- Email: [email protected]
Surgical pathology of cardiac tumors
Cardiovascular Pathology 12 (2003) 267 – 270
Surgical pathology of cardiac tumors $ Two decades at an urban institution Jonah Odim *, Vickram Reehal, Hillel Laks, Umang Mehta, Michael C. Fishbein Division of Cardiothoracic Surgery, Cardiology, and Anatomic Pathology, University of California Los Angeles School of Medicine, Los Angeles, CA, USA Received 6 November 2002; received in revised form 29 May 2003; accepted 9 June 2003
Abstract Background: As better diagnostic techniques and new operative approaches are developed pathologists will be called upon more often for intraoperative consultation to render a pathologic diagnosis and assess adequacy of resection. Methods: We conducted a retrospective survey of all patients presenting to our institution from 1979 to 1999. The surgical pathology and cardiothoracic surgery databases were used to identify these patients. Results: Of the 29 patients with primary cardiac neoplasms, 15 were male and 14 female. The mean age at surgery was 51.9 years (range, 7 months to 84 years). Twenty-six of 29 patients had a benign pathological diagnosis. The majority (20/26) of the benign tumors were myxomas. Other benign pathologic diagnoses included rhabdomyoma, fibroma, papillary fibroelastoma, localized fibrous tumor and lipoma. Three out of 29 were malignant: 1 fibrosarcoma, 1 malignant mesenchymoma, and 1 rhabdomyosarcoma. Six of the patients presented with thrombo-embolism, 8 with congestive heart failure symptoms, and three with chest discomfort. Five were asymptomatic or the neoplasm was an incidental finding. 2-D echocardiography established the diagnosis in all of the patients except one. Twenty-two of the 29 tumors were located in the atria (LA = 15, RA = 6, biatrial = 1) and 5 in the ventricles (LV = 1, RV = 2, biventricular = 2). Two patients in this series were referred for reresection. A median sternotomy approach was used for tumor extirpation in all patients. Three of the 29 patients have died at a mean follow-up period of 757 days (median, 118 days). There were two late deaths and one hospital (early) death in a reoperation for recurrent malignancy employing cardiac autotransplantation. One additional patient required cardiac reoperation. Conclusions: Primary cardiac neoplasms are rare and occur less commonly than metastatic disease of the heart. Congestive heart failure symptoms and thromboembolism account for close to half of the presenting signs and symptoms. 2-D echocardiography remains the mainstay of detection. Distinguishing between benign and malignant, thrombus and vegetation, and extracardiac structure is usually established by the size, shape, mobility and attachment of the mass. Clinical presentation and transesophageal echocardiographic views are extremely helpful in sharpening the accuracy of the diagnosis. Since surgery is the only reliable therapy pathologists will be called upon for intraoperative consultation. The majority of the neoplasms are benign. Malignant neoplasms are difficult to excise completely and portend a grave prognosis. D 2003 Elsevier Inc. All rights reserved. Keywords: Cardiac tumors; Myxoma; Sarcoma; Cardiac autotransplantation
1. Introduction Historically, postmortem detection of cardiac tumors was the prevailing rule. As a result, most large series are based primarily on autopsy data. As diagnostic techniques, in $ Presented at the annual meeting of the Southern California Chapter of the American College of Surgeons, January 21 – 23, 2000, Huntington Beach, CA. * Corresponding author. Division of Cardiothoracic Surgery, University of California Los Angeles School of Medicine, Box 951741, 10833 Le Conte Avenue, Los Angeles, CA 90095-1741, USA. Tel.: +1-310-2069520; fax: +1-310-825-7473. E-mail address: [email protected] (J. Odim).
1054-8807/03/$ – see front matter D 2003 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(03)00087-5
particular, 2-D echocardiography, have improved and attained widespread utility, we are detecting more antemortem cases of primary cardiac tumors. Early diagnosis and prompt complete resection is vital to successfully managing these patients long term [1,2,4]. This retrospective study reports the surgical pathology experience from one institution with primary cardiac tumors over two decades.
2. Methods We identified patients with primary cardiac tumors by searching the surgical pathology and cardiothoracic surgery
268
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
databases. A total of 29 patients with primary cardiac tumors were identified from 1979 to 1999 at UCLA medical center. Two additional patients were excluded due to insufficient information. The medical records of these patients were reviewed with special attention to demographic data, presenting signs and symptoms, method of diagnosis, pathology, tumor location, mortality, and complications. Follow up was accomplished by reviewing outpatient medical records and contact with referring physicians. All the patients in this series underwent operation by way of median sternotomy with minimal touch technique and cardiopulmonary bypass. All of the patients had tumor resection under cardioplegic arrest except one patient with sickle cell trait.
3. Results There were 15 male and 14 female patients with a mean age of 51.9 years (range 7 months to 84 years). Twenty-six (90%) of the tumors were benign and 3 (10%) were malignant (Table 1). The malignant tumors included a rhabdomyosarcoma, a malignant mesenchymoma and a fibrosarcoma. Twenty of the 26 benign tumors (77%) were myxomas. The remaining benign tumors consisted of a rhabdomyoma, a fibroma, papillary fibroelastoma, a localized fibrous tumor, and a lipoma (see Figs. 1 – 3). Twenty-two (76%) of all tumors arose from the atria, 5 (17%) from the ventricles, 1 each from the aortic and pulmonary valves, respectively (Table 2). Of the atrial tumors, 15 were found predominantly in the left chamber, 6 in the right chamber and a cardiac lipoma was biatrial. Of the ventricular tumors, 2 were located in the right chamber, 1 was located in the left chamber and 2 were biventricular (a fibrosarcoma and a rhabdomyosarcoma). About half of these patients presented with congestive heart failure and thrombo-embolism (Table 3). Eight patients (28%) presented with signs and symptoms of congestive heart failure including dyspnea, orthopnea, paroxysmal nocturnal dyspnea and peripheral edema. Six patients (21%) presented with thrombo-embolism including stroke (3), peripheral emboli (1), transient ischemic attacks (1) and right eye amaurosis fugax (1). A myxoma was discovered in an additional, otherwise asymptomatic, patient on a routine check-up. She initially declined surgery, but
Fig. 1. Rhabdomyosarcoma. Fleshy, whitish yellow, infiltrating irregular mass attached to wall of heart. A very cellular lesion composed of pleomorphic spindle cells with a high mitotic rate. Immunohistochemical stains for desmin and myoglobin were positive (not shown).
two months later, following the development of lower limb ischemia from tumor embolism she underwent resection. Four patients (14%) presented with chest discomfort including palpitations, pressure and chest pain. One of these patients had angina due to extrinsic compression of the left anterior descending artery (LAD) by fibrosarcoma involving both ventricles. Three patients presented with heart murmurs on auscultation precipitating further echocardiographic examination. Two of the three patients with cardiac murmurs were under two years of age. One patient presented with cough, pulmonary congestion, fever, chills and weight loss. One patient presented with atrial fibrillation. The last patient presented with symptoms of dizziness. Five patients (17%) were asymptomatic at presentation and the tumors were incidental findings. For example, one patient presented to the emergency department with a broken arm. An abnormal electrocardiogram prompted an echocardiogram later revealing the intracardiac mass. Echocardiography was the primary method (28 out of 29 patients) for diagnosis of cardiac tumors in this study. A cardiac tumor was discovered de novo at coronary revascularization surgery in only one patient in this series. There were two recurrent tumors in our series that were previously resected at other institutions. One patient had recurrent disease seven years after myxoma resection elsewhere. Another patient developed recurrent rhabdomyosarcoma two years following resection and adjuvant chemotherapy at an outside institution. This 42-year-old man underwent cardiac autotransplantation for recurrent disease. The disease was extensive in the left atrium
Table 1 Clinical presentation Congestive heart failure Thrombo-embolism Incidental/asymptomatic Chest discomfort Murmur Constitutional symptoms Dizziness Arrhythmia (atrial fibrillation)
8 5 6 4 3 1 1 1
(28%) (17%) (21%) (14%) (10%) (3%) (3%) (3%)
Fig. 2. Endocardial papillary fibroelastoma. Multiple small papillary projections consisting of collagenous and elastic tissue cores surrounded by extracellular matrix and covered by a layer of hyperplastic endothelial cells.
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
269
Table 3 Location of primary cardiac tumors
Fig. 3. Myxoma. Hemorrhagic mass attached to excised portion of atrial septum. The tumor is composed primarily of extracellular matrix containing numerous proliferated blood vessels and stromal cells. Hemorrhagic regions are prominent.
between the pulmonary veins and involved the mitral valve apparatus. Complete resection required reconstruction of the left atrium and mitral valve with pericardium and autotransplantation. This patient died in hospital 2 months after surgery from a sudden cardiac arrest. There was one case of metastasis to the lung, thigh and right ventricle 14 months following initial resection in a patient with malignant mesenchymoma of the pulmonary valve and artery. There have been two late deaths in this series. A 29year-old woman underwent tricuspid and mitral valve replacements 8 months after removal of a biventricular fibrosarcoma. This patient ultimately died from congestive heart failure 20 months later. The mean follow-up time in our series was 757 days (range, 5 days –15 years).
4. Discussion Columbus from Padua was credited with the first report of a cardiac tumor in 1559. Many centuries later Barnes indirectly made the first premortem clinical diagnosis of a primary cardiac sarcoma in 1934 after biopsy of a metastatic nodule. Four years later (1938) Beck successfully resected an intrapericardial teratoma [1,3,5]. In 1951, Goldberg used the invasive technique of angiography to diagnose a primary cardiac tumor. Before the introduction of echocardiography, 90% of primary cardiac tumors were diagnosed at autopsy
Table 2 Pathology of primary cardiac tumors Benign Myxoma Rhabdomyoma Fibroma Papillary fibroelastoma Localised fibrous tumor Lipoma
20 1 1 2 1 1
Malignant Rhabdomyosarcoma Malignant mesothelioma Fibrosarcoma
1 1 1
Left atrium Right atrium Biatrial Right ventricle Left ventricle Biventricular Aortic valve Pulmonary root
Benign
Malignant
14 6 1 2 1 1 1 0
1 0 0 0 0 1 0 1
[6 –10]. The advent of echocardiography has replaced the invasive methods and other nonspecific electrocardiographic and roentgenographic modalities for the diagnosis of cardiac tumors. More recently MRI and CT are being used to diagnose primary cardiac tumors [1,11,12]. They are particularly helpful in staging these lesions; to assess mural infiltration, to detect pericardial involvement, to determine tumor extension and to uncover metastatic disease. The incidence of primary cardiac neoplasms ranges from 0.0017% to 0.35%. It is known that about 72% of primary cardiac tumors are benign and 28% are malignant based on large autopsy series. Of the benign tumors, about 50% are myxomas. Large studies have shown that 75 – 80% of myxomas originate in the left atrium, 18% in the right atrium, and less than 2.5% are biatrial [13]. The remainder originate in the ventricles [8,10]. Our series showed a similar distribution. The Mayo clinic reported that the risk of recurrence of a myxoma is between 1– 3%. Many large series including ours have shown no recurrences, probably because a rim of uninvolved atrial wall is removed along with the myxoma. Hence, pathologic assessment of margins of resection is relevant to outcome. The third most common benign cardiac tumor is the papillary fibroelastoma [6]. Papillary fibroelastomas are small tumors that make up about 7% of all primary cardiac tumors. About 80% of papillary fibroelastomas are found on the valvular endocardium, but they are also found on papillary muscles, chordae tendineae, the ventricular septum or the endocardial surface. Papillary fibroelastomas of the aortic valve are known to cause sudden death and therefore must be removed expeditiously. Their characteristic papillary fronds are a dangerous source of emboli that can cause stroke and myocardial infarction. Two patients in this series with papillary fibroelastoma presented with stroke and congestive heart failure, respectively. There are a variety of tenable surgical approaches to cardiac tumors including the more recent minimally invasive approaches. The cardiac autotransplantation technique has been employed successfully only a few times to our knowledge; in Germany for a left atrial myxoma, in Yugoslavia for a malignant fibrous histiocytoma, and in Texas another for a malignant fibrous histiocytoma [7]. Autotransplantation requires excision of the diseased native heart with tumor resection and repair on the back table and then
270
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
retransplantation. Myocardial protection is achieved with hypothermia and substrate enhanced blood cardioplegia. There are no standard protocol and no criteria for quantifying the extent of negative margin required for adequate resection of cardiac tumors. One must balance the risk of recurrence with difficult cardiac reconstruction and the potential for inadequate residual myocardial mass. In this regard, the surgical pathologist has no guidelines to follow when called upon to evaluate the adequacy of resection during intraoperative consultation. The role of adjunctive therapy remains ill defined. The only clear correlation made with survival of malignant disease is completeness of resection and level of mitotic activity of the tumor [1]. The long-term prognosis of patients with malignant disease is extremely poor with postoperative survival less than a year on average [1]. In summary, congestive heart failure and thromboembolism are the most common presenting signs and symptoms of primary cardiac neoplasms. 2-D echocardiography remains the primary mode of diagnosis. Surgical resection is the treatment of choice in all patients with primary cardiac tumors. Surgical resection of benign cardiac tumors is very safe and can be done with a low mortality and low recurrence rate. Surgery has the potential to prolong and palliate life for patients with malignant disease but unfortunately most patients eventually succumb to their disease. Adjuvant therapy with chemotherapy and/or radiation therapy has not played a significant role in extending life for patients with malignant disease. As these tumors are being detected more frequently in life, surgical pathologists will be called upon more often for intraoperative consultation for diagnosis and assessment of margins of resection.
References [1] Perchinsky M, Lichtenstien S, Tyers GF. Primary cardiac tumors forty years’ experience with 71 patients. Cancer 1997;79:1809 – 15. [2] Gersak B, Mikek M, Smrkolj V, Gabrijelcic T. Results of treatment of 17 patients with heart tumor. Eur J Surg Oncol 1999;25:302 – 5. [3] Centofanti P, Di Rosa E, Deorsola L, Actis Dato G, Patane F, La Torre M, Barbato L, Verzini A, Fortunato G, Di Summa M. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999;68:1236 – 41. [4] Beghetti M, Gow R, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary cardiac tumors: a 15-year review. Am Heart J 1997;134:1107 – 14. [5] Bhan A, Mehrotra R, Choudhary S, Sharma R, Prabhakar D, Airan B, Kumar AS, Venugopal P. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thorac Surg 1998;66: 810 – 3. [6] Howard R, Aldea G, Shapira O, Kaszinica J, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881 – 5. [7] Reardon M, Defelice C, Sheinbaum R, Baldwin J. Cardiac autotransplant for surgical treatment of a malignant neoplasm. Ann Thorac Surg 1999;67:1793 – 5. [8] Peachell J, Mullen J, Bentley M, Taylor D. Biatrial myxoma: a rare cardiac tumor. Ann Thorac Surg 1998;65:1768 – 9. [9] Tackach T, Reul G, Ott D, Cooley D. Primary tumors in infants and children: immediate and long-term operative results. Ann Thorac Surg 1996;62:559 – 64. [10] Hwa J, Ward C, Nunn G, Cooper S, Lau K, Sholler G. Primary intraventricular cardiac tumors in children: contemporary diagnostic and management options. Pediatr Cardiol 1994;15:233 – 7. [11] Jacobs J, Konstantakos A, Holland F, Herskowitz K, Ferrer P, Perryman R. Surgical treatment for cardiac rhabdomyomas in children. Ann Thorac Surg 1994;58:1552 – 5. [12] Turner A, Batrick N. Primary cardiac sarcomas. A report of three cases and a review of the current literature. Int J Cardiol 1993;40: 115 – 9. [13] Reynen K. Cardiac myxomas. N Engl J Med 1995;33:1610 – 7.
Surgical pathology of cardiac tumors $ Two decades at an urban institution Jonah Odim *, Vickram Reehal, Hillel Laks, Umang Mehta, Michael C. Fishbein Division of Cardiothoracic Surgery, Cardiology, and Anatomic Pathology, University of California Los Angeles School of Medicine, Los Angeles, CA, USA Received 6 November 2002; received in revised form 29 May 2003; accepted 9 June 2003
Abstract Background: As better diagnostic techniques and new operative approaches are developed pathologists will be called upon more often for intraoperative consultation to render a pathologic diagnosis and assess adequacy of resection. Methods: We conducted a retrospective survey of all patients presenting to our institution from 1979 to 1999. The surgical pathology and cardiothoracic surgery databases were used to identify these patients. Results: Of the 29 patients with primary cardiac neoplasms, 15 were male and 14 female. The mean age at surgery was 51.9 years (range, 7 months to 84 years). Twenty-six of 29 patients had a benign pathological diagnosis. The majority (20/26) of the benign tumors were myxomas. Other benign pathologic diagnoses included rhabdomyoma, fibroma, papillary fibroelastoma, localized fibrous tumor and lipoma. Three out of 29 were malignant: 1 fibrosarcoma, 1 malignant mesenchymoma, and 1 rhabdomyosarcoma. Six of the patients presented with thrombo-embolism, 8 with congestive heart failure symptoms, and three with chest discomfort. Five were asymptomatic or the neoplasm was an incidental finding. 2-D echocardiography established the diagnosis in all of the patients except one. Twenty-two of the 29 tumors were located in the atria (LA = 15, RA = 6, biatrial = 1) and 5 in the ventricles (LV = 1, RV = 2, biventricular = 2). Two patients in this series were referred for reresection. A median sternotomy approach was used for tumor extirpation in all patients. Three of the 29 patients have died at a mean follow-up period of 757 days (median, 118 days). There were two late deaths and one hospital (early) death in a reoperation for recurrent malignancy employing cardiac autotransplantation. One additional patient required cardiac reoperation. Conclusions: Primary cardiac neoplasms are rare and occur less commonly than metastatic disease of the heart. Congestive heart failure symptoms and thromboembolism account for close to half of the presenting signs and symptoms. 2-D echocardiography remains the mainstay of detection. Distinguishing between benign and malignant, thrombus and vegetation, and extracardiac structure is usually established by the size, shape, mobility and attachment of the mass. Clinical presentation and transesophageal echocardiographic views are extremely helpful in sharpening the accuracy of the diagnosis. Since surgery is the only reliable therapy pathologists will be called upon for intraoperative consultation. The majority of the neoplasms are benign. Malignant neoplasms are difficult to excise completely and portend a grave prognosis. D 2003 Elsevier Inc. All rights reserved. Keywords: Cardiac tumors; Myxoma; Sarcoma; Cardiac autotransplantation
1. Introduction Historically, postmortem detection of cardiac tumors was the prevailing rule. As a result, most large series are based primarily on autopsy data. As diagnostic techniques, in $ Presented at the annual meeting of the Southern California Chapter of the American College of Surgeons, January 21 – 23, 2000, Huntington Beach, CA. * Corresponding author. Division of Cardiothoracic Surgery, University of California Los Angeles School of Medicine, Box 951741, 10833 Le Conte Avenue, Los Angeles, CA 90095-1741, USA. Tel.: +1-310-2069520; fax: +1-310-825-7473. E-mail address: [email protected] (J. Odim).
1054-8807/03/$ – see front matter D 2003 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(03)00087-5
particular, 2-D echocardiography, have improved and attained widespread utility, we are detecting more antemortem cases of primary cardiac tumors. Early diagnosis and prompt complete resection is vital to successfully managing these patients long term [1,2,4]. This retrospective study reports the surgical pathology experience from one institution with primary cardiac tumors over two decades.
2. Methods We identified patients with primary cardiac tumors by searching the surgical pathology and cardiothoracic surgery
268
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
databases. A total of 29 patients with primary cardiac tumors were identified from 1979 to 1999 at UCLA medical center. Two additional patients were excluded due to insufficient information. The medical records of these patients were reviewed with special attention to demographic data, presenting signs and symptoms, method of diagnosis, pathology, tumor location, mortality, and complications. Follow up was accomplished by reviewing outpatient medical records and contact with referring physicians. All the patients in this series underwent operation by way of median sternotomy with minimal touch technique and cardiopulmonary bypass. All of the patients had tumor resection under cardioplegic arrest except one patient with sickle cell trait.
3. Results There were 15 male and 14 female patients with a mean age of 51.9 years (range 7 months to 84 years). Twenty-six (90%) of the tumors were benign and 3 (10%) were malignant (Table 1). The malignant tumors included a rhabdomyosarcoma, a malignant mesenchymoma and a fibrosarcoma. Twenty of the 26 benign tumors (77%) were myxomas. The remaining benign tumors consisted of a rhabdomyoma, a fibroma, papillary fibroelastoma, a localized fibrous tumor, and a lipoma (see Figs. 1 – 3). Twenty-two (76%) of all tumors arose from the atria, 5 (17%) from the ventricles, 1 each from the aortic and pulmonary valves, respectively (Table 2). Of the atrial tumors, 15 were found predominantly in the left chamber, 6 in the right chamber and a cardiac lipoma was biatrial. Of the ventricular tumors, 2 were located in the right chamber, 1 was located in the left chamber and 2 were biventricular (a fibrosarcoma and a rhabdomyosarcoma). About half of these patients presented with congestive heart failure and thrombo-embolism (Table 3). Eight patients (28%) presented with signs and symptoms of congestive heart failure including dyspnea, orthopnea, paroxysmal nocturnal dyspnea and peripheral edema. Six patients (21%) presented with thrombo-embolism including stroke (3), peripheral emboli (1), transient ischemic attacks (1) and right eye amaurosis fugax (1). A myxoma was discovered in an additional, otherwise asymptomatic, patient on a routine check-up. She initially declined surgery, but
Fig. 1. Rhabdomyosarcoma. Fleshy, whitish yellow, infiltrating irregular mass attached to wall of heart. A very cellular lesion composed of pleomorphic spindle cells with a high mitotic rate. Immunohistochemical stains for desmin and myoglobin were positive (not shown).
two months later, following the development of lower limb ischemia from tumor embolism she underwent resection. Four patients (14%) presented with chest discomfort including palpitations, pressure and chest pain. One of these patients had angina due to extrinsic compression of the left anterior descending artery (LAD) by fibrosarcoma involving both ventricles. Three patients presented with heart murmurs on auscultation precipitating further echocardiographic examination. Two of the three patients with cardiac murmurs were under two years of age. One patient presented with cough, pulmonary congestion, fever, chills and weight loss. One patient presented with atrial fibrillation. The last patient presented with symptoms of dizziness. Five patients (17%) were asymptomatic at presentation and the tumors were incidental findings. For example, one patient presented to the emergency department with a broken arm. An abnormal electrocardiogram prompted an echocardiogram later revealing the intracardiac mass. Echocardiography was the primary method (28 out of 29 patients) for diagnosis of cardiac tumors in this study. A cardiac tumor was discovered de novo at coronary revascularization surgery in only one patient in this series. There were two recurrent tumors in our series that were previously resected at other institutions. One patient had recurrent disease seven years after myxoma resection elsewhere. Another patient developed recurrent rhabdomyosarcoma two years following resection and adjuvant chemotherapy at an outside institution. This 42-year-old man underwent cardiac autotransplantation for recurrent disease. The disease was extensive in the left atrium
Table 1 Clinical presentation Congestive heart failure Thrombo-embolism Incidental/asymptomatic Chest discomfort Murmur Constitutional symptoms Dizziness Arrhythmia (atrial fibrillation)
8 5 6 4 3 1 1 1
(28%) (17%) (21%) (14%) (10%) (3%) (3%) (3%)
Fig. 2. Endocardial papillary fibroelastoma. Multiple small papillary projections consisting of collagenous and elastic tissue cores surrounded by extracellular matrix and covered by a layer of hyperplastic endothelial cells.
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
269
Table 3 Location of primary cardiac tumors
Fig. 3. Myxoma. Hemorrhagic mass attached to excised portion of atrial septum. The tumor is composed primarily of extracellular matrix containing numerous proliferated blood vessels and stromal cells. Hemorrhagic regions are prominent.
between the pulmonary veins and involved the mitral valve apparatus. Complete resection required reconstruction of the left atrium and mitral valve with pericardium and autotransplantation. This patient died in hospital 2 months after surgery from a sudden cardiac arrest. There was one case of metastasis to the lung, thigh and right ventricle 14 months following initial resection in a patient with malignant mesenchymoma of the pulmonary valve and artery. There have been two late deaths in this series. A 29year-old woman underwent tricuspid and mitral valve replacements 8 months after removal of a biventricular fibrosarcoma. This patient ultimately died from congestive heart failure 20 months later. The mean follow-up time in our series was 757 days (range, 5 days –15 years).
4. Discussion Columbus from Padua was credited with the first report of a cardiac tumor in 1559. Many centuries later Barnes indirectly made the first premortem clinical diagnosis of a primary cardiac sarcoma in 1934 after biopsy of a metastatic nodule. Four years later (1938) Beck successfully resected an intrapericardial teratoma [1,3,5]. In 1951, Goldberg used the invasive technique of angiography to diagnose a primary cardiac tumor. Before the introduction of echocardiography, 90% of primary cardiac tumors were diagnosed at autopsy
Table 2 Pathology of primary cardiac tumors Benign Myxoma Rhabdomyoma Fibroma Papillary fibroelastoma Localised fibrous tumor Lipoma
20 1 1 2 1 1
Malignant Rhabdomyosarcoma Malignant mesothelioma Fibrosarcoma
1 1 1
Left atrium Right atrium Biatrial Right ventricle Left ventricle Biventricular Aortic valve Pulmonary root
Benign
Malignant
14 6 1 2 1 1 1 0
1 0 0 0 0 1 0 1
[6 –10]. The advent of echocardiography has replaced the invasive methods and other nonspecific electrocardiographic and roentgenographic modalities for the diagnosis of cardiac tumors. More recently MRI and CT are being used to diagnose primary cardiac tumors [1,11,12]. They are particularly helpful in staging these lesions; to assess mural infiltration, to detect pericardial involvement, to determine tumor extension and to uncover metastatic disease. The incidence of primary cardiac neoplasms ranges from 0.0017% to 0.35%. It is known that about 72% of primary cardiac tumors are benign and 28% are malignant based on large autopsy series. Of the benign tumors, about 50% are myxomas. Large studies have shown that 75 – 80% of myxomas originate in the left atrium, 18% in the right atrium, and less than 2.5% are biatrial [13]. The remainder originate in the ventricles [8,10]. Our series showed a similar distribution. The Mayo clinic reported that the risk of recurrence of a myxoma is between 1– 3%. Many large series including ours have shown no recurrences, probably because a rim of uninvolved atrial wall is removed along with the myxoma. Hence, pathologic assessment of margins of resection is relevant to outcome. The third most common benign cardiac tumor is the papillary fibroelastoma [6]. Papillary fibroelastomas are small tumors that make up about 7% of all primary cardiac tumors. About 80% of papillary fibroelastomas are found on the valvular endocardium, but they are also found on papillary muscles, chordae tendineae, the ventricular septum or the endocardial surface. Papillary fibroelastomas of the aortic valve are known to cause sudden death and therefore must be removed expeditiously. Their characteristic papillary fronds are a dangerous source of emboli that can cause stroke and myocardial infarction. Two patients in this series with papillary fibroelastoma presented with stroke and congestive heart failure, respectively. There are a variety of tenable surgical approaches to cardiac tumors including the more recent minimally invasive approaches. The cardiac autotransplantation technique has been employed successfully only a few times to our knowledge; in Germany for a left atrial myxoma, in Yugoslavia for a malignant fibrous histiocytoma, and in Texas another for a malignant fibrous histiocytoma [7]. Autotransplantation requires excision of the diseased native heart with tumor resection and repair on the back table and then
270
J. Odim et al. / Cardiovascular Pathology 12 (2003) 267–270
retransplantation. Myocardial protection is achieved with hypothermia and substrate enhanced blood cardioplegia. There are no standard protocol and no criteria for quantifying the extent of negative margin required for adequate resection of cardiac tumors. One must balance the risk of recurrence with difficult cardiac reconstruction and the potential for inadequate residual myocardial mass. In this regard, the surgical pathologist has no guidelines to follow when called upon to evaluate the adequacy of resection during intraoperative consultation. The role of adjunctive therapy remains ill defined. The only clear correlation made with survival of malignant disease is completeness of resection and level of mitotic activity of the tumor [1]. The long-term prognosis of patients with malignant disease is extremely poor with postoperative survival less than a year on average [1]. In summary, congestive heart failure and thromboembolism are the most common presenting signs and symptoms of primary cardiac neoplasms. 2-D echocardiography remains the primary mode of diagnosis. Surgical resection is the treatment of choice in all patients with primary cardiac tumors. Surgical resection of benign cardiac tumors is very safe and can be done with a low mortality and low recurrence rate. Surgery has the potential to prolong and palliate life for patients with malignant disease but unfortunately most patients eventually succumb to their disease. Adjuvant therapy with chemotherapy and/or radiation therapy has not played a significant role in extending life for patients with malignant disease. As these tumors are being detected more frequently in life, surgical pathologists will be called upon more often for intraoperative consultation for diagnosis and assessment of margins of resection.
References [1] Perchinsky M, Lichtenstien S, Tyers GF. Primary cardiac tumors forty years’ experience with 71 patients. Cancer 1997;79:1809 – 15. [2] Gersak B, Mikek M, Smrkolj V, Gabrijelcic T. Results of treatment of 17 patients with heart tumor. Eur J Surg Oncol 1999;25:302 – 5. [3] Centofanti P, Di Rosa E, Deorsola L, Actis Dato G, Patane F, La Torre M, Barbato L, Verzini A, Fortunato G, Di Summa M. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999;68:1236 – 41. [4] Beghetti M, Gow R, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary cardiac tumors: a 15-year review. Am Heart J 1997;134:1107 – 14. [5] Bhan A, Mehrotra R, Choudhary S, Sharma R, Prabhakar D, Airan B, Kumar AS, Venugopal P. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thorac Surg 1998;66: 810 – 3. [6] Howard R, Aldea G, Shapira O, Kaszinica J, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881 – 5. [7] Reardon M, Defelice C, Sheinbaum R, Baldwin J. Cardiac autotransplant for surgical treatment of a malignant neoplasm. Ann Thorac Surg 1999;67:1793 – 5. [8] Peachell J, Mullen J, Bentley M, Taylor D. Biatrial myxoma: a rare cardiac tumor. Ann Thorac Surg 1998;65:1768 – 9. [9] Tackach T, Reul G, Ott D, Cooley D. Primary tumors in infants and children: immediate and long-term operative results. Ann Thorac Surg 1996;62:559 – 64. [10] Hwa J, Ward C, Nunn G, Cooper S, Lau K, Sholler G. Primary intraventricular cardiac tumors in children: contemporary diagnostic and management options. Pediatr Cardiol 1994;15:233 – 7. [11] Jacobs J, Konstantakos A, Holland F, Herskowitz K, Ferrer P, Perryman R. Surgical treatment for cardiac rhabdomyomas in children. Ann Thorac Surg 1994;58:1552 – 5. [12] Turner A, Batrick N. Primary cardiac sarcomas. A report of three cases and a review of the current literature. Int J Cardiol 1993;40: 115 – 9. [13] Reynen K. Cardiac myxomas. N Engl J Med 1995;33:1610 – 7.