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Cardiac tumors
J
THoRAc CARDIOVASC SURG
88:439-446, 1984
Cardiac tumors Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients Between 1961 and 1983, 20 patients underwent operation at the Texas Heart Institute for primary tumors other than classical benign myxoma. Total follow-up was 70.7 patient years (mean 3.5 years). There were 10 adults and 10 childrenunder 12 years of age. There weretwo operativedeatm and four late deaths, All of the pediatric patients bad benign Iesiom (five fibromas, five rbabdomyomas), and only one patient in this group died (during the operation), All operativesurvivors are alivebetween 0.9 and 18 years postoperatively, although in three cases excision was incomplete. Of the 10 adult patients, five bad benign Iesi~ all were completely excised. In the other five adult patients, unresectable malignant tumors were found, and all four operativesurvivors died of metastatic disease within 8 monem. Benigncardiac tumors in childhood bave an excellent prognosiswhen completely excised and appear to bave a good short-term prognosis even when excision is incomplete. Although prognosis for benign tumors in adults is good, malignant tumors are associated with very poor survival.
Ian J. Reece, M.B., ER.C.S.,· Denton A. Cooley, M.D., O. H. Frazier, M.D., Grady L. Hallman, M.D., Penny L. Powers, B.S.N., and Carlos G. Montero, M.D.,
Houston, Texas
Cardiac tumors are infrequent and until the early 1950s were not amenable to surgical treatment. I With the introduction of cardiopulmonary bypass, interest in surgical treatment of cardiac tumors increased. Bypass was first utilized for tumor resection in 1954.2 Nadas and Ellison' reported that primary cardiac tumors are seen in 1:10,000 routine autopsies and are found ten to twenty times less frequently than cardiac involvement by secondary growths or direct extension. Griffiths,' in reviewing 500 primary tumors, found 50% to be myxomas and 20% to be rhabdomyomas. Almost all of the malignant lesions were sarcomatous. Between 1961 and 1983, over 52,500 patients underwent cardiac operations at this institution, and during
From the Section of Surgery, Texas Heart Institute of St. Luke's Episcopal and Texas Children's Hospitals, Houston, Texas. Received for publication Oct. 28, 1983. Accepted for publication Dec. 5, 1983. Address for reprints: Denton A. Cooley, M.D., Texas Heart Institute, P.O. Box 20345, Houston, Texas 77225. *Senior Registrar in Cardiothoracic Surgery, Glasgow Royal Infirmary, Glasgow, Scotland. Presently Cardiovascular Research Fellow, Cardiovascular Surgical Research Laboratories, Texas Heart Institute, Houston, Texas.
that period only 20 primary cardiac tumors were found. During the same period, 51 benign myxomas were excised. The incidence of primary tumor (other than benign myxoma) was less than one tumor in every 2,500 cases. This report details the clinicopathological spectrum of patients with cardiac tumors other than classical benign myxomas at this institution and includes long-term follow-up of children with completely and incompletely excised growths.
Patients and methods The hospital records and diagnostic data of 20 patients were reviewed. Survivors were contacted by telephone (l1/14 patients). One patient was lost to follow-up, and data for two patients were obtained from their last hospital visits 2 and 2.5 years postoperatively. Total follow-up for this series is 70.5 patient-years (mean 3.5 patient-years) and is 95% complete.
Results Between 1961 and 1983, 20 patients underwent operations for primary cardiac tumors other than classical benign myxoma. There were 12 females and eight males ranging in age from I month to 59 years (mean 439
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The Journal of Thoracic and Cardiovascular Surgery
Reece et al.
Table I. Cardiac tumors in children, Texas Heart Institute experience Case No.
Age, sex
Lesion
Procedure
Site
12, F
Fibroma
RV mass extending into AV groove
2
JIll, F
Rhabdomyoma
3
YIl, M
Rhabdomyoma
4
til, F
Rhabdomyoma
Posterior leaflet of MV and adjacent anulus obstructing MV Anterior wall of RV obstructing RV outflow Posterolateral LV wall
5
\012, F
Rhabdomyoma
6
1-\', M
Fibroma
7
2M
Fibroma
8
1'i2, F
Fibroma
9*
6F
Fibroma
IF
Rhabdomyoma
10
Enucleation of tumor after freeing RCA Excision of mass and base; repair of MV Excision of mass via right ventriculotomy Excision of tumor; emergency reattachment of papillary muscle Excised after endocardial mapping
LV free wall causing LVOT obstruction RV free wall adjacent to LAD Excised Ventricular septum and diaphragmatic Debulking of tumor, 90% excised wall of RV Ventricular septum causing LVOT Enucleated and excised obstruction LV free wall, septum displacing LA Biopsy only and MV RA mass extending into IVC and Excision of mass obstructing TV in diastole Ventricular septum adjacent to apex Excision
Resection
Complete Complete Complete Incomplete'
Complete
Incomplete Complete Incomplete Incomplete Complete
Legend: AV,Atrioventricular. RCA,Right coronary artery. RV, Right ventricle. LV, Leftventricle. RA,Rightatrium. LA,Left atrium. MV, Mitral valve. LVOT, Left ventricular outflow tract. LAD, Left anterior descending coronary artery. PVC, Premature ventricular contraction. IVe, Inferior vena cava. TV,Tricuspid valve. 'Wilms' tumor was diagnosed in infancy, and the patient underwent right nephrectomy, radiotherapy, and chemotherapy.
20.7 years). There were 10 children younger than 12 years of age, including seven children under 1 year. The mean age of those with fibromas (4.3 years) was significantly greater than the mean age of those with rhabdomyomas (5 months, p = 0.001). Fifteen of the tumors were benign (75%), and five were malignant (25%). Origin of the tumors included seven from atrial structures (three malignant), 10 from ventricular structures (one malignant), and one from the epicardial fat between the great vessels; two tumors appeared to originate at the atrioventricular junction (one malignant). Clinical presentation (Tables I and II). Of the 10 children, one had symptoms of dyspnea, and one had congestivecardiac failure. Three children were found to have murmurs on routine examination, and one was undergoing evaluation of seizures. Three children had arrhythmias: Two of these had recurrent ventricular tachycardia, and the other had had a cardiac arrest with subsequent congestive heart failure. Patient 5 was known to have tuberous sclerosis, and Patient 2 had evidence of the disease 1.5 years after removal of a cardiac tumor. Both of these children had rhabdomyomas that were completely excised. One child is asymptomatic 2 years postoperatively; the other is incapacitated from tuberous sclerosis 2.5 years postoperatively.
The incidence of tuberous sclerosis in patients with rhabdomyoma, therefore, was 40% (2/5). In two other children, tumors were suspected after chest roentgenogram for evaluation of noncardiac symptoms. Cardiomegaly was present clinically and was confirmed by chest roentgenogram in five children (50%). Calcification in the region of the right atrium was present in one child (Patient 9). Heart size appeared normal in four patients. Echocardiography was used preoperatively in four children. In each case, it revealed a filling defect or definite abnormality. Cardiac catheterization was performed in' seven cases and revealed or confirmed the presence of cardiac tumors in all instances. In two children, endomyocardial mapping was also done, and ectopic foci within the left ventricle associated with a tumor were found in both cases. Both tumors were rhabdomyomas. One was incompletely excised, and the other was completely excised. In one child (Patient 9), the position and extent of a right atrial mass was evaluated by inferior vena cavogram. In the 10 adult patients, a tumor was found during an operation for mitral regurgitation in one case (Patient 15) and for coronary artery disease in another (Patient 19). In the remaining eight adults, dyspnea was present in six (75%), chest pain in two (25%), and hemoptysis,
Volume 88 Number 3 September. 1984
Outcome
Cardiac tumors
Follow-up
Status
Survived
2 yr
Asymptomatic
Survived
2.5 yr
Survived
1 yr
Tuberous sclerosis, cataracts, and cardiomyopathy Asymptomatic
Died
Survived
2 yr
Asymptomatic
Survived
10 yr
Asymptomatic
Survived
18 yr
Occasional Pv'Cs only
Survived
2.5 yr
Asymptomatic
Survived
IO yr
Asymptomatic
Survived
2 yr
No cardiac symptoms
heart failure, cardiac tamponade, and paroxysmal supraventricular tachycardia on one occasion each. One patient was found to have cardiomegaly on routine chest roentgenogram. The important clinical features and histologic diagnoses are summarized in Tables I and
II. Operation and survival. The operation was performed with cardiopulmonary bypass in 19 patients and via median sternotomy in 18. Excision was considered by the surgeon to be macroscopically complete in seven of the 10 children (70%), in all of the adults with benign lesions, but in none of the patients with malignant tumors. There was one operative death in the pediatric group. This child (Patient 4, Table I) died after detachment of the posterior papillary muscle following resection of a left ventricular rhabdomyoma, despite emergency reoperation and reimplantation of the muscle. Therefore, the operative survival rate was 90%, and there have been no late deaths during follow-up of 1 to 18 years (mean 4.1 years). The three children in whom excision was incomplete were all free of cardiac symptoms or signs attributable to residual tumor at a mean follow-up of 4.5 years (range 1 to 10 years). One child who had biopsy only is alive and well 2.5 years after operation. In the adult group, there was one operative death (10%) in a patient (Patient 11) undergoing her third operation for recurrent myxosarcoma of the left atrium,
441
which proved to be unresectable. All patients with malignant tumors who survived the operation died within 8 months of metastatic disease. One adult with a benign lesion (Patient 15) was lost to follow-up; the four other patients are all asymptomatic 31 months to 22 years after operation (mean follow-up 11.4 years). Discussion Benign lesions: Pediatric patients. Benign rhabdomyoma. Benign rhabdomyoma is the most common cardiac tumor in both. infants and children,' though the neoplastic nature of cardiac rhabdomyoma is controversial." Heath" considers this malformation to be a hamartoma, and the strong association between cardiac rhabdomyoma and tuberous sclerosis?" is consistent with this concept. Since cardiac rhabdomyoma was first described in 1862 by von Recklinghausen," more than 110 cases have been reported in the literature; tuberous sclerosis was present in about 50% of these cases. All of our patients were under 1 year of age at the time of operation, and it is probable that the ease with which these lesions were excised is a consequence of operating before there was significant displacement of surrounding structures or major secondary changes in the cardiac muscle or lungs by obstructive lesions. The poor prognosis in untreated lesions reported by Kidder,'? with a 60% mortality by 1 year of age, was undoubtedly reversed by early operation in our patients. The mean follow-up in the operative survivors is now 1.9 years with no late deaths. Subaortic stenosis caused by cardiac rhabdomyoma has been described 1I. 12 and was present in one of our patients. The lesion was successfully excised through the aortic valve. Three patients had arrhythmia: Two had ventricular tachycardia, and one patient had a "cardiac arrest" followed by congestive heart failure. In the two patients with recurrent ventricular tachycardia, intraoperative endocardial mapping was performed, and in one patient cryoablation was used to remove an ectopic focus adjacent to an incompletely excised tumor. Although the differentiation of ventricular from supraventricular tachycardia may be difficult in infants," the correct diagnosis is important. In our two patients, tumors were found in the left ventricular wall and apical septal region and were undoubtedly the site of ectopic ventricular activity. Engle, Ebert, and Redo" reported two similar cases in which the tumors were successfully removed and the ventricular tachycardia cured; they stressed the importance of recognizing the association of tachycardia with left ventricular tumors in infants. Nodal extrasystole" and heart block with atrial flutter" have also been
The Journal of Thoracic and Cardiovascular
44 2 Reece et al.
Surgery
Table II. Cardiac tumors in adults, Texas Heart Institute experience Case No.
Lesion
II
Recurrent myxosarcoma
12
43, F
13
58, F
14
21, F
15
58, M
16
32,t F
17
33, M
18
20, M
19
59, M
20
51,M
Site
Procedure
Posterior wall of LA extending Left thoracotomy; exploration of heart/Lx into superior PVsand obstructing MV Fibromyxosarcoma Posterior leaflet of MV extending MVR, atrial thrombectomy; into anulus and into LV; repair of anulus; biopsy of deposits on RV, LV, PA tumor Angiosarcoma Between cavae involving RCA and Debulking of tumor AV groove Epicardial fat of RV extending Excision Lipoma onto RA, involving RCA Anterolateral aspect of RA Excision Hemangioma (patient required mitral repair for ruptured chordae) Angiomatous hamartoma RV extending into AV groove and Excision, 4 ern of RCA excised onto RA, 8 em wide at base AV groove, extending into RA Excision Angiosarcoma and RV Large LA mass involving MV, Leiomyosarcoma Excision LV, and PVs Lipoma RA, attached at IVC/RA Excision ACBX3 junction, 3 by 6 cm mass Lipoma (1.4 kg) Epicardial fat extending between Excision great vessels into transverse sinus
Resection None
Incomplete
Incomplete Complete Complete
Complete Incomplete Incomplete Complete Complete
Legend: LA, Left atrium. PV, Pulmonary vein. MV, Mitral valve. OR, Operating room. VF, Ventricular fibrillation. RV, Right ventricle. LV, Left ventricle. PA, Pulmonary artery. AV, Atrioventricular. RA, Right atrium. RCA, Right coronary artery. IVC, Inferior vena cava. ACB, Aorta-coronary bypass. 'Two previous explorations with partial tumor excision, I and 2 years previously. tOne previous exploration elsewhere.
reported in association with cardiac rhabdomyoma but were not Seenin any of our patients. In the large series of rhabdomyomas reported by Fenoglio, McAllister, and Ferraris," arrhythmia occurred in six of 36 patients and was ventricular in three and atrial in three. However, the site of the ectopy did not always correlate with the site of the lesion. Long-term survival after excision of cardiac rhabdomyomas has been reported, 14, 18 and in those patients without tuberous sclerosis, the prognosis should be excellent. The aggressive surgical approach suggested by Houser, Forbes, and Stewart'? should be encouraged in these infants, since early operation appears to be associated with excellent results, although some survivors will eventually have tuberous sclerosis. Benign fibroma. Cardiac fibromas are almost always solitary and are the second most common cardiac tumor in infants and children.' These tumors are firm and compress the surrounding structures as they grow, sometimes to huge sizes (Fig. 1).6 In common with other large series, complete excision of fibromas was less common than was complete excision of rhabdomyomas and was accomplished in
only two patients.' In our series, debulking of the tumor was performed in two patients; only in patient 8 was it impossible to undertake any procedure other than biopsy. Four of these patients had symptoms of cardiac obstruction, and one had no cardiac symptoms. The majority of fibromas are found in the interventricular septum or ventricular free walls"; they are seen only rarely in the atria." Although calcification has been seen in rhabdomyoma," intracardiac lipoma," and left atrial myxoma," it appears to be more common in fibromas" but was present in only one case in this series. Ventricular and supraventricular tachycardias did not occur in our patients with fibromas, although arrhythmia is said to occur in some 30% of these patients." Sudden death, usually following physical exertion, occurs in up to 25% of patients.P-" Although left ventricular outflow tract obstruction has been reported as a consequence of a pedunculated fibroma," it is usually secondary to septal enlargement 29, 30 and is rare." The ventricular free walls are often involved, the fibroma lying within the myocardium. Patient I had the unusual feature of encapsulation
Volume 88
Cardiac tumors 4 4 3
Number 3 September, 1984
Outcome
Follow-up
Died in OR; VF on induction of anesthesia
Died, multiple metastases
2 mo
Died, multiple metastases
6 mo
Asymptomatic
13 yr
Uneventful hospital recovery Lost to follow-up
10 days
Asymptomatic
22 yr
Died, multiple metastases
6 mo
Died, cerebral metastases
8 mo
Asymptomatic
31 mo
Asymptomatic
8 yr
of the right coronary artery on the epicardial surface of the heart. The fibroma was dissected from the artery, which was left intact. Although first described in 1855 by Luschka," multiple fibromas in the same heart have been reported only twice'l" and were not seen in our series. The prognosis for an incompletely resected tumor must be guarded, but it would seem logical to explore all cases in which the diagnosis is made, since almost all rhabdomyomas and more than 50% of fibromas can be completely excised and many other fibromas substantially debulked. Long-term survival after complete excision of the fibroma was reported by Williams and associates," with survival up to 17 years after operation, and is confirmed in our patients. It has been suggested that, with noninvasive investigations and follow-up, operation may be avoided in some cases." However, our experience with these patients in terms of low operative mortality and excellent clinical outcome should encourage early surgical intervention before the development of secondary myocardial or pulmonary vascular changes or sudden death." The role of echocardiography, especially two-dimensional echo studies, is well-established in the diagnosis and follow-up of infants and children with cardiac tumors." 19,34. 3S Cardiac catheterization, although less
useful for diagnostic purposes, is still valuable" and is necessary to exclude other congenital lesions in this young group of patients, especially when murmurs or heart failure are present. Simultaneous biventricular contrast injection is particularly useful in delineating septal enlargement (Fig. 2). Benign lesions: Adult patients. lipoma. Paget" was the first to describe cardiac lipoma, but his description concerned a sheep! In his review of cardiac tumors, Heath" cited only 30 cases in the literature. Lipomas may arise from epicardial or pericardiaI fat and often contain elements of surrounding connective tissues. As would be expected, the prognosis for this group of patients is excellent; however, in our patient 14, the lesion surrounded the right coronary artery and protruded into the right atrial cavity, so that extensive dissection and repair were necessary. Arrhythmias have been reported in patients with lipomatous atrial septal hypertrophy and may cause significant mortality and morbidity,' but they do not appear to be associated with lipomas on the surface of the heart and did not occur in our patients. Hemangioma. Hemangiomatous malformations of the heart have been reported infrequently,' most being incidental autopsy findings. Patient 16, however, had undergone thoracotomy at another institution for a presumed pericardial cyst associated with premature atrial extrasystoles. An extensive hemangioma which involved the anterior atrioventricular groove and the right coronary artery was found and left in situ. Several weeks later, via a median sternotomy, the 450 gm tumor was excised with a 4 em length of right coronary artery and a portion of the right ventricular outflow tract. The patient remains well 22 years after the operation and has no cardiac symptoms. A similar case was reported by Scully, Mark, and Mclveely," in which excision proved impossible but in which the echocardiogram was useful in delineating the mass and its extent, so that a correct preoperative pathological diagnosis could be made. It would appear, however, that even if a large hemangiomatous tumor is diagnosed preoperatively, exploration should still be undertaken, as there will be patients in whom complete excision can be performed and in whom long-term survival may then be expected. Malignant lesions: Adult patients. Primary malignant tumors of the heart occur approximately 20 times less frequently than does metastatic cardiac involvement." Metastatic deposits are not infrequently found at autopsy in patients dying of carcinomatosis (10%), but generally they do not displace, deform, or obstruct
444 Reece et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 1. Operative photograph from Patient 6, illustrating the relatively large size of this fibroma, which was completely excised.
Fig. 2. Biventricular angiogram in Patient 6 showing the extent of septal thickening resulting from a benign fibroma. cardiac structures. Primary malignant tumors invade the heart and surrounding structures and produce chest pain, pericardial effusion, and cardiac obstruction. Angiosarcoma. Angiosarcoma, first described by Mennig in 1888,40 is the most frequent primary malignant cardiac tumor, accounting for about 15% of the cardiac sarcomas.' Approximately63%of more than 50 cases reported have been found principally in the right atrium," although the diagnosis was made in fewer than 10 patients during life." Our two patients, one of whom has been describedpreviously,"had many of the clinical and pathological features of this tumor. Symptoms of
right-sided heart failure were present in both, and pericardial tamponade occurred in one. Both had chest pain. The fact that these symptomshad been presentfor almost a year suggests that delayed presentation may playa role in the poor prognosis of this disease. Gross cardiomegaly on chest roentgenogram, but no evidence of disseminateddisease, again suggests that the outlook could be improved with earlier diagnosis. Echocardiography was of diagnostic use in one of our patients and revealed extensive intracavitary extension and displacement of the tricuspidvalve. Classically, metastasesoccur in the lungs; pulmonary metastasis occurred early (3 months) in one patient and was present at autopsy in the other. Both patients had hepatic metastases also. Despite extensive surgical resection, tumor excision was macroscopically incomplete in both patients, and both were subsequently treated with a variety of chemotherapeutic agents. Interestingly, no residual disease was found in the hearts at autopsy, although the chemotherapy did not affect the metastases. The combineduse of operationand chemotherapyhas been associatedwith prolongedsurvival in some patients with cardiac hemangiosarcoma.r'" but these treatments did not appear to influencesignifIcantly the courseof the disease in our patients, both of whom died 6 months after operation. Eighty-eight percent of previously reported patients were dead within 9 months," although Hollingsworth and Sturgill? had a patient who underwent incomplete excision followed by cobalt 60 radiotherapy and a prolonged course of systemicchemotherapy who was free of disease 10 months later. The
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Cardiac tumors 44 5
Number 3 September, 1984
occurrence of a bloody pericardial effusion, right-sided heart failure, and pleuritic chest pain should suggest cardiac angiosarcoma. Early aggressive operation followed by radiation and systemic chemotherapy offer, at present, the only hope of prolonged survival. Myxosarcoma. Myxosarcomas arise generally in the left atrium and often involve the mitral valve. Although some are clearly myxomatous, many are predominantly fibrous and have been called malignant fibrous histiocytoma or fibromyxosarcoma.' These tumors are locally invasive and may metastasize or produce systemic
emboli.' In our series of malignant cardiac tumors, 2 patients were found to have this lesion. Both had symptoms of mitral valve disease, one with mitral obstruction and the other with mitral regurgitation from extensive irregularity and derangement of the posterior leaflet of the mitral valve. The second patient (Patient 12) had multiple intrapericardial and epicardial secondary deposits. At autopsy, 2 months after the operation, multiple hepatic deposits were found as well. Mediastinal glands removed at operation were also invaded by the tumor. Patient 11 underwent removal of a left atrial myxoma in 1980. The excised mass was thought to be a low-grade malignancy. One year later another mass, this time sessile and adjacent to the left upper lobe vein, was excised; angiography 6 months later revealed another recurrence. The tumor appeared to involve the posterior wall of the left atrium, and radical excision including the adjacent left lung was planned. However, the patient died during exploration, and resection was, therefore, not possible. The clinical course of this patient is similar to that of a case reported by Gableman and associates." It confirms the palliative value of resection of recurrent left atrial tumors and again emphasizes the need for wide excision of atrial myxomas at the initial operation. Since mitral valve symptoms are the only consistent feature of this disease, echocardiography may be of value in differentiating benign from malignant lesions when the tumor extends into adjacent structures. Early and wide excision seem to offer the best treatment, although our operative survivor died 2 months postoperatively. Leiomyosarcoma. This tumor is rare; only eight cases have been reported through 1979.5 A well-documented case by Hardin and colleagues" exhibited features very similar to those of our Patient 19. Both patients had dyspnea, pulmonary congestion, and hemoptysis. In both cases, the tumor filled the left atrium and appeared to originate from the endocardium between the right pulmonary veins. Death occurred 3 months after the
onset of symptoms. Our patient died of multiple cerebral metastases 8 months postoperatively despite radiotherapy and chemotherapy.
Conclusion The clinical and pathological spectrum of cardiac tumors in both adults and children seen over a 22 year period confirms the excellent short-term and mediumterm prognosis in children with completely excised benign lesions and indicates a good early prognosis in cases in which only partial excision is possible. Benign tumors in adults appear to have an excellent long-term prognosis, but this series confirms the very poor results obtained with primary malignant cardiac tumors and indicates that even if the primary lesion can be eradicated, metastases may well be present in occult form and will usually cause death within a year.
2
3 4 5
6 7 8
9
10 11
12
13
REFERENCES Maurer ER: Successful removal of tumors of the heart. J THORAC SURG 23:479-483, 1952 Crafoord CL: Discussion on mitral stenosis and insufficiency, (Proceedings of the) International Symposium on Cardiovascular Surgery, Henry Ford Hospital, Detroit, CR Lam, ed., Philadelphia, 1955, W. B. Saunders Company, p 202 Nadas HS, Ellison RC: Cardiac tumors in infancy. Am J Cardiol 21:363-366, 1968 Griffiths GC: A review of primary tumors of the heart. Prog Cardiovasc Dis 7:465-470, 1965 McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system, Atlas of Tumor Pathology, Vol 15, Washington D. C., 1978, Armed Forces Institute of Pathology Heath D: Pathology of cardiac tumors. Am J Cardiol 21:315-327, 1968 Critchley M, Earl CJC: Tuberous sclerosis and allied conditions. Brain 55:311-314, 1932 Shaher RM, Mintzer J, Farina M, Alley R, Bishop M: Clinical presentation of rhabdomyoma of the heart in infancy and childhood. Am J Cardiol 30:95-103, 1972 von Recklinghausen F: Verhandlungen der Gesellschaft fur Gerburtshulfe, Herr v. Recklinghausen tegt der Gesellschaft ein Herz von einem Neugeborenen. Monatsear Geburtsk 20:1-2, 1862 Kidder LA: Congenital glycogenic tumors of the heart. Arch Pathol 49:55-59, 1950 Kuehl KS, Perry LW, Chandra R, Scott LP: Left ventricular rhabdomyoma. A rare cause of subaortic stenosis in the newborn. Pediatrics 46:464-468, 1970 Shaher RM, Farina A, Alley R, Mansen P, Bishop M: Congenital subaortic stenosis in infancy caused by rhabdomyoma of the left ventricle. J THORAC CARDIOVASC SURG 63:157-163, 1972 Ehlers KH: Supraventricular and ventricular dysrhythrnias in infants and children, Cardiovascular Clinics.
4 4 6 Reece et al.
Pooiatric Cardiology, Vol 4, MA Engle, ed., Philadelphia, 1972, F. A. Davis Company, pp 59-84 14 Engle MA, Ebert PA, Redo SF: Recurrent ventricular tachycardia due to resectable cardiac tumor. Report of two cases in two-year-olds with heart failure. Circulation 50:1052-1057, 1974 15 Golding R, Reed G: Rhabdomyoma of the heart. Two unusual clinical presentations. N Engl J Moo 276:957959, 1967 16 Engle MA, Ito T, Ehlers KH, Goldberg WP: Rhabdomyomatosis of the heart. Diagnosis during life with clinical and pathological findings (abstr). Circulation 26:713, 1962 17 Fenoglio JJ, McAllister HA, Ferrans VJ: Cardiac rhabdomyoma. A clinicopathological and electron microscopic study. Am J CardioI38:241-251, 1974 18 Harinck E, Moulaert AJMG, Rohmer J, Brom G: Cardiac rhabdomyoma in infancy. Case report. Acta Paediatr Scand 63:283-286, 1974 19 Houser S, Forbes N, Stewart S: Rhabdomyoma of the heart. A diagnostic and therapeutic challenge. Ann Thorac Surg 29:373-377, 1980 20 Culliford AT, Isom OW, Trehan NK, Doyle E, Gorstein F, Spencer FC: Benign tumors of the right atrium necessitating extensive resection and reconstruction. J THORAC CARDIOVASC SURG 76:178-182, 1978 21 Bickford BJ, Egan M, Bryce AG: Surgical treatment of tumors of the heart. A report of two cases. Br J Surg 73:514-519,1956 22 Haviar V, Siska K, Klein F: Uber eine mit Erfolg operierte Herzgeschwulst von interessantem feingeweblichen Aufbau. Cardiologia 29:132-142, 1956 23 Stewart J, Saunders NR: Left atrial myxoma with extensive calcification. Thorax 37:224-225, 1982 24 Waaler PE, Svendsen S, Halvorsen JF: Intramural calcified fibroma of the heart. Acta Paediatr Scand 61:217222, 1972 25 Aryanpur I, Nazarian I, Razmura M, Shiekh MA, Khonsari S: Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision. Am J Dis Child 130:1265-1267, 1976 26 Geha AS, Weidman WH, Soule EH, McGoon DC: Intramural ventricular cardiac fibroma. Circulation 36:427-440, 1967 27 Folger GM, Peters HJ: Nodular fibroelastosis (fibroelastic hamartoma). A tumorous malformation of the heart. Am J Cardiol 21:420-427, 1968 28 Oliva PB, Breckinridge JC, Johnson ML, Brantigan CO, O'Meara OP: Left ventricular outflow obstruction produced by a pedunculated fibroma in a newborn. Clinical, angiographic, echocardiographic and surgical observations. Chest 74:590-593, 1978 29 McCue CM, Henningar GR, Davis E, Ray J: Congenital subaortic stenosis caused by fibroma of left ventricle. Pediatrics 16:372-377, 1955
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30 Luschka H: Fibroma in the heart muscle. Virchows Arch (Pathol Anat) 8:343-347, 1855 31 Zander R: Fibroma of the heart. Vichows Arch (Pathol Anat) 80:507-510, 1880 32 Fernando SSE: Cardiac fibroma (fibrous hamartoma) of infancy. Two case reports. Pathology 11: 111-117, 1979 33 Williams DB, Danielson GK, McGoon DC, Feldt RH, Edwards WD: Cardiac fibroma. Long-term survival after excision. J THORAC CARDIOVASC SURG 84:230-236, 1982 34 Charuzi Y, Mills H, Buchbinder NA, Marshall LA: Primary intramural cardiac tumors. Long-term follow-up. Am Heart J 106:414-419, 1983 35 Bini RM, Westaby S, Bargeron LM, Pacifico AD, Kirklin JW: Investigation and management of primary cardiac tumors in infants and children. J Am Coli Cardiol 2:351-357, 1983 36 Arciniegas E, Hakimi M, Farooki ZQ, Truccone NJ, Green EW: Primary cardiac tumors in children. J THORAC CARDIOVASC SURG 79:582-591, 1980 37 Paget SJ: Lectures on Surgical Pathology, 00 3, W Turner, ed., London, 1870, Songman, p 447 38 Scully RE, Mark EJ, McNeely BU: Case records of the Massachusetts General Hospital. Case 4-1983. N Engl J Moo 308:206-214, 1983 39 Fallon JT, Erdmann JA: Cardiac neoplasms, The Practice of Cardiology, RA Johnson, E Haber, WG Austen, OOs., Boston, 1980, Little Brown & Company, pp 695-705 40 Hudson REB: Cardiovascular Pathology, Baltimore, 1970, The Williams & Wilkins Company, p 1594 41 Ohtsuki Y, Kobayashi S, Hayashi T, Ohmori M: Angiosarcoma of the heart. Report of a case and review of the literature. Acta Pathol Jpn 23:407-413, 1973 42 Hollingsworth JH, Sturgill BC: Treatment of primary angiosarcoma of the heart. Am Heart J 78:254-258, 1969 43 Luper WE, Klima T, Klima M: Angiosarcoma of the heart. Cardiovasc Dis Bull Texas Heart Inst 4:87-97, 1977 44 Coqueran J: Primary heart sarcomas. Two operated cases. Ann Chir Thorac Cardiovasc 11:57-63, 1972 45 Wenger R: Successfully operated haemangioblastoma of the right atrium. Z Kreislauforsch 61:953-963, 1972 46 Glancy DL, Morales JB, Roberts WC: Angiosarcoma of the heart. Am J Cardiol 21:413-419, 1968 47 Gableman C, Al-Sadir J, Lamberti J, Fozzard HA, Laufer E, Replogle RL, Myerowitz PD: Surgical treatment of recurrent primary malignant tumor of the left atrium. J THORAC CARDIOVASC SURG 77:914-921, 1979 48 Hardin NJ, Wilson JM, Gray GF, Gay W A: Experience with primary tumors of the heart. Clinical and pathological study of seventeen cases. Johns Hopkins Moo J 134:141-155,1974
THoRAc CARDIOVASC SURG
88:439-446, 1984
Cardiac tumors Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients Between 1961 and 1983, 20 patients underwent operation at the Texas Heart Institute for primary tumors other than classical benign myxoma. Total follow-up was 70.7 patient years (mean 3.5 years). There were 10 adults and 10 childrenunder 12 years of age. There weretwo operativedeatm and four late deaths, All of the pediatric patients bad benign Iesiom (five fibromas, five rbabdomyomas), and only one patient in this group died (during the operation), All operativesurvivors are alivebetween 0.9 and 18 years postoperatively, although in three cases excision was incomplete. Of the 10 adult patients, five bad benign Iesi~ all were completely excised. In the other five adult patients, unresectable malignant tumors were found, and all four operativesurvivors died of metastatic disease within 8 monem. Benigncardiac tumors in childhood bave an excellent prognosiswhen completely excised and appear to bave a good short-term prognosis even when excision is incomplete. Although prognosis for benign tumors in adults is good, malignant tumors are associated with very poor survival.
Ian J. Reece, M.B., ER.C.S.,· Denton A. Cooley, M.D., O. H. Frazier, M.D., Grady L. Hallman, M.D., Penny L. Powers, B.S.N., and Carlos G. Montero, M.D.,
Houston, Texas
Cardiac tumors are infrequent and until the early 1950s were not amenable to surgical treatment. I With the introduction of cardiopulmonary bypass, interest in surgical treatment of cardiac tumors increased. Bypass was first utilized for tumor resection in 1954.2 Nadas and Ellison' reported that primary cardiac tumors are seen in 1:10,000 routine autopsies and are found ten to twenty times less frequently than cardiac involvement by secondary growths or direct extension. Griffiths,' in reviewing 500 primary tumors, found 50% to be myxomas and 20% to be rhabdomyomas. Almost all of the malignant lesions were sarcomatous. Between 1961 and 1983, over 52,500 patients underwent cardiac operations at this institution, and during
From the Section of Surgery, Texas Heart Institute of St. Luke's Episcopal and Texas Children's Hospitals, Houston, Texas. Received for publication Oct. 28, 1983. Accepted for publication Dec. 5, 1983. Address for reprints: Denton A. Cooley, M.D., Texas Heart Institute, P.O. Box 20345, Houston, Texas 77225. *Senior Registrar in Cardiothoracic Surgery, Glasgow Royal Infirmary, Glasgow, Scotland. Presently Cardiovascular Research Fellow, Cardiovascular Surgical Research Laboratories, Texas Heart Institute, Houston, Texas.
that period only 20 primary cardiac tumors were found. During the same period, 51 benign myxomas were excised. The incidence of primary tumor (other than benign myxoma) was less than one tumor in every 2,500 cases. This report details the clinicopathological spectrum of patients with cardiac tumors other than classical benign myxomas at this institution and includes long-term follow-up of children with completely and incompletely excised growths.
Patients and methods The hospital records and diagnostic data of 20 patients were reviewed. Survivors were contacted by telephone (l1/14 patients). One patient was lost to follow-up, and data for two patients were obtained from their last hospital visits 2 and 2.5 years postoperatively. Total follow-up for this series is 70.5 patient-years (mean 3.5 patient-years) and is 95% complete.
Results Between 1961 and 1983, 20 patients underwent operations for primary cardiac tumors other than classical benign myxoma. There were 12 females and eight males ranging in age from I month to 59 years (mean 439
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The Journal of Thoracic and Cardiovascular Surgery
Reece et al.
Table I. Cardiac tumors in children, Texas Heart Institute experience Case No.
Age, sex
Lesion
Procedure
Site
12, F
Fibroma
RV mass extending into AV groove
2
JIll, F
Rhabdomyoma
3
YIl, M
Rhabdomyoma
4
til, F
Rhabdomyoma
Posterior leaflet of MV and adjacent anulus obstructing MV Anterior wall of RV obstructing RV outflow Posterolateral LV wall
5
\012, F
Rhabdomyoma
6
1-\', M
Fibroma
7
2M
Fibroma
8
1'i2, F
Fibroma
9*
6F
Fibroma
IF
Rhabdomyoma
10
Enucleation of tumor after freeing RCA Excision of mass and base; repair of MV Excision of mass via right ventriculotomy Excision of tumor; emergency reattachment of papillary muscle Excised after endocardial mapping
LV free wall causing LVOT obstruction RV free wall adjacent to LAD Excised Ventricular septum and diaphragmatic Debulking of tumor, 90% excised wall of RV Ventricular septum causing LVOT Enucleated and excised obstruction LV free wall, septum displacing LA Biopsy only and MV RA mass extending into IVC and Excision of mass obstructing TV in diastole Ventricular septum adjacent to apex Excision
Resection
Complete Complete Complete Incomplete'
Complete
Incomplete Complete Incomplete Incomplete Complete
Legend: AV,Atrioventricular. RCA,Right coronary artery. RV, Right ventricle. LV, Leftventricle. RA,Rightatrium. LA,Left atrium. MV, Mitral valve. LVOT, Left ventricular outflow tract. LAD, Left anterior descending coronary artery. PVC, Premature ventricular contraction. IVe, Inferior vena cava. TV,Tricuspid valve. 'Wilms' tumor was diagnosed in infancy, and the patient underwent right nephrectomy, radiotherapy, and chemotherapy.
20.7 years). There were 10 children younger than 12 years of age, including seven children under 1 year. The mean age of those with fibromas (4.3 years) was significantly greater than the mean age of those with rhabdomyomas (5 months, p = 0.001). Fifteen of the tumors were benign (75%), and five were malignant (25%). Origin of the tumors included seven from atrial structures (three malignant), 10 from ventricular structures (one malignant), and one from the epicardial fat between the great vessels; two tumors appeared to originate at the atrioventricular junction (one malignant). Clinical presentation (Tables I and II). Of the 10 children, one had symptoms of dyspnea, and one had congestivecardiac failure. Three children were found to have murmurs on routine examination, and one was undergoing evaluation of seizures. Three children had arrhythmias: Two of these had recurrent ventricular tachycardia, and the other had had a cardiac arrest with subsequent congestive heart failure. Patient 5 was known to have tuberous sclerosis, and Patient 2 had evidence of the disease 1.5 years after removal of a cardiac tumor. Both of these children had rhabdomyomas that were completely excised. One child is asymptomatic 2 years postoperatively; the other is incapacitated from tuberous sclerosis 2.5 years postoperatively.
The incidence of tuberous sclerosis in patients with rhabdomyoma, therefore, was 40% (2/5). In two other children, tumors were suspected after chest roentgenogram for evaluation of noncardiac symptoms. Cardiomegaly was present clinically and was confirmed by chest roentgenogram in five children (50%). Calcification in the region of the right atrium was present in one child (Patient 9). Heart size appeared normal in four patients. Echocardiography was used preoperatively in four children. In each case, it revealed a filling defect or definite abnormality. Cardiac catheterization was performed in' seven cases and revealed or confirmed the presence of cardiac tumors in all instances. In two children, endomyocardial mapping was also done, and ectopic foci within the left ventricle associated with a tumor were found in both cases. Both tumors were rhabdomyomas. One was incompletely excised, and the other was completely excised. In one child (Patient 9), the position and extent of a right atrial mass was evaluated by inferior vena cavogram. In the 10 adult patients, a tumor was found during an operation for mitral regurgitation in one case (Patient 15) and for coronary artery disease in another (Patient 19). In the remaining eight adults, dyspnea was present in six (75%), chest pain in two (25%), and hemoptysis,
Volume 88 Number 3 September. 1984
Outcome
Cardiac tumors
Follow-up
Status
Survived
2 yr
Asymptomatic
Survived
2.5 yr
Survived
1 yr
Tuberous sclerosis, cataracts, and cardiomyopathy Asymptomatic
Died
Survived
2 yr
Asymptomatic
Survived
10 yr
Asymptomatic
Survived
18 yr
Occasional Pv'Cs only
Survived
2.5 yr
Asymptomatic
Survived
IO yr
Asymptomatic
Survived
2 yr
No cardiac symptoms
heart failure, cardiac tamponade, and paroxysmal supraventricular tachycardia on one occasion each. One patient was found to have cardiomegaly on routine chest roentgenogram. The important clinical features and histologic diagnoses are summarized in Tables I and
II. Operation and survival. The operation was performed with cardiopulmonary bypass in 19 patients and via median sternotomy in 18. Excision was considered by the surgeon to be macroscopically complete in seven of the 10 children (70%), in all of the adults with benign lesions, but in none of the patients with malignant tumors. There was one operative death in the pediatric group. This child (Patient 4, Table I) died after detachment of the posterior papillary muscle following resection of a left ventricular rhabdomyoma, despite emergency reoperation and reimplantation of the muscle. Therefore, the operative survival rate was 90%, and there have been no late deaths during follow-up of 1 to 18 years (mean 4.1 years). The three children in whom excision was incomplete were all free of cardiac symptoms or signs attributable to residual tumor at a mean follow-up of 4.5 years (range 1 to 10 years). One child who had biopsy only is alive and well 2.5 years after operation. In the adult group, there was one operative death (10%) in a patient (Patient 11) undergoing her third operation for recurrent myxosarcoma of the left atrium,
441
which proved to be unresectable. All patients with malignant tumors who survived the operation died within 8 months of metastatic disease. One adult with a benign lesion (Patient 15) was lost to follow-up; the four other patients are all asymptomatic 31 months to 22 years after operation (mean follow-up 11.4 years). Discussion Benign lesions: Pediatric patients. Benign rhabdomyoma. Benign rhabdomyoma is the most common cardiac tumor in both. infants and children,' though the neoplastic nature of cardiac rhabdomyoma is controversial." Heath" considers this malformation to be a hamartoma, and the strong association between cardiac rhabdomyoma and tuberous sclerosis?" is consistent with this concept. Since cardiac rhabdomyoma was first described in 1862 by von Recklinghausen," more than 110 cases have been reported in the literature; tuberous sclerosis was present in about 50% of these cases. All of our patients were under 1 year of age at the time of operation, and it is probable that the ease with which these lesions were excised is a consequence of operating before there was significant displacement of surrounding structures or major secondary changes in the cardiac muscle or lungs by obstructive lesions. The poor prognosis in untreated lesions reported by Kidder,'? with a 60% mortality by 1 year of age, was undoubtedly reversed by early operation in our patients. The mean follow-up in the operative survivors is now 1.9 years with no late deaths. Subaortic stenosis caused by cardiac rhabdomyoma has been described 1I. 12 and was present in one of our patients. The lesion was successfully excised through the aortic valve. Three patients had arrhythmia: Two had ventricular tachycardia, and one patient had a "cardiac arrest" followed by congestive heart failure. In the two patients with recurrent ventricular tachycardia, intraoperative endocardial mapping was performed, and in one patient cryoablation was used to remove an ectopic focus adjacent to an incompletely excised tumor. Although the differentiation of ventricular from supraventricular tachycardia may be difficult in infants," the correct diagnosis is important. In our two patients, tumors were found in the left ventricular wall and apical septal region and were undoubtedly the site of ectopic ventricular activity. Engle, Ebert, and Redo" reported two similar cases in which the tumors were successfully removed and the ventricular tachycardia cured; they stressed the importance of recognizing the association of tachycardia with left ventricular tumors in infants. Nodal extrasystole" and heart block with atrial flutter" have also been
The Journal of Thoracic and Cardiovascular
44 2 Reece et al.
Surgery
Table II. Cardiac tumors in adults, Texas Heart Institute experience Case No.
Lesion
II
Recurrent myxosarcoma
12
43, F
13
58, F
14
21, F
15
58, M
16
32,t F
17
33, M
18
20, M
19
59, M
20
51,M
Site
Procedure
Posterior wall of LA extending Left thoracotomy; exploration of heart/Lx into superior PVsand obstructing MV Fibromyxosarcoma Posterior leaflet of MV extending MVR, atrial thrombectomy; into anulus and into LV; repair of anulus; biopsy of deposits on RV, LV, PA tumor Angiosarcoma Between cavae involving RCA and Debulking of tumor AV groove Epicardial fat of RV extending Excision Lipoma onto RA, involving RCA Anterolateral aspect of RA Excision Hemangioma (patient required mitral repair for ruptured chordae) Angiomatous hamartoma RV extending into AV groove and Excision, 4 ern of RCA excised onto RA, 8 em wide at base AV groove, extending into RA Excision Angiosarcoma and RV Large LA mass involving MV, Leiomyosarcoma Excision LV, and PVs Lipoma RA, attached at IVC/RA Excision ACBX3 junction, 3 by 6 cm mass Lipoma (1.4 kg) Epicardial fat extending between Excision great vessels into transverse sinus
Resection None
Incomplete
Incomplete Complete Complete
Complete Incomplete Incomplete Complete Complete
Legend: LA, Left atrium. PV, Pulmonary vein. MV, Mitral valve. OR, Operating room. VF, Ventricular fibrillation. RV, Right ventricle. LV, Left ventricle. PA, Pulmonary artery. AV, Atrioventricular. RA, Right atrium. RCA, Right coronary artery. IVC, Inferior vena cava. ACB, Aorta-coronary bypass. 'Two previous explorations with partial tumor excision, I and 2 years previously. tOne previous exploration elsewhere.
reported in association with cardiac rhabdomyoma but were not Seenin any of our patients. In the large series of rhabdomyomas reported by Fenoglio, McAllister, and Ferraris," arrhythmia occurred in six of 36 patients and was ventricular in three and atrial in three. However, the site of the ectopy did not always correlate with the site of the lesion. Long-term survival after excision of cardiac rhabdomyomas has been reported, 14, 18 and in those patients without tuberous sclerosis, the prognosis should be excellent. The aggressive surgical approach suggested by Houser, Forbes, and Stewart'? should be encouraged in these infants, since early operation appears to be associated with excellent results, although some survivors will eventually have tuberous sclerosis. Benign fibroma. Cardiac fibromas are almost always solitary and are the second most common cardiac tumor in infants and children.' These tumors are firm and compress the surrounding structures as they grow, sometimes to huge sizes (Fig. 1).6 In common with other large series, complete excision of fibromas was less common than was complete excision of rhabdomyomas and was accomplished in
only two patients.' In our series, debulking of the tumor was performed in two patients; only in patient 8 was it impossible to undertake any procedure other than biopsy. Four of these patients had symptoms of cardiac obstruction, and one had no cardiac symptoms. The majority of fibromas are found in the interventricular septum or ventricular free walls"; they are seen only rarely in the atria." Although calcification has been seen in rhabdomyoma," intracardiac lipoma," and left atrial myxoma," it appears to be more common in fibromas" but was present in only one case in this series. Ventricular and supraventricular tachycardias did not occur in our patients with fibromas, although arrhythmia is said to occur in some 30% of these patients." Sudden death, usually following physical exertion, occurs in up to 25% of patients.P-" Although left ventricular outflow tract obstruction has been reported as a consequence of a pedunculated fibroma," it is usually secondary to septal enlargement 29, 30 and is rare." The ventricular free walls are often involved, the fibroma lying within the myocardium. Patient I had the unusual feature of encapsulation
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Cardiac tumors 4 4 3
Number 3 September, 1984
Outcome
Follow-up
Died in OR; VF on induction of anesthesia
Died, multiple metastases
2 mo
Died, multiple metastases
6 mo
Asymptomatic
13 yr
Uneventful hospital recovery Lost to follow-up
10 days
Asymptomatic
22 yr
Died, multiple metastases
6 mo
Died, cerebral metastases
8 mo
Asymptomatic
31 mo
Asymptomatic
8 yr
of the right coronary artery on the epicardial surface of the heart. The fibroma was dissected from the artery, which was left intact. Although first described in 1855 by Luschka," multiple fibromas in the same heart have been reported only twice'l" and were not seen in our series. The prognosis for an incompletely resected tumor must be guarded, but it would seem logical to explore all cases in which the diagnosis is made, since almost all rhabdomyomas and more than 50% of fibromas can be completely excised and many other fibromas substantially debulked. Long-term survival after complete excision of the fibroma was reported by Williams and associates," with survival up to 17 years after operation, and is confirmed in our patients. It has been suggested that, with noninvasive investigations and follow-up, operation may be avoided in some cases." However, our experience with these patients in terms of low operative mortality and excellent clinical outcome should encourage early surgical intervention before the development of secondary myocardial or pulmonary vascular changes or sudden death." The role of echocardiography, especially two-dimensional echo studies, is well-established in the diagnosis and follow-up of infants and children with cardiac tumors." 19,34. 3S Cardiac catheterization, although less
useful for diagnostic purposes, is still valuable" and is necessary to exclude other congenital lesions in this young group of patients, especially when murmurs or heart failure are present. Simultaneous biventricular contrast injection is particularly useful in delineating septal enlargement (Fig. 2). Benign lesions: Adult patients. lipoma. Paget" was the first to describe cardiac lipoma, but his description concerned a sheep! In his review of cardiac tumors, Heath" cited only 30 cases in the literature. Lipomas may arise from epicardial or pericardiaI fat and often contain elements of surrounding connective tissues. As would be expected, the prognosis for this group of patients is excellent; however, in our patient 14, the lesion surrounded the right coronary artery and protruded into the right atrial cavity, so that extensive dissection and repair were necessary. Arrhythmias have been reported in patients with lipomatous atrial septal hypertrophy and may cause significant mortality and morbidity,' but they do not appear to be associated with lipomas on the surface of the heart and did not occur in our patients. Hemangioma. Hemangiomatous malformations of the heart have been reported infrequently,' most being incidental autopsy findings. Patient 16, however, had undergone thoracotomy at another institution for a presumed pericardial cyst associated with premature atrial extrasystoles. An extensive hemangioma which involved the anterior atrioventricular groove and the right coronary artery was found and left in situ. Several weeks later, via a median sternotomy, the 450 gm tumor was excised with a 4 em length of right coronary artery and a portion of the right ventricular outflow tract. The patient remains well 22 years after the operation and has no cardiac symptoms. A similar case was reported by Scully, Mark, and Mclveely," in which excision proved impossible but in which the echocardiogram was useful in delineating the mass and its extent, so that a correct preoperative pathological diagnosis could be made. It would appear, however, that even if a large hemangiomatous tumor is diagnosed preoperatively, exploration should still be undertaken, as there will be patients in whom complete excision can be performed and in whom long-term survival may then be expected. Malignant lesions: Adult patients. Primary malignant tumors of the heart occur approximately 20 times less frequently than does metastatic cardiac involvement." Metastatic deposits are not infrequently found at autopsy in patients dying of carcinomatosis (10%), but generally they do not displace, deform, or obstruct
444 Reece et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 1. Operative photograph from Patient 6, illustrating the relatively large size of this fibroma, which was completely excised.
Fig. 2. Biventricular angiogram in Patient 6 showing the extent of septal thickening resulting from a benign fibroma. cardiac structures. Primary malignant tumors invade the heart and surrounding structures and produce chest pain, pericardial effusion, and cardiac obstruction. Angiosarcoma. Angiosarcoma, first described by Mennig in 1888,40 is the most frequent primary malignant cardiac tumor, accounting for about 15% of the cardiac sarcomas.' Approximately63%of more than 50 cases reported have been found principally in the right atrium," although the diagnosis was made in fewer than 10 patients during life." Our two patients, one of whom has been describedpreviously,"had many of the clinical and pathological features of this tumor. Symptoms of
right-sided heart failure were present in both, and pericardial tamponade occurred in one. Both had chest pain. The fact that these symptomshad been presentfor almost a year suggests that delayed presentation may playa role in the poor prognosis of this disease. Gross cardiomegaly on chest roentgenogram, but no evidence of disseminateddisease, again suggests that the outlook could be improved with earlier diagnosis. Echocardiography was of diagnostic use in one of our patients and revealed extensive intracavitary extension and displacement of the tricuspidvalve. Classically, metastasesoccur in the lungs; pulmonary metastasis occurred early (3 months) in one patient and was present at autopsy in the other. Both patients had hepatic metastases also. Despite extensive surgical resection, tumor excision was macroscopically incomplete in both patients, and both were subsequently treated with a variety of chemotherapeutic agents. Interestingly, no residual disease was found in the hearts at autopsy, although the chemotherapy did not affect the metastases. The combineduse of operationand chemotherapyhas been associatedwith prolongedsurvival in some patients with cardiac hemangiosarcoma.r'" but these treatments did not appear to influencesignifIcantly the courseof the disease in our patients, both of whom died 6 months after operation. Eighty-eight percent of previously reported patients were dead within 9 months," although Hollingsworth and Sturgill? had a patient who underwent incomplete excision followed by cobalt 60 radiotherapy and a prolonged course of systemicchemotherapy who was free of disease 10 months later. The
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Cardiac tumors 44 5
Number 3 September, 1984
occurrence of a bloody pericardial effusion, right-sided heart failure, and pleuritic chest pain should suggest cardiac angiosarcoma. Early aggressive operation followed by radiation and systemic chemotherapy offer, at present, the only hope of prolonged survival. Myxosarcoma. Myxosarcomas arise generally in the left atrium and often involve the mitral valve. Although some are clearly myxomatous, many are predominantly fibrous and have been called malignant fibrous histiocytoma or fibromyxosarcoma.' These tumors are locally invasive and may metastasize or produce systemic
emboli.' In our series of malignant cardiac tumors, 2 patients were found to have this lesion. Both had symptoms of mitral valve disease, one with mitral obstruction and the other with mitral regurgitation from extensive irregularity and derangement of the posterior leaflet of the mitral valve. The second patient (Patient 12) had multiple intrapericardial and epicardial secondary deposits. At autopsy, 2 months after the operation, multiple hepatic deposits were found as well. Mediastinal glands removed at operation were also invaded by the tumor. Patient 11 underwent removal of a left atrial myxoma in 1980. The excised mass was thought to be a low-grade malignancy. One year later another mass, this time sessile and adjacent to the left upper lobe vein, was excised; angiography 6 months later revealed another recurrence. The tumor appeared to involve the posterior wall of the left atrium, and radical excision including the adjacent left lung was planned. However, the patient died during exploration, and resection was, therefore, not possible. The clinical course of this patient is similar to that of a case reported by Gableman and associates." It confirms the palliative value of resection of recurrent left atrial tumors and again emphasizes the need for wide excision of atrial myxomas at the initial operation. Since mitral valve symptoms are the only consistent feature of this disease, echocardiography may be of value in differentiating benign from malignant lesions when the tumor extends into adjacent structures. Early and wide excision seem to offer the best treatment, although our operative survivor died 2 months postoperatively. Leiomyosarcoma. This tumor is rare; only eight cases have been reported through 1979.5 A well-documented case by Hardin and colleagues" exhibited features very similar to those of our Patient 19. Both patients had dyspnea, pulmonary congestion, and hemoptysis. In both cases, the tumor filled the left atrium and appeared to originate from the endocardium between the right pulmonary veins. Death occurred 3 months after the
onset of symptoms. Our patient died of multiple cerebral metastases 8 months postoperatively despite radiotherapy and chemotherapy.
Conclusion The clinical and pathological spectrum of cardiac tumors in both adults and children seen over a 22 year period confirms the excellent short-term and mediumterm prognosis in children with completely excised benign lesions and indicates a good early prognosis in cases in which only partial excision is possible. Benign tumors in adults appear to have an excellent long-term prognosis, but this series confirms the very poor results obtained with primary malignant cardiac tumors and indicates that even if the primary lesion can be eradicated, metastases may well be present in occult form and will usually cause death within a year.
2
3 4 5
6 7 8
9
10 11
12
13
REFERENCES Maurer ER: Successful removal of tumors of the heart. J THORAC SURG 23:479-483, 1952 Crafoord CL: Discussion on mitral stenosis and insufficiency, (Proceedings of the) International Symposium on Cardiovascular Surgery, Henry Ford Hospital, Detroit, CR Lam, ed., Philadelphia, 1955, W. B. Saunders Company, p 202 Nadas HS, Ellison RC: Cardiac tumors in infancy. Am J Cardiol 21:363-366, 1968 Griffiths GC: A review of primary tumors of the heart. Prog Cardiovasc Dis 7:465-470, 1965 McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system, Atlas of Tumor Pathology, Vol 15, Washington D. C., 1978, Armed Forces Institute of Pathology Heath D: Pathology of cardiac tumors. Am J Cardiol 21:315-327, 1968 Critchley M, Earl CJC: Tuberous sclerosis and allied conditions. Brain 55:311-314, 1932 Shaher RM, Mintzer J, Farina M, Alley R, Bishop M: Clinical presentation of rhabdomyoma of the heart in infancy and childhood. Am J Cardiol 30:95-103, 1972 von Recklinghausen F: Verhandlungen der Gesellschaft fur Gerburtshulfe, Herr v. Recklinghausen tegt der Gesellschaft ein Herz von einem Neugeborenen. Monatsear Geburtsk 20:1-2, 1862 Kidder LA: Congenital glycogenic tumors of the heart. Arch Pathol 49:55-59, 1950 Kuehl KS, Perry LW, Chandra R, Scott LP: Left ventricular rhabdomyoma. A rare cause of subaortic stenosis in the newborn. Pediatrics 46:464-468, 1970 Shaher RM, Farina A, Alley R, Mansen P, Bishop M: Congenital subaortic stenosis in infancy caused by rhabdomyoma of the left ventricle. J THORAC CARDIOVASC SURG 63:157-163, 1972 Ehlers KH: Supraventricular and ventricular dysrhythrnias in infants and children, Cardiovascular Clinics.
4 4 6 Reece et al.
Pooiatric Cardiology, Vol 4, MA Engle, ed., Philadelphia, 1972, F. A. Davis Company, pp 59-84 14 Engle MA, Ebert PA, Redo SF: Recurrent ventricular tachycardia due to resectable cardiac tumor. Report of two cases in two-year-olds with heart failure. Circulation 50:1052-1057, 1974 15 Golding R, Reed G: Rhabdomyoma of the heart. Two unusual clinical presentations. N Engl J Moo 276:957959, 1967 16 Engle MA, Ito T, Ehlers KH, Goldberg WP: Rhabdomyomatosis of the heart. Diagnosis during life with clinical and pathological findings (abstr). Circulation 26:713, 1962 17 Fenoglio JJ, McAllister HA, Ferrans VJ: Cardiac rhabdomyoma. A clinicopathological and electron microscopic study. Am J CardioI38:241-251, 1974 18 Harinck E, Moulaert AJMG, Rohmer J, Brom G: Cardiac rhabdomyoma in infancy. Case report. Acta Paediatr Scand 63:283-286, 1974 19 Houser S, Forbes N, Stewart S: Rhabdomyoma of the heart. A diagnostic and therapeutic challenge. Ann Thorac Surg 29:373-377, 1980 20 Culliford AT, Isom OW, Trehan NK, Doyle E, Gorstein F, Spencer FC: Benign tumors of the right atrium necessitating extensive resection and reconstruction. J THORAC CARDIOVASC SURG 76:178-182, 1978 21 Bickford BJ, Egan M, Bryce AG: Surgical treatment of tumors of the heart. A report of two cases. Br J Surg 73:514-519,1956 22 Haviar V, Siska K, Klein F: Uber eine mit Erfolg operierte Herzgeschwulst von interessantem feingeweblichen Aufbau. Cardiologia 29:132-142, 1956 23 Stewart J, Saunders NR: Left atrial myxoma with extensive calcification. Thorax 37:224-225, 1982 24 Waaler PE, Svendsen S, Halvorsen JF: Intramural calcified fibroma of the heart. Acta Paediatr Scand 61:217222, 1972 25 Aryanpur I, Nazarian I, Razmura M, Shiekh MA, Khonsari S: Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision. Am J Dis Child 130:1265-1267, 1976 26 Geha AS, Weidman WH, Soule EH, McGoon DC: Intramural ventricular cardiac fibroma. Circulation 36:427-440, 1967 27 Folger GM, Peters HJ: Nodular fibroelastosis (fibroelastic hamartoma). A tumorous malformation of the heart. Am J Cardiol 21:420-427, 1968 28 Oliva PB, Breckinridge JC, Johnson ML, Brantigan CO, O'Meara OP: Left ventricular outflow obstruction produced by a pedunculated fibroma in a newborn. Clinical, angiographic, echocardiographic and surgical observations. Chest 74:590-593, 1978 29 McCue CM, Henningar GR, Davis E, Ray J: Congenital subaortic stenosis caused by fibroma of left ventricle. Pediatrics 16:372-377, 1955
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30 Luschka H: Fibroma in the heart muscle. Virchows Arch (Pathol Anat) 8:343-347, 1855 31 Zander R: Fibroma of the heart. Vichows Arch (Pathol Anat) 80:507-510, 1880 32 Fernando SSE: Cardiac fibroma (fibrous hamartoma) of infancy. Two case reports. Pathology 11: 111-117, 1979 33 Williams DB, Danielson GK, McGoon DC, Feldt RH, Edwards WD: Cardiac fibroma. Long-term survival after excision. J THORAC CARDIOVASC SURG 84:230-236, 1982 34 Charuzi Y, Mills H, Buchbinder NA, Marshall LA: Primary intramural cardiac tumors. Long-term follow-up. Am Heart J 106:414-419, 1983 35 Bini RM, Westaby S, Bargeron LM, Pacifico AD, Kirklin JW: Investigation and management of primary cardiac tumors in infants and children. J Am Coli Cardiol 2:351-357, 1983 36 Arciniegas E, Hakimi M, Farooki ZQ, Truccone NJ, Green EW: Primary cardiac tumors in children. J THORAC CARDIOVASC SURG 79:582-591, 1980 37 Paget SJ: Lectures on Surgical Pathology, 00 3, W Turner, ed., London, 1870, Songman, p 447 38 Scully RE, Mark EJ, McNeely BU: Case records of the Massachusetts General Hospital. Case 4-1983. N Engl J Moo 308:206-214, 1983 39 Fallon JT, Erdmann JA: Cardiac neoplasms, The Practice of Cardiology, RA Johnson, E Haber, WG Austen, OOs., Boston, 1980, Little Brown & Company, pp 695-705 40 Hudson REB: Cardiovascular Pathology, Baltimore, 1970, The Williams & Wilkins Company, p 1594 41 Ohtsuki Y, Kobayashi S, Hayashi T, Ohmori M: Angiosarcoma of the heart. Report of a case and review of the literature. Acta Pathol Jpn 23:407-413, 1973 42 Hollingsworth JH, Sturgill BC: Treatment of primary angiosarcoma of the heart. Am Heart J 78:254-258, 1969 43 Luper WE, Klima T, Klima M: Angiosarcoma of the heart. Cardiovasc Dis Bull Texas Heart Inst 4:87-97, 1977 44 Coqueran J: Primary heart sarcomas. Two operated cases. Ann Chir Thorac Cardiovasc 11:57-63, 1972 45 Wenger R: Successfully operated haemangioblastoma of the right atrium. Z Kreislauforsch 61:953-963, 1972 46 Glancy DL, Morales JB, Roberts WC: Angiosarcoma of the heart. Am J Cardiol 21:413-419, 1968 47 Gableman C, Al-Sadir J, Lamberti J, Fozzard HA, Laufer E, Replogle RL, Myerowitz PD: Surgical treatment of recurrent primary malignant tumor of the left atrium. J THORAC CARDIOVASC SURG 77:914-921, 1979 48 Hardin NJ, Wilson JM, Gray GF, Gay W A: Experience with primary tumors of the heart. Clinical and pathological study of seventeen cases. Johns Hopkins Moo J 134:141-155,1974