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Operative treatment of an unresectable tumor of the left ventricle
J
THORAC CARDIOVASC SURG
81:797-799, 1981
Operative treatment of an unresectable tumor of the left ventricle A 17-year-old girl presented with an enlarged cardiac silhouette on routine chest roentgenogram. After clinical evaluation, echocardiography, and pericardiocentesis failed to provide a diagnosis, exploratory thoracotomy and biopsy revealed an unresectable left ventricular fibroma. The tumor continued to enlarge and began causing ventricular arrhythmia. Therefore. she underwent cardiac transplantation 2 years after the initial diagnosis was made. She is now alive and well 18 months following transplantation.
Stuart W. Jamieson, M.D., Vincent A. Gaudiani, M.D., Bruce A. Reitz, M.D., Philip E. Oyer, M.D., Edward B. Stinson, M.D., and Norman E. Shumway, M.D.,
Stanford, Calif.
Cardiac tumors are an uncommon cause of cardiovascular disease. The majority are atrial myxomas, which are generally amenable to resection. Ventricular tumors are distinctly rare. The subject of this case report is a 17-year-old girl who had an enlarging and unresectable left ventricular fibroma. She was successfully treated by cardiac replacement and is the first patient known to undergo transplantation for this indication.
Case report A 17-year-old white woman was well until routine preoperative evaluation for a dental operation revealed a markedly enlarged cardiac silhouette by chest roentgenogram. She had no symptoms referable to the cardiovascular system. On physical examination she was a well-developed, healthy appearing woman. Blood pressure was 130/80 mm Hg, and pulse rate was 80 beats/min and regular. She had normal peripheral and jugular venous pulses. The point of maximal impulse was in the fifth intercostal space in the midclavicular line, and heart sounds were normal. Hemogram, electrolytes, blood urea nitrogen, glucose, and sedimentation rate were also within normal limits. Representative chest roentgenograms are shown in Fig. I. The electrocardiogram showed sinus rhythm with an intraventricular conduction delay. Echocardiography showed normal chamber size and no evidence of pericardial fluid. Left ventricular wall thickness could not be accurately assessed. Gallium 66 citrate scan did From the Department of Cardiovascular Surgery, Stanford University School of Medicine, Stanford, Calif. Received for publication Aug. 19, 1980. Accepted for publication Nov. 10, 1980. Address for reprints: Stuart W. Jamieson, M.D., Department of Cardiovascular Surgery, A206, Stanford University Medical Center, Calif. 94305.
not show areas of abnormal uptake within the thorax. Thallium 201 myocardial scan, however, showed areas of patchy uptake over the anterolateral wall of the left ventricle. Cardiac catheterization showed normal left and right ventricular pressures. The cardiac index was 5.7 L'min/rn-. Left atrial contrast injection showed cephalad displacement of the left atrial appendage by tumor or fluid mass. Repeated attempts at pericardiocentesis were unsuccessful. The working diagnosis at this time was pericardial cyst. She was admitted electively 8 months after her initial workup for exploratory thoracotomy. She had remained asymptomatic, but outpatient Holter monitoring had shown multifocal premature beats, and she was begun on digoxin. At exploration the pericardium was not adherent to the epicardium. There were four large confluent tumor masses occupying the anterolateral and posterior surfaces of the left ventricle and replacing approximately 60% of the left ventricular muscle mass. In some areas left ventricular wall thickness was estimated at 4 to 5 cm. Biopsy was obtained, and the diagnosis of left ventricular fibroma was made. Because the tumor was too extensive to permit resection, she was referred to our center as a potential transplant recipient. By this time she had experienced several episodes of presyncope probably caused by arrhythmias. Repeat evaluation confirmed normal resting hemodynamics. When a suitable donor became available she underwent cardiac transplantation. Cardiac replacement was performed without difficulty. At operation the pericardium was densely adherent to the heart, which was grossly enlarged. These adhesions appeared to be the result of her previous operation rather than intrinsic involvement with the tumor. The heart was somewhat difficult to remove because of its size, but it was not felt that any tumor had been left within the pericardial cavity. The resected specimen is shown in Fig. 2. At pathological examination it weighed 1,030 gm. With the exception of the septum, the remainder of the left ventricular myocardium had been replaced by firm, white tumor, 12.5 by 8.0 by 8.5 cm. Ventricular chamber size was normal. Coronary arteries were also normal, although the left anterior descending was splayed out
0022-5223/81/050797+03$00.30/0 © 1981 The C. V. Mosby Co.
797
The Journal of Thoracic and Cardiovascular Surgery
798 Jamieson et al.
, Fig. 1. Preoperative chest x-ray films.
Fig. 2. Cut section of the heart including the left ventricular septum and free wall with a large tumor completely replacing the left ventricular free wall.
Fig. 3. Histopathological section reveals benign proliferation of fibrocytic and histiocytic appearing cells with a pronounced storiform pattern and bland cell structure.
over the tumor mass. Histopathological section of the tumor showed a benign proliferation of fibrocytic and histiocytic appearing cells with a pronounced storiform pattern and with bland cell structure (Fig. 3). Mitoses were rare. The final pathological diagnosis was that of a fibrous histiocytoma of the left ventricular free wall. After a routine postoperative course, she was discharged
on the thirty-second hospital day. She has not experienced rejection and is normally active. Her chest roentgenogram at recent follow-up is shown in Fig. 4.
Comment
This report documents the first successfully treated patient who had a cardiac tumor which because of its
Volume 81
Unresectable tumor of left ventricle
Number 5
May, 1981
799
Fig. 4. Postoperative chest x-ray films at recent follow-up.
size and location was unresectable. According to McAllister and Fenoglio, 1 left ventricular fibroma and related tumors account for 3% of tumors and cysts of the heart and pericardium collected by the Armed Forces Institute of Pathology. At our center we have treated 19 cardiac tumors in approximately 11,600 patients for an incidence of 0.2%; only four were other than atrial myxomas. None of these tumors originated in the left ventricular free wall, as did the tumor in the case presented. Left ventricular fibromas and related tumors share the characteristics of fibromas elsewhere in soft tissue. Although benign, they may interdigitate with left ventricular muscle at the tumor border and replace functioning muscle mass. In addition, they commonly expand into the ventricular conduction system and cause arrhythmias. Unless resected, such tumors invariably cause death either by arrhythmias or intractable congestive heart failure, usually at a young age. In the series of Geha and associates, 2 31 of 36 such tumors occurred in infancy or childhood. Resectability depends upon the location and size of the tumor. Ten of the 17 patients in McAllister's'' series had septal tumors and died. Of the remaining seven, two had unresectable tumors and one died following operation. Four were alive at least 2 years after operation. In our case, the
tumor encompassed the entire free wall of the left ventricle and for this reason could not be resected. Cardiac output was maintained by septal contraction against the fixed tumor mass. Although our patient had multifocal ventricular arrhythmias, she did not have a severe enough degree of cardiac failure to alter pulmonary vascular resistance and was therefore an ideal candidate for cardiac replacement. Although cardiac transplantation has been shown to effectively palliate end-stage ischemic and idiopathic cardiomyopathy," this patient provided the first known opportunity to effectively treat an inoperable cardiac tumor. REFERENCES McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system, Atlas of Tumor Pathology, Fascicle 15, Series 2, Washington. 1978, Armed Forces Institute of Pathology 2 Geha AS, Wiedman WH, Soule EH, McGoon DC: Intramural ventricular fibroma. Successful removal in two cases and review of the literature. Circulation 36:427-440. 1967 3 McAllister HA: Primary tumors and cysts of the heart and pericardium. CUff Probl Cardiol 4: 1-51, 1979 4 Jamieson SW, Reitz BA, Oyer PE, et al: Current management of cardiac transplant recipients. Br Heart J 42:703708, 1979
THORAC CARDIOVASC SURG
81:797-799, 1981
Operative treatment of an unresectable tumor of the left ventricle A 17-year-old girl presented with an enlarged cardiac silhouette on routine chest roentgenogram. After clinical evaluation, echocardiography, and pericardiocentesis failed to provide a diagnosis, exploratory thoracotomy and biopsy revealed an unresectable left ventricular fibroma. The tumor continued to enlarge and began causing ventricular arrhythmia. Therefore. she underwent cardiac transplantation 2 years after the initial diagnosis was made. She is now alive and well 18 months following transplantation.
Stuart W. Jamieson, M.D., Vincent A. Gaudiani, M.D., Bruce A. Reitz, M.D., Philip E. Oyer, M.D., Edward B. Stinson, M.D., and Norman E. Shumway, M.D.,
Stanford, Calif.
Cardiac tumors are an uncommon cause of cardiovascular disease. The majority are atrial myxomas, which are generally amenable to resection. Ventricular tumors are distinctly rare. The subject of this case report is a 17-year-old girl who had an enlarging and unresectable left ventricular fibroma. She was successfully treated by cardiac replacement and is the first patient known to undergo transplantation for this indication.
Case report A 17-year-old white woman was well until routine preoperative evaluation for a dental operation revealed a markedly enlarged cardiac silhouette by chest roentgenogram. She had no symptoms referable to the cardiovascular system. On physical examination she was a well-developed, healthy appearing woman. Blood pressure was 130/80 mm Hg, and pulse rate was 80 beats/min and regular. She had normal peripheral and jugular venous pulses. The point of maximal impulse was in the fifth intercostal space in the midclavicular line, and heart sounds were normal. Hemogram, electrolytes, blood urea nitrogen, glucose, and sedimentation rate were also within normal limits. Representative chest roentgenograms are shown in Fig. I. The electrocardiogram showed sinus rhythm with an intraventricular conduction delay. Echocardiography showed normal chamber size and no evidence of pericardial fluid. Left ventricular wall thickness could not be accurately assessed. Gallium 66 citrate scan did From the Department of Cardiovascular Surgery, Stanford University School of Medicine, Stanford, Calif. Received for publication Aug. 19, 1980. Accepted for publication Nov. 10, 1980. Address for reprints: Stuart W. Jamieson, M.D., Department of Cardiovascular Surgery, A206, Stanford University Medical Center, Calif. 94305.
not show areas of abnormal uptake within the thorax. Thallium 201 myocardial scan, however, showed areas of patchy uptake over the anterolateral wall of the left ventricle. Cardiac catheterization showed normal left and right ventricular pressures. The cardiac index was 5.7 L'min/rn-. Left atrial contrast injection showed cephalad displacement of the left atrial appendage by tumor or fluid mass. Repeated attempts at pericardiocentesis were unsuccessful. The working diagnosis at this time was pericardial cyst. She was admitted electively 8 months after her initial workup for exploratory thoracotomy. She had remained asymptomatic, but outpatient Holter monitoring had shown multifocal premature beats, and she was begun on digoxin. At exploration the pericardium was not adherent to the epicardium. There were four large confluent tumor masses occupying the anterolateral and posterior surfaces of the left ventricle and replacing approximately 60% of the left ventricular muscle mass. In some areas left ventricular wall thickness was estimated at 4 to 5 cm. Biopsy was obtained, and the diagnosis of left ventricular fibroma was made. Because the tumor was too extensive to permit resection, she was referred to our center as a potential transplant recipient. By this time she had experienced several episodes of presyncope probably caused by arrhythmias. Repeat evaluation confirmed normal resting hemodynamics. When a suitable donor became available she underwent cardiac transplantation. Cardiac replacement was performed without difficulty. At operation the pericardium was densely adherent to the heart, which was grossly enlarged. These adhesions appeared to be the result of her previous operation rather than intrinsic involvement with the tumor. The heart was somewhat difficult to remove because of its size, but it was not felt that any tumor had been left within the pericardial cavity. The resected specimen is shown in Fig. 2. At pathological examination it weighed 1,030 gm. With the exception of the septum, the remainder of the left ventricular myocardium had been replaced by firm, white tumor, 12.5 by 8.0 by 8.5 cm. Ventricular chamber size was normal. Coronary arteries were also normal, although the left anterior descending was splayed out
0022-5223/81/050797+03$00.30/0 © 1981 The C. V. Mosby Co.
797
The Journal of Thoracic and Cardiovascular Surgery
798 Jamieson et al.
, Fig. 1. Preoperative chest x-ray films.
Fig. 2. Cut section of the heart including the left ventricular septum and free wall with a large tumor completely replacing the left ventricular free wall.
Fig. 3. Histopathological section reveals benign proliferation of fibrocytic and histiocytic appearing cells with a pronounced storiform pattern and bland cell structure.
over the tumor mass. Histopathological section of the tumor showed a benign proliferation of fibrocytic and histiocytic appearing cells with a pronounced storiform pattern and with bland cell structure (Fig. 3). Mitoses were rare. The final pathological diagnosis was that of a fibrous histiocytoma of the left ventricular free wall. After a routine postoperative course, she was discharged
on the thirty-second hospital day. She has not experienced rejection and is normally active. Her chest roentgenogram at recent follow-up is shown in Fig. 4.
Comment
This report documents the first successfully treated patient who had a cardiac tumor which because of its
Volume 81
Unresectable tumor of left ventricle
Number 5
May, 1981
799
Fig. 4. Postoperative chest x-ray films at recent follow-up.
size and location was unresectable. According to McAllister and Fenoglio, 1 left ventricular fibroma and related tumors account for 3% of tumors and cysts of the heart and pericardium collected by the Armed Forces Institute of Pathology. At our center we have treated 19 cardiac tumors in approximately 11,600 patients for an incidence of 0.2%; only four were other than atrial myxomas. None of these tumors originated in the left ventricular free wall, as did the tumor in the case presented. Left ventricular fibromas and related tumors share the characteristics of fibromas elsewhere in soft tissue. Although benign, they may interdigitate with left ventricular muscle at the tumor border and replace functioning muscle mass. In addition, they commonly expand into the ventricular conduction system and cause arrhythmias. Unless resected, such tumors invariably cause death either by arrhythmias or intractable congestive heart failure, usually at a young age. In the series of Geha and associates, 2 31 of 36 such tumors occurred in infancy or childhood. Resectability depends upon the location and size of the tumor. Ten of the 17 patients in McAllister's'' series had septal tumors and died. Of the remaining seven, two had unresectable tumors and one died following operation. Four were alive at least 2 years after operation. In our case, the
tumor encompassed the entire free wall of the left ventricle and for this reason could not be resected. Cardiac output was maintained by septal contraction against the fixed tumor mass. Although our patient had multifocal ventricular arrhythmias, she did not have a severe enough degree of cardiac failure to alter pulmonary vascular resistance and was therefore an ideal candidate for cardiac replacement. Although cardiac transplantation has been shown to effectively palliate end-stage ischemic and idiopathic cardiomyopathy," this patient provided the first known opportunity to effectively treat an inoperable cardiac tumor. REFERENCES McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system, Atlas of Tumor Pathology, Fascicle 15, Series 2, Washington. 1978, Armed Forces Institute of Pathology 2 Geha AS, Wiedman WH, Soule EH, McGoon DC: Intramural ventricular fibroma. Successful removal in two cases and review of the literature. Circulation 36:427-440. 1967 3 McAllister HA: Primary tumors and cysts of the heart and pericardium. CUff Probl Cardiol 4: 1-51, 1979 4 Jamieson SW, Reitz BA, Oyer PE, et al: Current management of cardiac transplant recipients. Br Heart J 42:703708, 1979