cardiac failure (dyspnea, orthopnea) were the most common symptoms (n = 48). Malaise or syncope occurred in 16 cases. Two patients experienced cardiac arrest before surgery and fully recovered after resuscitation. Other nonspecific cardiac symptoms were present in 34 patients: cough, thoracic pain, palpi-tations, myocardial infarction due to coronary embolism. Malaise, palpitations, or thoracic pain could vary with body postural changes.
2) Signs of embolism were present in 33 patients, either with 1 (n = 25) or several (n = 8) locations. Embolic locations were the central nervous system (n = 24), in 1 case in the retinal artery, the upper and lower extremities (n = 15), and the coronary arteries (n = 4).
3) Thirty-eight patients presented with systemic or constitutional symptoms. These symptoms were obviously nonspecific, including fever (n = 22), weight loss or fatigue (n = 20), and immunologic diseases mimicking symptoms such as myalgia, muscle weakness, arthralgia, or Raynaud syndrome (n = 6).
4) Other clinical presentations
4.1) Twenty-nine patients had neurologic symptoms, particularly transient or permanent motor or sensory neurologic deficiencies (n = 23), when cerebral emboli were present (stroke). Three of these patients developed multiple cerebral aneurysms, induced by cerebral emboli. Three patients had epileptic fits, or multilocular sclerosis mimicking symptoms. Six patients also suffered from other neurologic manifestations, such as vertigo or coma. One patient with streptococcal meningitis underwent surgery for a supposed antibiotic-resistant endocarditis suggested by echocardiographic findings, but surgery uncovered an incidental noninfected myxoma.
4.2) One patient had fever and positive blood cultures for Streptococcus viridans.
4.3) Three patients were investigated for liver abnormalities connected in fact with right cardiac failure.
4.4) For 3 patients, the main symptoms were abdominal pain or hemoptysis.
Physical examination (cardiac auscultation)
On cardiac auscultation 72 patients (64%) had abnormal findings, 18 (16%) of them with postural changes or with time examination modifications. These murmurs looked like significant mitral stenosis findings: apical diastolic or presystolic murmur (n = 60, 53%). A tumor plop was heard in 17 patients (15%), all before 1989. Other auscultatory features were changes in preexisting murmurs, gallops, or modification of the first (split) or second heart sound (n = 12, 11%).
A significant association was observed between cardiac auscultation abnormalities and 1) systemic symptoms (fever, weight loss or fatigue); and 2) cardiac symptoms and signs (cardiac failure, etc.) (Table 5). Conversely, no association between auscultatory signs and embolic or neurologic symptoms was significant.
The most frequent laboratory finding was ESR (Westergren method) >20 mm/hr, found in 36 patients (see Table 1). Anemia (hemoglobin <12 g/dL in men, <11 g/dL in women) was observed in 15 patients. Leukocytosis (white blood cell counts >10,000/mm3) or thrombocythemia (platelet counts >500,000/mm3) was present in 6 patients.
The association between these serologic abnormalities and systemic manifestations was significant (see Table 5). However, there was no significant association between serologic signs and cardiac, embolic, or neurologic symptoms.
Two-thirds of the patients (n = 69) had abnormal electrocardiographic findings (see Table 1). Left atrial hypertrophy was the most frequent ECG sign (n = 39). Atrial arrhythmias (atrial fibrillation or flutter) or conduction disturbances were rare. Other electrocardiographic findings were ST-segment abnormalities (n = 24) or nonspecific ECG abnormalities (n = 27): ventricular hypertrophy, micro-voltage, extrasystoles.
The association of ECG abnormalities with systemic manifestations and cardiac signs was significant (see Table 5). There was no significant association with embolic or neurologic symptoms.
Chest X-rays were abnormal in 56 patients (see Table 1). Nonspecific findings included pulmonary interstitial markings or congestive heart failure signs (n = 30), and nonspecific cardiomegaly (n = 35). Left atrial enlargement (n = 12), calcifications that made the tumor visible (n = 10), and pleural effusion were unusual (n = 8).
The association of radiologic abnormalities with systemic and cardiac manifestations was significant (see Table 5). Chest X-ray abnormalities were significantly less frequent in patients with embolic or neurologic signs.
Before 1977, all cases were diagnosed by angiocardiography with left heart catheterization (n = 14, 12.5%). After 1977, echocardiography was the usual diagnostic method (n = 96, 85.7%). Initially, only transthoracic M-mode echocardiography was available (Figure 3). Later, 2-dimensional imaging and particularly transesophageal echocardiography became the preferred methods in many cases. For 2 patients (1.8%), the diagnosis was established by CT or magnetic resonance imaging (MRI).
The pathologic diagnosis of myxoma was obtained after surgical tumor resection for all cases. On gross pathologic examination, the tumor diameter ranged from 1 cm to 15 cm. The precise size was available for 104 cases: it was greater than 5 cm in 62 myxomas, and smaller in 42 (in some cases tumors had been fragmented during surgery). The weight of the tumors ranged from 15 g to 180 g (mean, 37 g). In our series 85% of the myxomas were pedunculated with a short stalk (<1 cm), and 15% were sessile. Of the left atrial myxomas in our series, 80% arose around the septal fossa ovalis margin and 5% from the posterior left atrial wall, with only a few other locations: external wall (3%), superior wall (3%), infero-medial wall (3%), left auricle base (3%), posterior commissure (2%), and small mitral valve (1%).
The myxoma surface was studied in 96 cases: it was friable or villous in 34% of these (n = 33) and smooth (Figure 4) in the other cases (n = 63, 66%). The less common villous or papillary (Figure 5) myxomas had a surface that consisted of multiple fine or very fine villous, gelatinous, and fragile extensions, with a tendency to fragment spontaneously (Figure 6).
The histopathologic findings were based on the presence of characteristic myxoma cells embedded in an amorphous myxoid matrix, composed of an acid-mucopolysaccharide-rich stroma. The myxoma cells had a spindle or stellate shape, an ovoid nucleus, and a pink eosinophilic cytoplasm; they were scattered throughout the matrix. The surface of the tumor was covered by a single layer of flat endothelial-like cells. Calcification was observed in 9% of the cases.
We differentiated histologic “active myxoma,” with a dense myxoma cell population, from “inactive myxoma,” with a sparse cell infiltrate and sometimes calcification or even ossification. Also, we distinguished different levels of histologic differentiation: 1) “normal differentiation” (Figure 7), with numerous rudimentary or well-formed vessels surrounded successively by cells then by condensed matrix; 2) “poor differentiation” (Figure 8), with many isolated, dispersed cells or lepidic cells gathered in short chains throughout the matrix. Sixty-four percent of the cases were “active” left atrial myxomas, half of them with poor and half with normal differentiation; 36% were “inactive” myxomas (two-thirds with poor differentiation). All recurrences were observed in patients with active myxoma and poor differentiation.
No significant association could be found between myxoma size (< or > 5 cm) and diagnosis circumstances (asymptomatic or symptomatic cases); villous surface; or presence of embolic, neurologic, systemic, or serologic symptoms or signs (Table 6). However, a myxoma size >5 cm was significantly associated with cardiac symptoms, auscultatory signs, and ECG and chest X-ray abnormalities. Diagnosis of larger myxomas paradoxically appeared to be more difficult, and the delay to diagnosis was shorter for small tumors than for large tumors (see Table 6).
Villosity was associated significantly with neurologic symptoms, embolic complications, and cardiac auscultation abnormalities. However, there was no significant association between villosity and cardiac, systemic, serologic, ECG, or X-ray signs or symptoms; serendipitous discovery; or delay in diagnosis (Table 7).
All patients had surgery. Four patients (3.5%) died during the perioperative period: 1 patient died in 1967 during a second surgical intervention for a recurrence of myxoma 6 years after the first surgical removal; a 66-year-old woman, operated on in 1965 with severe cardiac insufficiency and instable hemodynamic function, died on the second postoperative day from acute renal insufficiency; a 5-year-old boy died in 1978 from acute mitral insufficiency by a mitral annulus rupture; and a 73-year-old man died in 1996 from bacterial meningitis concomitant with the myxoma, whereas the incidental tumor had been removed successfully.
Only 1 patient needed a prosthetic mitral valve replacement: bacterial endocarditis was associated with the myxoma. The most frequent postoperative complication was transient arrhythmias (supraventricular arrhythmia or atrial fibrillation) in 29 patients (26%). A permanent cardiac pacemaker implantation for persistent bradycardia was necessary for only 2 patients.
During the postoperative course, 13 patients (11.5%) were lost to follow-up: foreign patients who returned alive to their native country and did not answer our questionnaire. Among the other 99 patients, 4 died and 95 (96%) were alive during the follow-up period. Patients who presented with peripheral arterial embolization fully recovered. The longest follow-up for patients alive with multiple cerebral aneurysms is 14 years (a woman suffering a stable cerebellum syndrome). In the present series, survivors were followed for a median of 3 years (range, 1 mo-17 yr).
Six patients (5%) had a recurrence of myxoma. Three patients with sporadic myxoma relapsed in the left atrium, (2.5, 8, and 17 years later, respectively). Three other patients had Carney complex. One of them suffered 2 recurrences in the left atrium, 16 and 26 months later, respectively. The other 2 patients with Carney complex had an initial myxoma in the right atrium; for 1 of them the recurrence was localized in the left atrium 3 years later, and for the other the recurrences were multiple in the left atrium (0.5 and 3.5 years later, respectively) and then in the right atrium 6.5 years later.
We report here the largest series of patients with left atrial myxoma consecutively diagnosed in a single institution, to our knowledge. The patients presented over a 40-year period.
Our 2:1 female-to-male ratio is consistent with other studies, in which this ratio varies from 2:1 to 3:1 (35,47,55). Myxomas can occur in all age-groups but are most frequent between the third and sixth decades of life (13,30,44,47). They occur predominantly in adult women (70%), although currently there is no explanation for this female predominance (perhaps due in part to hormonal influence). In our series, younger age is significantly associated with neurologic signs or cardiac auscultation abnormalities, and gender is associated with neurologic or embolic symptoms in men and systemic symptoms in women.
The clinical features of these tumors are determined by their location, size, and mobility; there are no pathognomonic signs and symptoms that suggest the presence of a myxoma (35,47,55). In a minority of cases, there are no symptoms at all (47) (10% in our series). However, there is no significant association between the size of the tumor (< or > 5 cm) and the circumstances of discovery (symptomatic or asymptomatic). Left atrial myxomas become symptomatic when they obstruct the mitral valve, embolize peripherally, or cause systemic effects:
1) Obstructive symptoms (valvular ball-valve obstruction) occur in 54%–95% of patients (35,55), 67% in our series. Many cases masquerade as mitral valve disease, but, conversely in mitral stenosis, atrial fibrillation and history of rheumatic fever are uncommon (47); only 9% of patients had arrhythmias in our study. Cardiac obstruction can be revealed by unusual, rapidly progressive congestive cardiac failure (dyspnea on exertion or orthopnea), or by malaise, syncope, or sudden death (due to complete mitral valve obstruction by the myxoma or coronary arteries embolism). The extent of valvular obstruction may vary with body position, and these symptoms’ postural changes are particularly suggestive of myxoma (35,47). Cardiac failure remains the most frequent manifestation (43% in our series). In our study, the presence of cardiac signs paradoxically increased the diagnosis delay, probably because one tends to believe that clinical symptoms are explained by another more common disease (myocardiopathy due to hypertension, ischemic processes, etc.).
2) Systemic emboli are the second arm of the classic triad, occurring in 10%–45% of myxoma patients (4,35,55), 29% in our study. More than two-thirds of myxomatous emboli migrate to the central nervous system (28,50) (24/33 in our series), but any arterial bed may be affected, leading to a great variety of symptoms and signs. Recorded cases document emboli in the upper and lower extremities, aortic saddle, coronary arteries, kidneys, liver, spleen, eye, skin, and more (47). Cerebral emboli may lead to numerous fusiform aneurysms, described as typical of cardiac myxomas (48), as in 3 patients in our study, whose long-term outcome was good (follow-up: 14, 12, 8 yr, respectively). We demonstrate that the presence of an irregular or friable (villous) surface is significantly associated with cerebral and peripheral embolisms.
3) Constitutional signs are the third arm of the classic triad (8,47,55). While these symptoms are rarely prominent, they may occur in up to 90% of cases (35). The prevalence is lower in our series (34%), perhaps because we studied a population at a cardiovascular institute. These signs and symptoms include myalgia, muscle weakness, arthralgia, fever, weight loss, and fatigue. They are obviously nonspecific and may occur in a variety of infectious (endocarditis or rheumatic fever), malignant, or immunologic diseases (rheumatoid arthritis, vasculitis, connective tissue diseases). Nonspecific cutaneous manifestations have also been reported in myxoma patients (18,31).
Cardiac auscultatory findings may vary between examinations, and may depend on body position. Apical presystolic or diastolic murmurs, suggesting significant mitral stenosis, may be heard in more than half of the patients (47,55), 54% in our series. The specific auscultatory finding of myxoma is called “tumor plop”: it is a protodiastolic heart sound of low frequency heard 0.08–0.15 seconds after the second heart sound (21,35). It may be confused with a third heart sound or a mitral opening snap. The tumor plop is uncommon, found in only 15% of patients in our series. We established a significant association between cardiac auscultation abnormalities and cardiac or systemic symptoms.
Serologic and hematologic tests are abnormal in only one-third of the patients (47,55), usually those presenting with systemic symptoms (a significant association was found in our study). The main but nonspecific laboratory abnormalities are anemia and elevated ESR, serum C-reactive protein, or globulin level. Anemia is generally normochromic or hypo-chromic; hemolytic anemia may occur by erythrocytes mechanical destruction on the tumor; polycythemia has also been reported. Less common findings are leukocytosis and thrombocythemia. Recent findings suggest that the production and release of a cytokine by the tumor itself may be responsible for systemic inflammatory and autoimmune manifestations (27). Interleukin-6 (IL6) has been implicated, with high serum levels of IL6 in patients with myxoma that return to normal after surgical removal (51). A correlation between the size of the tumor and the serum level of IL6 has also been suggested (54). Increased IL6 mRNA levels have also been found in myxoma tissue (52), probably explaining myxoma tissue infiltration in some cases by lymphocytes and plasma cells.
ECG findings are nonspecific (35,47,55) but frequently observed (62% in our series). Left atrial hypertrophy occurs in one-third of patients, reflecting the hemodynamic alterations caused by the tumor. Atrial fibrillation is uncommon (less than 10% of cases) in contrast to the findings in mitral valve disease. We observed a significant association between ECG signs and the presence of cardiac or systemic symptoms.
Chest X-rays also reveal nonspecific features in half the patients (35,47,55) and are not helpful for diagnosis, except when the tumor is heavily calcified (9% in our study). Otherwise, a left atrium enlargement and signs of pulmonary congestion may be revealed. In our series, chest X-ray abnormalities were significantly associated with cardiac or systemic symptoms, but there were fewer radiologic signs when the myxoma was revealed by embolic or neurologic manifestations. In fact, cardiac signs or symptoms are more prevalent with large tumors, and one may hypothesize that friable, embolic tumors becoming symptomatic are diagnosed before the reach the size (about 5 cm) that leads to cardiac and radiologic signs. Our data suggest a positive association between small tumor size and embolic frequency (see Table 6).
Echocardiography is the screening and diagnostic method of choice (36,47,55). It is accurate, reliable, noninvasive, and it does not entail any risk of tumor fragmentation and embolization (unlike angiography and cardiac catheterization). Transthoracic examination may be extended by the transesophageal approach (17,59). Transthoracic echocardiography is less invasive, with an excellent sensitivity, up to 95%(41,42), but transesophageal echocardiography sensitivity reaches 100%(17,39). Currently, the decision for surgery is generally based only on echocardiographic evidence of the disease; in our series angiocardiography has not been used since 1977. However, coronary arteriography in patients over 40 years old is usually required in order to rule out concomitant coronary artery disease (47). Other potential diagnostic methods include CT (40) and MRI (20,45). The advantage of CT and MRI over echocardiography is that they provide sectional views of mediastinal, pulmonary, and thoracic structures. They are also more accurate in assessing tumor attachment, endocardial site localization, and tumor stalk presence and size (45).
Anatomic data indicate that most left atrial myxomas arise from the atrial septum, usually near or around the fossa ovalis margin (14,19,55). Tumor mobility depends on the extent of attachment and on the stalk length. Gross appearance is generally white, gray-white, yellowish, or brownish with a polypoid, round or oval tumor, pedunculated or sessile. Tumor consistency is gelatinous (7), and the surface is either smooth or villous (66% and 34%, respectively, in our study). Smooth-surfaced myxomas usually appear with constitutional symptoms, while friable, irregular, or villous tumors usually embolize (30). In the literature (7,30), histologic findings such as fibrosis, calcification, and smooth surface are seen in older people, suggesting that they represent degenerative phenomena. In our series, there was no significant association between villosity and age. The size and weight of myxomas vary, but in our study, the mean duration of symptoms was paradoxically longer in patients with larger tumors.
When, based on histologic appearance, we could distinguish active or inactive myxomas and normally or poorly differentiated myxomas according to the aspect of the cell infiltrate (33), we found that all recurrences actually occurred in patients with “active and poorly differentiated” myxomas.
Many hypotheses regarding the origin of cardiac myxomas have been proposed (5,14,19). The current opinion of most authors is that myxomas are benign neoplasms of endocardial origin (24,32). It is no longer believed that myxomas are organized thrombi. Some myxomas may have a complex of pleomorphic cells that at first view may be considered malignant. However, the rare presence of multiple surface cell layers or mitotic activity is not associated with recurrence or any particular mode of presentation, and it is important not to predict an aggressive behavior on the basis of these atypical cells (7). Conversely, “myxoid imitators,” which are true cardiac sarcomas, must not be confused with myxomas (2).
The cells giving rise to the tumor are considered to be multipotential mesenchymal cells, and immunohistochemical analysis (5,7) has shown that myxoma cells express endothelial markers (60) as tumor vessel cells (QBEnd = CD34 and factor VIII-related antigen). Tumor cells are vimentin-positive, and some may be smooth-muscle-actin positive. Positivity for S-100 protein and cytokeratin (scarce cases with glandular structures) is rarely observed, as is the histocytic marker KP-1 (7,24). Burke (7) showed that endothelial differentiation was best demonstrated by CD34 positivity, which is also a sensitive endothelial marker for Kaposi sarcoma. This corroborates the concept that myxomas arise from primitive stromal cells having the capacity to differentiate along endothelial lines. A recent study (29) confirms that myxomas produce vascular endothelial growth factor, with a correlation between tumor size and the expression of this factor (suggesting a role in angiogenesis).
Myxomas may “metastasize”(6,49), commonly to the brain, but these “metastases” do not have any malignant potential (32). The myxoma tissue emboli may remain intraluminal; may penetrate the vessel wall and may cause stroke; or may remain asymptomatic, but without any cerebral tissue invasion. However, years later, the vessel walls myxomatous invasion may lead to formations resembling mycotic aneurysms (2). Three patients in our series had embolic implants that grew into the surrounding arterial walls, with development of multiple intracranial aneurysms, without any detectable recurrences or malignant behavior elsewhere during long-term follow-up.
Treatment and follow-up
The treatment of choice for myxomas is surgical removal (3,12,15,37,38,57). Once the diagnosis is made, urgent excision of the tumor is essential. Embolic complications or sudden death are always possible, even in asymptomatic patients: 8% of patients in some studies (1,34,58). During the time of our series, we did not observe sudden death due to cardiac myxoma, but 2 patients had cardiac arrest in the hospital before surgery with successful resuscitation. With current operative techniques, surgery is generally a safe procedure, and, as most tumors are usually pedunculated, they can be removed easily (57). The operative procedure may differ according to surgeons’ habits (15,25,34,38). In our series, mortality was low. Only 3 patients died during the postoperative period before 1978, and another patient died in 1996 of meningitis not imputable to myxoma. The most frequent postoperative complication was early transient arrhythmias or atrioventricular disorders, with an incidence of 26%.
Cardiac myxomas usually occur sporadically, but recurrent or familial cases have been described. The reported recurrence rate ranges from 5% to 14% in the literature (4,15,23,35,38,39), 5% in our series. Recurrences are reported from as soon as a few months to as long as 8 years after excision of the myxoma (0.5–6.5 yr in our series). The cause of recurrence is not clear, but probable risk factors include inadequate or incomplete resection, intracardiac implantation, intraoperative displacement of tumor material, embolization, multicentricity of the tumor, and/or a reserve of tumor precursor cells in the subendocardium. In most cases of sporadic myxoma, incomplete resection of the attachment area seems to be the cause of recurrence (30,34). Patients with genetic abnormalities (11,56) are at high risk of recurrence and multifocal tumors. They usually suffer from 2 or more of the following conditions: cardiac and skin myxomas (single or multiple), spotty pigmentation, cutaneous lentiginosis, myxoid fibroadenomas of the breast, endocrine overactivity (pituitary adenomas, primary adrenal hyperplasia with Cushing syndrome) and testicular tumors (9,10). They are described as having “Carney complex” or “myxoma syndrome.” The overall risk of recurrence for familial and Carney complex myxomas is about 12% and 22%, respectively (56), whereas it is only 1%–3% for sporadic tumors (47). In our series, 3 cases fulfilled the cardiac myxoma “Carney complex” criteria. At the time of diagnosis, familial myxoma patients are usually considerably younger than nonfamilial myxoma patients. Histologically, myxomas of the 2 patient groups are not different. A gene linked to Carney complex has been identified recently (26).
The long-term prognosis is excellent. In the literature (25,34,38,57), the operative mortality rate is low (0–3%), and hospital mortality after excision of intracardiac myxomas ranges from 0 to 12% (3.5% in our study). Transient supraventricular arrhythmias may follow surgical removal (26% in our series). A few patients may require permanent cardiac pacing for atrioventricular conduction disturbances (only 2 patients in our series). In spite of the presence of a tumor that interferes with mitral valve function enough to result in heart failure, the mitral valve is rarely damaged (wrecking ball effect). In our study, only 1 patient required mitral valve replacement, for bacterial endocarditis associated with left atrial myxoma. However, despite the low recurrence rate, myxoma patients need to be followed carefully, especially familial, younger, and Carney complex cases. An annual echocardiogram is recommended over the 3-to 6-year period following surgery, at least.
The differential diagnosis, besides mitral valve disease, encompasses other benign and malignant primary heart tumors (lipomas, papillary fibroelastomas, fibromas, sarcomas, etc.), metastatic tumors (20–40 times more common than primary tumors) and thrombi, which occur in patients with myocardial infarction or dilated cardiomyopathy (47). When systemic symptoms are present (fever, weight loss, fatigue), the differential diagnosis should consider rheumatic heart disease, subacute bacterial endocarditis, and connective tissue disease or vasculitis (8,35). The combination of embolic events and constitutional symptoms is due more commonly to infective endocarditis, but may also reveal myxoma. The presence of splenomegaly or septic syndrome helps to differentiate the 2 diseases, as these are extremely rare in myxoma. However, myxomas are occasionally infected, making the clinical differentiation of myxoma and endocarditis even more difficult (47). An exhaustive review of infected cardiac myxomas (46) reported 40 cases with a presentation often similar to subacute bacterial endocarditis. There is no clear clinical distinction between infected and noninfected myxoma (for cardiac symptoms and the rate of systemic or cerebral emboli); however, constitutional symptoms increase significantly in patients with infected myxoma (fever, elevated ESR), and men are more often affected than women.
Our series of 112 cases of left atrial myxoma allows some useful clinical remarks. It illustrates that this benign tumor may cause a wide range of clinical symptoms not only such as cardiac disease, but also infective, immunologic, or neurologic diseases. Diagnosis is rarely made only on clinical grounds, because there are no specific physical signs or symptoms. Myxoma usually occurs sporadically, but familial or recurrent cases have been reported. Recent findings suggest that production and release of the IL6 cytokine by the tumor may be responsible for inflammatory or autoimmune manifestations. Two-dimensional echocardiography, including the transesophageal approach, is the technique of choice for diagnosis and follow-up of this tumor. Although myxomas are rare, they should be considered in the differential diagnosis of mitral valve disease and congestive heart failure, especially in patients with sinus rhythm. Echocardiography is also indicated for any patient with embolic phenomena. An embolic event in young adults, in the absence of signs or symptoms of endocarditis or arrhythmia, must lead to a primary consideration of myxoma as the embolic source. The material obtained by embolectomy should always be examined histologically. Further biologic studies are needed to understand better the clinical features of myxoma; in particular, adhesion protein in myxoma cells or matrix should be studied for embolic myxomas (villous surface). Immunohistostaining with detection of potential estrogen receptors could be performed to attempt explanation for the high female-to-male ratio and age distribution.
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.
The surgical removals were performed during the 40-year period by Drs Barral, Champsaur, Chassignolle, Dureau, Frieh, Jegaden, Maret, Marion, Michaud, Mikaeloff, Ninet, Termet, Villard.
We are indebted to Dr Alison Foote for editing the manuscript.
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