Home SVCC Area: English - Español - Português
Primary Tumors of the Heart
Raúl A. Breglia, MD
Hospital Nacional de Clínicas,
Universidad Nacional de Córdoba,
Metastatic tumoral compromise of the heart is relatively frequent and it is generally located in the pericardium on tumors originated mainly in lungs, mammas, digestive system, and also in patients with leukemia and lymphomas (1,2).
On the other hand, primitive cardiac tumors have a very low incidence. According to different autopsy reports their incidence varies from 0.001% to 0.28% and are in most cases (75%) benign (3-5). Diagnosis is usually difficult since clinical manifestations are varied and sometimes patients may be asymptomatic or present nonspecific symptoms. Before the advent of modern imaging, cardiac tumors usually constituted autopsy findings or verifications related to cardiac surgery. In the past decade, modern procedures substantially modified this perspective making diagnosis remarkably easier. Today, there are permanent case reports in the universal medical literature, which make possible a programmed surgical approach to treat these tumors.
GENERAL CHARACTERISTICS OF PRIMITIVE CARDIAC TUMORS
Generally, cardiac tumors may not present specific clinical manifestations. Frequently, patients are asymptomatic as long as the tumoral masses do not become large. In these cases, cardiomegaly, abnormal cardiac contours at chest radiography or an incidental verification of an abnormal image on a routine echocardiogram can be observed. When determining symptoms, the tumor's size, as well as its localization -related to the pericardium, intramural or protruding into any of the cardiac chambers- its firmness or friability, its degree of invasiveness, its potential growth, etc, are important. When the tumors determine the presence of symptoms, these are generally nonspecific and suggest myocardial, pericardial or valvular dysfunction (6,7). There may also be symptoms of heart failure (frequently dyspnea), arrhythmia or embolic manifestations to the cerebral, coronary and systemic beds. Tumors originated in the pericardium (mesotheliomas) or which invade it from the myocardium cause effusion, which usually leads to tamponade. Myocardial lesions may affect the conduction system of the heart resulting in arrhythmia. Intracavitary tumors obstruct blood flux into the respective ventricle and produce systemic or pulmonary congestion. If these tumors present a pedicle which allows them to move, there may be changing signs of valvular obstruction, sometimes influenced by postural changes, as in the case of atrial myxomas. When intracavitary tumors cause embolism, these are produced by the detachment of small friable tissue fragments or by the fragmentation of thrombi originated in their surfaces. Patients with atrial myxomas usually present general constitutional symptoms. Other malignant tumors may present fever or weight loss.
DIAGNOSIS APPROACH OF PRIMARY TUMORS OF THE HEART
Usually, as in the case of the specific clinical manifestations, small tumors do not produce radiologic abnormalities in the size and configuration of the cardiac contour. The size and location of the tumor influence the respective findings. Intramural tumors usually produce cardiomegaly or variable deformations of the cardiac contour. The enlargement of a particular cavity is related to the predominant effects of the tumor on each of them. Atrial myxomas mimic mitral valvular stenosis and that determines a radiological image suggestive of that entity. Similar radiological images may be produced by sarcomas located in the left atrium. Calcic images can be perceived in the direct radiography in cases of fibromas, teratomas, osteosarcomas, etc. Tumoral compromise of the pericardium causes effusion and its common radiological images. Other radiologic findings may consist of congestion of pulmonary vasculature or tumoral thickening of the mediastinum.
Transthoracic echocardiography is usually the first elective procedure (9-11). Besides its noninvasive nature and its accessibility, it facilitates the study of the cardiac morphology from different angles, the mobility of its structures and a considerable spectrum of its functioning. However, as single method is not enough to carry out a complete evaluation of all the cases. Transesophageal echocardiography is very useful for a more detailed evaluation (12). With these techniques tumors are displayed as masses variable in sizes and characteristics. Myxomas can be easily recognized by echography through the finding of lobular echogenic masses attached to the endocardial surface, sometimes with wide mobility and clear prolapse through the respective atrioventricular valves. Doppler echocardiography may also display stenosis or associated valvular failure. Fibromas are represented as isolated masses with variable echogenicity and occasional calcifications. Unlike fibromas, rhabdomyomas generally appear as multifocal masses and never display calcifications. Teratomas characteristically appear as complex intrapericardial masses located in the right side of the heart, including multilocular cysts and calcifications, with frequent effusion in the pericardium. Sometimes its pedicled attachment to the base of the aorta can be visualized. The finding of calcifications is also characteristic of osteosarcomas.
Axial Computed Tomography (ACT) and Magnetic Resonance (MR) can provide very useful additional information for a better characterization of the tumor (13-17). ACT does not only facilitate the study of the heart but it also allows for the study of the adjacent mediastinum and thus the extracardiac effect of the tumors. It also facilitates evaluation of soft structures and the recognition of calcifications and fatty tissues, all of which may result very useful as a complement of echography. ACT does not provide real time images like the echocardiography and it is not useful for the analysis of valvular mobility. MR is better than ACT for the characterization of soft tissues and is very useful for the evaluation of intramural masses.
The distinction between primitive or metastatic cardiac tumors must be done according to the general clinical context of the patient. Regarding the benign or malignant character of primary tumors, these can display invasive and infiltrating images, compromise of more than one cavity, mediastinal invasion, progressive growth or distant metastasis, although the distinction is frequently made at the time of surgery or necropsy. Some malignant tumors that typically affect the left atrium, as in the case of fibrosarcomas or leiomyosarcomas, can usually be confused with myxomas. As for the tentative characterization of the nature of the tumor, is important its localization -in relation to endocardium (intracavitary), myocardium or pericardium-, its single or multiple form, its location on a certain cavity, etc, according to the characteristic for each of the particular tumors described below.
SPECIFIC FORMS OF PRIMITIVE TUMORS OF THE HEART
Primary cardiac tumors originated in cardiac structures can be classified as follows, in relation to the type of tissue from which they arise ().
I- Benign tumors
a) Myxomas. They represent the most common primary tumor of the heart (30% to 50% of all cases) and they constitute the most common clinical variety (18-22). Myxomas affect patients of all ages (3-83 years), predominantly women, sometimes with familiar predisposition. They are gelatinous masses (myxomatous), lobulated, attached to the endocardium via a variable sized pedicle or by a wide base that project towards the interior of the cavity without infiltrating the underlying tissues. Myxomas are frequently found in the left atrium (75%-80%) specially associated to the fossa ovalis. They are less frequently found in the right atrium and rarely in the ventricles.
shows a soft surface, whitish and friable polypoid formation of 9 x 5.5 x 3 cm, in a 38-year-old man with left atrial myxoma presenting mitral valve obstructive symptoms and signs. A cut section showed hemorrhage and other areas of mucoid appearance.
Tumoral tissue presents a mucoid and gelatinous matrix constituted of mucopolysaccharide acids, with dark hemorrhagic areas and other whitish areas of fibrous tissue.
and show histologic images corresponding to surgically excised atrial myxomas. A proliferation of fusiform well-differentiated cells found in small groups is observed. A lax stroma of myxomatous appearance may also be visualized. There are leukocytes with histiocytes containing brown pigmentation like hemosiderin. There are several small vessels and signs of erythrocyte extravasation.
Many patients with myxomatous tumors are asymptomatic and when clinical symptoms are present, they are usually related to the tendency of the tumor to cause valvular obstruction -in predominantly fibrous tumors-, or embolism -in polypoid tumors with greater myxomatous structure. Obstructive symptoms are present in half of the cases and are usually associated to mitral or tricuspid valve, simulating organic stenosis. Symptoms depend on the intracavitary motion of the tumor, sometimes existing in certain tumors with wide mobility a clear relation to body postural changes.
Two view echocardiography image, apical window, of a left atrial myxoma is shown in . In "A" a tumor obstructing the mitral orifice during diastole is observed. In "B", in systole, the tumor moves towards the upper part of the left atrium.
Transesophageal biatrial echocardiogram view (upper left atrium) in shows an ovoid mass attached to the interatrial septum though a wide base corresponding to a left atrial myxoma.
Echocardiography image of a left intraatrial myxoma with considerable motion in the different stages of the cardiac cycle is shown in (two-chamber view echocardiography, apical window).
demonstrates the utility of other techniques for recognition of cardiac tumors. In this case, MR show left atrial tumor attached to the interatrial septum in the "ostium secundum" region corresponding to a myxoma.
Emboli are specially produced in irregular tumors in whose surface thrombus are more likely form, though they may also be originated in detachments of the tumoral tissue itself. Embolic symptoms of myxomas are less frequent than obstructive and they are related to cerebral, vascular, coronary, renal and splenic beds and also to limbs beds. In some cases, right atrial myxomas may produce pulmonary embolism (23,24). Complete obstruction of mitral or tricuspid orifice may, occasionally, cause death.
Some patients also present general unspecific symptoms such as arthralgia, myalgia and muscular tenderness, fatigue, fever, splinter or subungual hemorrhages, Reynaud phenomenon, hyperagammaglobulinemia, etc. These symptoms frequently mimic those of infectious endocarditis and have been related to autoimmune mechanisms possibly associated with plasmatic increase of interleukin-6 from myxomatous tissue (25,26).
Although myxomas generally appear as unique tumors located in the left atrium with scarce tendency to relapse after surgery (1%-3%), some patients show a different biological behavior. They are generally young individuals, most frequently men, with certain familiar tendency to develop myxomas. In these patients, tumors acquire multiform character, appear in cardiac chambers different from left atrium, evidence higher tendency to relapse after surgery and show certain remarkable associations: one of them is "Carney complex" characterized by the presence of cardiac, mammal and cutaneous myxomas associated to pigmentation, hormonal hyperactivity, adrenal and testicular abnormalities, etc. Patients with this dominant hereditary autosomal disorder frequently present cardiac myxomas identical in histological characteristics than isolated tumors (27-29).
four-chamber apical window echocardiography evidences two myxomas, one in the left atrium and other in the right atrium.
b) Fibromas. Consist of congenital intramyocardial tumors, usually unique and circumscribed, that may project into cardiac chambers and some times obliterate them. Typically affect children, specially under 1 year of age, although adolescents and adults may be affected (15% of the cases). Fibromas occurring in infants are cellular and fibroblast-rich, whereas in adults they are predominantly composed of collagen with frequent calcification foci. Some patients may be asymptomatic or present an unexplained murmur. Common manifestations are heart failure or arrhythmia by compression of the conductive system. Some patients may experience sudden death which can be attributed to this last mechanism (30-34).
InMR images of a one-year-old child demonstrates a tumoral mass corresponding to a fibroma located in the apex of left ventricle and the lower part of the interventricular septum.
c) Rhabdomyomas. Represent the most common benign cardiac tumors in children and most of them are discovered in patients under 1 year of age. Children affected by this tumor frequently present tuberous sclerosis (mental deficiency, hamartoma, epilepsy and familiar sebaceous adenoma) and sometimes they present congenital cardiopaties.
Rhabdomyomas consist of yellowish firm circumscribed nodular masses that measure 1mm to 3 or 4 centimeters in diameter. They are most frequently found in the left or right myocardial ventricle, although they can also occur in the atrial muscle. Sometimes tumors appear as miliary multiple disseminated nodules (rhabdomyomatosis).
In most cases patients are asymptomatic. Tumors are frequently discovered at prenatal echography and show a marked tendency to spontaneous regression. When clinical manifestations appear they consist mainly of murmurs, tachyarrithmia or heart failure (35-38).
In, a four-cavity apical window echocardiography, corresponding to a rhabdomyoma, shows a multiple echogenic mass closely associated to the right ventricle.
In, four-cavity apical window echocardiographic view shows multiple echogenic masses of rhabdomyomas in a neonate in the left ventricular myocardium that projects into the ventricular cavity.
d) Lipomas. They are rare benign tumors typically found in adults. Lipomas are circumscribed, spherical or elliptical masses of adipose tissue. Cases of multiple cardiac lipomas have also been reported. They are frequently found in the epicardial region of the atrial or ventricular myocardium, sometimes attached via a pedicle, although they can also be intramural or subendocardial. Myocardial lipomas, truly benign tumors of the heart, must be differentiated from nonencapsulated masses of mature and fetal adipose tissue that may develop in the interatrial septum ("lipomatous hypertrophy of the interatrial septum"). Frequently, lipomas do not cause symptoms and when they appear they are associated to cardiac flux obstruction or conduction system's compromise. Rarely, an enormous tumor may cause severe cardiopulmonary compression resulting in death (39,40).
e) Hemangiomas. Consist of vascular formations that affect all age groups and account for 5% to 10% of all benign tumors. They may occur in any cardiac chamber and consist of a mesh of dilated, cavernous, capillary or arteriovenous vessels. Intramural hemangiomas are spongy and poorly circumscribed. Endocardial-based hemangiomas constitute well circumscribed thin masses. Hemangiomas are frequently incidental findings in asymptomatic patients. When symptoms are present the may be very varied, including dyspnea on exertion, chest pain, arrhythmias, right-sided heart failure or pericardial effusion which may be hemorrhagic. Cases of syncope and sudden death have been reported (41,42).
f) Papillary fibroelastomas. Although rare, they constitute the second cause of benign cardiac tumors. They are typically found in adults, predominantly affect cardiac valves and account for most cases of valvular tumor. They are incidental findings of echocardiography, necropsy, cardiac catheterization or surgery. Like in many other tumors, patients are asymptomatic or present nonspecific cardiac symptoms. However, embolic phenomenon in different regions may occur, including coronary and cerebral beds, caused by fragments that arise from the tumoral tissue itself or by thrombotic material that may form on its surface. Treatment consist of surgical excision of the tumor with possible valve repair if necessary (43-45).
g) Paraglangliomas. They are rare tumors of the crhomaffin intrinsic cardiac system which typically affect the atria. In the few reported cases they were poorly circumscribed, and they were predominantly subepicardial, in the roof of the left atrium. Other less frequent cardiac locations have been described as well as extracardiac concomitant locations of the tumor. They are composed of histologic elements similar to extracardiac paragangliomas and secrete catecholamines, causing symptoms typical of pheochromocytoma. Elevated levels of plasma norepinephrine, vanillylmandelic acid and metanephrine have been found. 111-indio octreotide scintigraphy and MR have been used to locate the tumor when 131-Iodo-metaidobenzylguadina and ACT failed to reveal it (46-50).
h) Teratomas. They constitute rare primary pericardial tumors which typically affect infants and children (exceptionally, intramyocardial teratomas have been described). They are specially found in the right side of the heart, generally connected to one of the great vessels via a pedicle. Habitually, tumor size is associated to patient age at the time of diagnosis, though unusually large intrapericardial teratomas have been reported. They are composed of the same elements that form extracardiac teratomas, including multiple cysts of different sizes and ectodermic, mesodermic and ectodermic tissue elements (skeletal muscle, cartilage, liver, intestine, neuroglia, glands, etc.). These tumor can be diagnosed by prenatal fetal echography which shows a multilocular cystic mass, pericardial effusion and sometimes pleural effusion, subcutaneous edema and polyhydramnios. They may cause respiratory distress, pericardial effusion, sometimes tamponade, and also signs of cardiac compression or pleural effusion after birth. Usually treatment of these tumors consist of surgical excision during the neonatal period, which may be life saving (51-60).
II- Malignant tumors
a) Sarcomas. Constitute the majority of primary malignant tumors that affect the heart and after myxomas, they are the second most common cardiac tumor. They typically affect adults and very rarely children (61).
Surgical statistics show that the most frequent tumors are angiosarcomas (35%-40%), most of which (80%) are located in the right atrium. They predominantly affect men around 50 years of age and are very rare in children although they have been found in people of several age groups. Angiosarcomas consist of large proliferations of endothelial cells delimiting irregular anastomosing vascular spaces. They are invasive tumoral masses of necrotic and hemorrhagic aspect that affect the myocardium, protrude into the atrial cavity (most frequently the right atrium) and compromise the adjacent pericardium. In other cases, the malignant tissue spreads, infiltrating the pericardium, sometimes obliterating its cavity. Angiosarcomas are typically located in the right atrium and that explains why they mostly affect right cardiac chambers influx and cause clinical manifestations of right heart failure. They also produce pericardial effusion, generally hemorrhagic, and tamponade. Patients may also present some general symptoms such as fever and weight lose.
shows a four chamber view transesophageal echocardiogram corresponding to an angiosarcoma. A large mass affecting most of the right atrial cavity and pericardial effusion is seen.
shows another angiosarcoma. MR shows a large mass that affects most of right atrial cavity, with a nodule that corrodes the anterior wall of the thorax. High intensity signals indicate hemorrhagic tumor.
Other primary cardiac tumors more typically affect the left atrium and cause symptoms associated to mitral flux obstruction and the consequent pulmonary congestion. Undifferentiated sarcomas, leiomyosarcomas, fibrosarcomas, and osteosarcomas can be found within this last category. They are predominantly located in the left atrium and sometimes they may be confused, at least at the beginning, with myxomas (69-72).
Leiomyosarcomas usually present earlier than the rest of sarcomas (40 years). They arise from soft muscular tissue surrounding the subendocardium; however, they can also originate in soft muscle of adjacent pulmonary vessels. They are usually sessile, and generally irregular and lobulated. Unlike myxomas, they are usually associated to the posterior part of the left atrium, with a tendency to invade the mitral valve and pulmonary veins rather than being associated to the fossa ovalis.
Fibrosarcomas arise from cells with morphologic characteristics of fibroblasts, while osteosarcomas contain cellular bone generating elements with osteoblastic, chondroblastic or fibroblastic differentiation and frequent calcification (73-76).
Rhabdomyosarcomas originate in the striated muscle and in spite of its very low incidence (4%-7% of all sarcomas) they constitute the most frequent malignant tumor in the pediatric age. They develop in myocardial cavity in any location, without preference for any specific chamber. They are usually multiple. Nodular pericardial invasion and valvular compromise may occur (77-79).
At the time of diagnosis tumors are generally large and the degree of invasiveness high and pulmonary, osseous, ganglionic, hepatic, cerebral, renal, metastasis may already exist. In general, the hystopathological characterization of these tumors is performed through material obtained from autopsy specimens or from a metastatic nodule.
Sarcomas have an ominous prognosis with one year survival after diagnosis. Surgical excision, even partial, is indicated to relieve obstructive symptoms. When large excisions are successfully performed reoccurrence is still frequent. In some cases of unresected sarcomas, good results have been obtained with cardiac transplant (80,81).
b) Lymphomas. Although disseminated lymphomas frequently compromise cardiac structures, there are rare cases of B cells non-Hodgkin lymphomas that compromise the heart. These lymphomas typically affect the right atrium followed by the left ventricle, left atrium, atrial and ventricular septum. Concomitant compromise of several cardiac chambers is also frequent. The contiguous invasion of the pericardium is habitual. Although primary lymphomas can affect all adults, they may be considerably associated to states of immunodeficiency, specially in patients suffering from AIDS. This circumstance makes it mandatory to suspect them clinically when such patients present symptoms of cardiac compromise without apparent reason. Patients usually present severe and progressive heart failure, chest pain, arrhythmias, tamponade and superior vena cava syndrome. The prognosis of affected patients is very poor, in general with survivals of no longer than a year after diagnosis, although in some cases of early diagnosis and treatment with chemotherapy survivals may be longer (82,83).
MR image shows a tumoral mass of a primitive cardiac lymphoma that affects the anterior right atrial and ventricular wall and spreads to the pericardium at the heart's apex.
c) Mesotheliomas. Although pleural mesotheliomas usually affect the pericardium, there are primitive pericardial tumors that arise from their own mesothelial cells and constitute the most frequent malignant pericardial tumors. These tumors predominantly affect men and may be detected at any age. They consist of coalescent irregular lobular masses that obliterate the pericardial space and tend to constrict the heart. Although a mild infiltration into the subepicardial muscle may occur, the underlying myocardium is frequently not affected. Patients present symptoms such as dyspnea, palpitations, chest pain and signs of pericarditis including effusion and tamponade. The analysis of the pericardial fluid may show atypical cells, but cellularity frequently presents unspecific characteristics. In occasions, clinical symptoms may be similar to constrictive idiopathic pericarditis and diagnosis in living patients is generally made at the time of surgery. Mesotheliomas are high malignancy tumors, with possible mediastinal and pulmonary dissemination. Generally, complete surgical resection is impossible, with a survivals no longer than 1 year after the time of diagnosis (84-88).
TREATMENT OF PRIMITIVE CARDIAC TUMORS
Benign primitive cardiac tumors are treated with surgery which, in many cases, may determine the complete cure of the process. It is important to distinguish the "benign" character of tumors, expressed on anatomopathological terms, from its potential malignant effects, viewed from a different angle. In spite of their histological benign character, many of these tumors may cause deleterious consequences because of their hemodynamic (obstruction of the valvular or intracavitary flux, pericardial effusion and tamponade), electric (arrhythmias and conduction disorders) or mechanical (distant embolism) effects. For that reason, in all cases surgical excision should be tried.
Because of their frequency, atrial myxomas usually represent the most important case reports of surgical teams and their resection determine in most cases the complete cure of patients. Myxomas may still relapse after surgery in 1%-5% of the cases (89,90), either because of incomplete resection, due to a new growth from a second tumoral focus or because the individual presents particular biologic features, sometimes multiple myxomatous foci, and are more prompt to these situations as was described before. Apart from myxomas, other histologically benign cardiac tumors may be successfully excised if most of the functional surrounding myocardium and other fundamental anatomic structures are respected.
In general, malignant tumors have a poor prognosis when diagnosis is confirmed. This is because of its invasive and infiltrating behavior that affects myocardial mass and to the possibility of mediastinal tumoral compromise or distant metastasis. Surgery usually plays a role in palliation and tends to relieve some kind of symptoms. In some cases, surgery can be associated to chemotherapy and radiotherapy for this purpose. In other cases, heart transplantation of non surgical malignant tumors has been performed with variable results.
Special thanks to Dr. Gladys Martellotto, Dr. Daniel Boccardo and Dr. Carlos Quiroga Mayor for the material that they so kindly submitted to me to complete the captions of the images.
1. Abraham JM: Neoplasms metastatic to the heart: Review of 3314 consecutive autopsies. Am J Cardiovasc Pathol 1990; 3:195
2. Lam KY, Dickens P, Chan ACL: Tumors of the heart: A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993: 117: 1027
3. Salcedo EE, Cohen GI, White RD, Davison MB: Cardiac tumors: Diagnosis and treatment. Curr Probl Cardiol 1992: 17:73
4. Tazelaar HD, Locke TJ, McGregir GGA: Pathology of surgicallly excised primary cardiac tumors. Mayo Clin Proc 1992; 67:957
5. Reynen K: Frequency of primary tumors of the heart. Am J Cardiol 1996;77:107
6. Perchinsnky MJ, Lichtenstein SV, Tyers GF: Primary cardiac tumors: forty years' experience with 71 patients. Cancer 1997;79:1809-1815
7. Roberts WC: Primary and secondary neoplasms of the heart. Am J Cardiol 1997;80: 671-682
8. Abrams HL, Adams DF, Grant HA: The radiology of tumors of the heart. Radiol Clin North Am 1971;9:299-326
9. Popp R, Harrison D: Ultrasound for the diagnosis of atrial tumor. Ann Intern Med 1969: 71:785
10. Loope D, Bulkley J, Weiss J: Two dimensional echocardiographic diagnosis of left atrial myxoma. Chest 1978:78:55
11. DePace NL, Soulen RL, Kotler MN, Minz GS: Two dimensional echocardiographic detection of intraatrial masses. Am J Cardiol 1981;48:954-960
12. Alam M, Rosman HS, Grullon C: Transesophageal echocardiography in evaluation of atrial masses. Angiology 1995;46:123-128
13. Dawson WB, Mayo JR, Müller NL: Computed tomography of cardiac and pericardial tumors. J Can Assoc Radiol 1990;41:270-275
14. Winkler M, Higgins CB: Suspected intracardiac masses: evaluation with MR imaging. Radiology 1987;165:117-122
15. Lund JT, Ehman RL, Julsrud PR, Sinak, LJ, Tajik AJ: Cardiac masses: assessment by MR imaging. Am J Roentgenol 1989;152:469-473
16. Araoz PA, Eklund HE, Welch TJ, Breen JF: CT and MR imaging of primary cardiac malignacies. Radiographics 1999;19:1421-1434
17. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR: Primary cardiac and pericardial neoplasms: Radiologic-pathologic correlation. Radiographics 2000;20:1073-1103
18. Greenwood WF: Profile of atrial myxoma. Am J Cardiol 1968;21:367
19. Bulkley B, Hutchim G: Atrial myxomas: a fifty year review. Am Heart J 1979:97:639
20. St John Sutton MG, Mercier L, Guliani ER, Lie JT: Atrial myxomas: A review of clinical experience in 40 patients. Mayo Clin Proc 1980:55:371
21. Premaratne S, Hasaniya NW, Arakai HY, Mugiishi MM, Mamiya RT, Mc Namara JJ: Atrial myxomas: experiences with 35 patients in Hawaii. Am J Surg 1995;169:600:603
22. Bjessmo S, Ivert T: Cardiac myxoma: 40 years' experience in 63 patients. Ann Thorac Surg 1997;63:697-700
23. Mashushi, Takao E, Shi-Ichi K, Hirozaku H: Right atrial myxoma with pulmonary embolism. Cardiology 1992;81:178
24. Di Carli S, Sechi L, Ciami R et al: Right atrial myxoma with pulmonary embolism. Cardiology 1994;84:368
25. Segin JR, Beigbeder JY, Hvass U et al: Interleukin-6 production by cardiac myxomas may explain constitutional symptoms. J Thorac Cardiovasc Surg 1992;103:599
26. Seino Y, Ikeda U, Shimada K: Increased expression of interleukin 6-mRNA in cardiac myxomas. Br Heart J 1993;69:565
27. Carney JA, Gordon J, Carpenter PC et al: The complex of myxomas, spotty pigmentation and endocrine overactivity. Medicine 1985;64:270
28. Carney JA, Hruska LS, Beauchamp GD, Gordon H: Dominant inheritance of the complex of myxomas, spotty pigmentation and endocrine overactivity. Mayo Clin Proc 1986:61:165
29. Carney JA: The Carney complex (myxomas, spotty pigmentation, and schwannomas). Dermatol Clin 1995:13:19-26
30. Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM: Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J 1997;134:1107-1114
31. Parmley LF, Salley RK, Williams JP, Head GB: The clinical spectrum of cardiac fibroma with diagnostic an surgical considerations: noninvasive imaging enhances management. Ann Thorac Surg 1988;45:455-465
32. Yomaguchi M, Hosokawa Y, Ohashi H, Imai M, Oshima Y, Minamiji K: Cardiac fibroma: long-term fate after excision. J Cardiovasc Surg 1992;103:140-145
33. Burke AP, Rosado de Christenson M, Templeton PA, Virmani R: Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994;108-862-870
34. Beghetti M, Haney I, Williams WG, Mawson J, Freedom RM, Gow RM: Massive right ventricular fibroma treated with partial resection and a cavopulmonary shunt. Ann Thorac Surg 1996:62:882-884
35. Smythe JF, Dyck JD, Smallhorn JF, Freedom RM: Natural history of cardiac rhabdomyoma in infancy and childhood. Am J Cardiol 1990:66:1247-1249
36. Berkenblit R, Spindola-Franco H, Frater RW, Fish BB, Glickstein JS: MRI in the evaluation and management of a newborn infant with cardiac rhabdomyoma. Ann Thorac Surg 1997;63:1475-1477
37. Black MD, Kadletz M, Smallhorn JF, Freedom RM: Cardiac rhabdomyomas and obstructive left heart disease: histologically but not funcionally benign. Ann Thorac Surg 1998;65:1388-1390
38. Nir A, Tajik J, Freeman WK, et al: Tuberous sclerosis and cardiac rhabdomyoma. Am J Cardiol 1995; 76:419-421
39. Val-Bernal JF, Villoria F, Fernández FA: Polypoid (pedunculated) subepicardial lipoma: a cardiac lesion resembling the epiploic appendage. Cardiovasc Pathol 2000;9:55-57
40. Ashar K, van Hoeven KH: Fatal lipoma of the heart. Am J Cardiovasc Pathol 1992;4:85-90
41. Burke A, Johns JP, Virmani R: Hemangiomas of the heart: a clinicopathologic study of ten cases. Am J Cardiovasc Pathol 1990;3:283-289
42. Brizard C, Latremouille C, Jebara VA, et al: Cardiac hemangiomas. Ann Thorac Surg 1993:56:390-394
43. Klarich KW, Enriquez-Serrano M, Gura GM, Edwards WD, Tajik AJ, Seward JB: Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol 1997;30:784-790
44. Minatoya k, Okabayashi h, Yokota T, Hoover EL: Cardiac papillary fibroelastomas: rationale for excison. Ann Thorac Surg 1996;62:1519-1521
45. Grinda JM, Couetil JP, Chavaud S, et al: Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;117:106-110
46. Orringer MB, Sisson JC, Glazer G, et al: Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc Surg 1985;89:753-757
47. Conti VR, Saydjari R, Amparo EG: Paraganglioma of the heart. Chest 1986;90:604-606
48. Aravot DJ, Banner NR, Cantor AM, Theodoropoulos S, Yacoub MH: Location, localization, and surgical treatment of cardiac pheochromocytoma. Am J Cardiol 1992;69:283-285
49. Cane ME, Berrizbeitia LD, Yang SS, Mahapatro D, McGrath LB: Paraganglioma of the interatrial septum. Ann Thorac Surg 1996;61:1845-1847
50. Lin JC, Palafox BA, Jackson HA, Cohen AJ, Gazzaniga AB: Cardiac pheochromocytoma: resection after diagnosis by 111-indium octeocride scan. Ann Thorac Surg 1999;67:555-558
51. Seguin JR, Coulon P, Huret C, Grolleau-Roux R, Chaptal PA: Intrapericardial teratoma in infancy: a rare disease. J Cardiovasc Surg 1986; 27:509-511
52. Aldousani AW, Joyner JC, Price RA, Boulden T, Watson D, DiSessa TG: Diagnosis and treatment of intrapericardial teratoma. Pediatr Cardiol 1987;8:51-53
53. Cyr DR, Guntheroth WG, Nyberg DA, Smith JR, Nudelman SR, Ek M: Prenatal diagnosis of an intrpericardial teratoma: a cause of nonimmune hydrops. J Ultrasound Med 1988; 7:87-90
54. Rehuban KS, McDaniel NL, Feldman PS, Mayes DC, Rodgers BM: Intrapericardial teratoma causing nonimmune hydrops fetalis and pericardial tamponade: a case report. Pediatr Cardiol 1991;12:54-56
55. Dewan RK, Gupta K, Meena BK, Aggarwal M: Intrapericardial benign teratoma with unusual presentation. Indian J Chest Dis Allied Sci 1998;40:287-290
56. Beghetti M, Prieditis M, Rabeyka IM, Mawson J: Intrapericardial teratoma. Circulation 1998;97:1523-1524
57. Tollens T, Casselman F, Devlieger H, et al: Fetal cardiac tamponade due to an intrapericardial teratoma. Ann Thorac Surg 1998;66:559-560
58. Valioulis I, Aubert D, Lassauge F, Slimane MA: Intrapericardial teratoma diagnosed prenatally in a twin fetus. Pediatr Surg Int 1999;15:284-286.
59. Marianaschi SM, Seddio F, Abella RF, Colagrande L, Iorio FS, Marcelletti CF: Intrapericardial teratoma in a newborn: a case report. J Card Surg 1999;14:169-171
60. Pratt JW, Cohen DM, Mutabagani KH, Davis JT, Wheller JJ: Neonatal intrapericardial teratomas: clinical and surgical considerations. Cardiol Young 2000;10:27-31
61. Putnam JB, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA: Primary cardiac sarcomas. Ann Thorac Surg 1991; 51:906-910
62. Jannigan DT, Husain A, Robinson NA: Cardiac angiosarcomas: a review and a case report. Cancer 1986:57:852-859
63. Dichek DA, Holmvang G, Fallon JT: Angiosarcoma of the heart: three years survival and follow up by nuclear magnetic resonance imaging. Am Heart J 1988;115:1323-1324
64. Rettmark K, Stierle U, Sheikhzadeh A, Diederich KW: Primary angiosarcoma of the heart: report of a case and review of the literature. Jpn Heart J 1993;34:667-683
65. Klima U, Wimmer-Greinecker G, Harringer W, Mair R, Gross C, Brucke P: Cardiac angiosarcoma: a diagnostic dilemma. Cardiovasc Surg 1993:1:674-676
66. Rosenkranz ER, Murphy DJ: Diagnosis and neonatal resection of right atrial angiosarcoma. Ann Thorac Surg 1994:57:1014-1015
67. Kakizaky S, Takagi H, Hosaka Y: Cardiac angiosarcoma responding to multidisciplinary treatment. Int J Cardiol 1997; 62:273-275
68. Reising C, Thieman K, Nuss F, Latcham AP: Primary cardiac angiosarcoma with right coronary artery-to-pericardial fistula: a case report. Angiology 1999:777-780
69. Ludomirsky A, Vargo TA, Murphy DJ, Gresik MV, Ott DA, mullins CE: Intracardiac undifferentiated sarcoma in infancy. J Am Coll Cardiol 1985;6:1362-1364
70. Segesser LV, Cox J, Gross J, et al: Surgery in primary leiomyosarcoma of the heart. Thorac Cardiovasc Surg 1986:34:391-394
71. Lo FL, Chou YH, Tiu CM, et al: Primary cardiac leiomyosarcoma imaging with 2-D echocardiography, electron beam CT and 1.5 T MR. Eur J Radiol 1998;27:72-76
72. Durand E, Vanel D, Mousseaux E, Menigan P, Fornes P, Brittoun J: A recurrent left atrium leiomyosarcoma. Eur J Radiol 1998;8:97-99
73. Itoh A, Okubo S, Nakanishi N, et al: Recurrent epicardial fibrosarcoma which arose 12 years after the first resection. Eur Heart J 1991;12:270-272
74. Knobel B, Rosman P, Kishon Y, Husar M: Intracardiac primary fibrosarcoma: case report and literature review. Thorac Cardiovasc Surg 1992;40:227-230
75. Yashar J, Witoszka M, Savage DD, et al: Primary osteosarcoma of the heart. Ann Thorac Surg 1979:28:594-600
76. Reynard JS Jr, Gregoratos G, Gordon MJ, Bloor CM: Primary osteosarcoma of the heart. Am Heart J 1985;109:598-600
77. Bemis EL, Pemberton AH, Laurie A: Rhabdomyosarcoma of the heart. Cancer 1972;29:924-929
78. Satoh M, Horimoto M, Sakurai K, Funayama N, Igarashi K, Yamashiro K: Primary cardiac rhabdomyosarcoma xhibiting transient an pronounced regression with chemotherapy. Am Heart J 1990;120:1458-1460
79. Szucs RA, Reher RB, YanovichS, Tatum JL: Magnetic resonance imaging of cardiac rhabdomyosarcoma: quantifying the response to teraphy. Cancer 1991;67:2066-2070
80. Michler RE, Goldstein DJ: Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 1997;24:534-539
81. Harlamert HA, Moulton JS, Lewis W: Primary malignant fibrous histiocytoma of the heart treated with orthotopic heart transplantation. Circulation 1998;97:703-704
82. Ceresoli GL, Ferrei AJM, Bucci E, Ripa C, Ponzoni M, Villa E: Primary cardiac lymfoma in immunocompetent patients: diagnostic and therapeutic management. Cancer 1997;80:1497-1506
83. Aboulafia DM, Bush R, Picozzi VJ: Cardiac tamponade due to primary pericardial lymphoma in a patient with AIDS. Chest 1994;106:1295-1299
84. Kaul TK, Fields BL, Kahn DR: Primary malignant pericardial mesothelioma: a case report and review. J Cardiovasc Surg 1994;35:261-267
85. Oreopoulus G, Mickleborough L, Daniel L, De Sa M, Merchant N, Butany J: Primary pericardial mesothelioma presenting as constrictive pericarditis. Can J Cardiol 1999;15:1367-1372
86. Watanabe M, Suzuki H, Fukutome K, Yamada N, Nakano T, Shiraishi T, Yatani R: An autopsy case of a malignant pericardial mesothelioma in a Japanese young man. Pathol Int 1999;49:658-662
87. Quinn DW, Qureschi F, Mitchell IM: Pericardial mesotelioma: the diagnostic dilemma of misleading images. Ann Thorac Surg 2000;69:1926-1927
88. Watanabe A, Sakata J, Kawamura H, Yamada O, Matsuyama T: Primary pericardial mesothelioma presenting as constrictive pericarditis: a case report. Jpn Circ J 2000 64:385-388
89. Mc Carthy PM, Piehler JM, Schaff HV, et al: The significance of multiple, recurrent, and "complex" cardiac myxomas. Thorac Cardiovasc Surg 1986;91:389
90. Waller DA, Ettles DF, Saunders NR, Williams G: Recurrent cardiac myxoma: The surgical implications of two distinct groups of patients. Thorac Cardiovasc Surg 1989;37:226
91. Terry LN, Kilgerman MM: Pericardial and myocardial involvement by lymphomas and leukemias. The role of radiotherapy. Cancer 1970;25:1003
92. Gerfein OB: Lymphosarcoma of the rihgt atrium. Angiographic and hemodynamic documentation of response to chemotheraphy. Arch Intern Med. 1975;135:325
93. Aufiero TX, Pae WE Jr, Clemson BS, et al: Heart transplantation for tumor. Ann Thorac Surg 1993;56:1174
94. Crespo MG, Pulpon LA, Pradas G, et al: Heart transplantation for cardiac angiosarcoma: Should its indication be questioned? J Heart Lung Transplant 1993;12:527
95. Yuh DD, Kubo SH, Francis GS, et al: Primary cardiac lymphoma treated with orthotopic heart transplantation: A case report. J Heart Lung Transplant. 1994:13:358
96. Baay P, Karwande SV, Kushner JP, et al: Succsessful treatment of a cardiac angiosarcoma with combined modality teraphy. J Heart Lung Transplant 1994;13:923
Your questions, contributions and commentaries will be answered
by the lecturer or experts on the subject in the Clinical Cardiology list.
Please fill in the form (in Spanish, Portuguese or English) and press the "Send" button.
2nd Virtual Congress of Cardiology
Dr. Florencio Garófalo
Dr. Raúl Bretal
Dr. Armando Pacher
Technical Committee - CETIFAC
Copyright© 1999-2001 Argentine Federation of Cardiology
All rights reserved
This company contributed to the Congress: