- Email: [email protected]
Primary left atrial myxosarcoma
564
CASE REPORT HARRIS ET AL PRIMARY LEFT ATRIAL MYXOSARCOMA
centers with some modifications [3, 41. 1Jntil recently, lung transplantation has not been considered an option for patients with ventilator-dependent respiratory failure by most transplant programs. Recent successful transplantations in 4 patients with short-term ventilator dependence have been reported by the Washington University Lung Transplantation Group [ 11. Respiratory failure developed in their patients while on the active waiting list or when graft failure occurred. They state that preoperative short-term ventilator dependence (up to 1 2 days) of patients without major complications or other organ dysfunction is not associated with increased morbidity or mortality [l]. When our patient was evaluated for transplantation there were no contraindications other than chronic ventilator dependence. To rescue our patient from life-long ventilator-dependence we initiated a program of daily bicycle training. Once a good level of physical conditioning was achieved we accepted her for transplantation. To provide excellent respiratory function foir this young, active individual we chose double-lung transplantation rather than the alternative single-lung transplantation. This case demonstrates that selected long-term ventilator-dependent patients may be considered good candidates for lung transplantation provided that they are well trained and no concurrent contraindications such as severe organ dysfunction or inanition exist.
Addendum Fifteen months postoperatively the patient is in good physical condition without any infection or rejection problems. At the moment she is on holidays in the Philippines. Lung function parameters measured in June 1993 had not changed significantly (vital capacity, 2.11 L [58% predicted]; forced expiratory volume in 1second, 1.9 L [59%predicted]; arterial oxygen tension, 85 mm
Hg).
Special acknowledgment is made to the members of the Vienna Lung Transplantation Group: Thoracic Surgery-Walter Klepetko, MD, Michael Grimm, MD, Adelheid End, MD, Gunther Laufer, MD; Pulmonary Medicine-Otto C. Burghuber, MD, Werner Schlick, MD; Anaesthesiology and Intensive CareMichael Hiesmayr, MD, Werner Moosbauer, MD, Peter Mares, MD; Pathology-Gerhard Dekan, MD; Physical TherapyGabriela Michalek, RPT; Transplant Coordinator-Thomas Inhauser, MD; Administration-Helga Koller.
References Low DE, Trulock EP, Kaiser LR, et al. Lung transplantation of ventilator-dependent patients. Chest 1992;101:%11. Klepetko W, Grimm M, Laufer G, et al. One and one-half year experience with unilateral and bilateral lung transplantation. J Cardiac Surg 1992;712&33. Marshall SE, Kramer MR, Lewiston NJ, Starneis VA, Theodore J. Selection and evaluation of recipients for heart-lung and lung transplantation. Chest 1990;98:1488-94. Cooper JD. The evaluation of technique and indications for lung transplantation. Ann Surg 1990;212:249-56.
Ann Thorac Surg 1993;56:5646
Primary Left Atrial Myxosarcoma Gary J. Harris, MD, Fermin 0. Tio, MD, and Frederick L. Grover, MD Division of Cardiothoracic Surgery and Department of Pathology, The University of Texas Health Science Center at San Antonio, San Antonio, Texas
A patient in whom rapidly progressive congestive heart failure developed due to the presence of a left atrial myxosarcoma is described. The histologic picture is consistent with the hypothesis that cardiac myxoid tumors are neoplasms derived from undifferentiated cells found in the endocardium. Unfortunately, most cardiac malignancies are detected too late for curative resection, and palliation remains the mainstay of therapy. (Ann Thorac Surg 1993;56:5646)
P
rimaiy neoplasms of the heart are rare. Autopsy series report a combined incidences of 0.0017% to 0.1% [l, 21 for both benign and malignant primary cardiac tumors. Malignant tumors, the majority of which are sarcomas, represent up to 25% of the cardiac neoplasms. Therefore, the incidence of primary cardiac malignancies is between 0.0004% and 0.025%. Patients with cardiac neoplasms may present with a spectrum of cardiovascular symptoms; however, because the symptoms are often vague, a high index of suspicion is required to make the diagnosis. Fortunately, echocardiography can provide the diagnosis as well as information regarding preoperative cardiac function. We present the case of a patient with progressive congestive heart failure caused by a left atrial myxosarcoma. The clinical course and pathologic findings are described in an attempt to obtain a better understanding of this rare neoplasm. A 26-year-old white woman with a 3-week history of ventricular tachycardia resistant to digoxin and quinidine therapy underwent transthoracic echocardiography after pulmonary edema developed. Echocardiography revealed a large, pedunculated, left atrial tumor extending through the mitral orifice. The patient was in moderate respiratory distress. The blood pressure was 132/84 mm Hg; pulse, 112 beats/min; and respirations, 24/min. Significant physical findings included jugular venous distention to the angle of the jaw, bilateral rales, and trace pretibial edema. Cardiac examination revealed an early decrescendo, diastolic murmur, and a left third heart sound. The electrocardiogram revealed right axis deviation; no Accepted for publication Nov 19, 1992. No reprints are available. Address correspondence to Dr Hams, Surgical Service (112), Veterans Administration Medical Center, 800 Zom Ave, Louisville, KY 40206-1499.
CASE REPORT HARRIS ET AL PRIMARY LEFT ATRIAL MYXOSARCOMA
Ann Thorac Surg 1993;56:564-6
Fig 1 . Photomicrograph of the solid area of the atrial mass showing prominent pleomorphism and hyperchromicity of tumor cells. Clear cytoplasm with distinct cytoplasmic membranes appearing as perinuclear halo (arrow) is seen in many of the cells. (Hematoxylin and eosin, X280 before 51 % reduction.)
acute changes were noted. Chest roentgenogram revealed increased pulmonary vascular markings and mild cardiomegaly. Admission laboratory studies were normal. Cardiac catheterization revealed a right atrial pressure of 12 mm Hg, right ventricular pressures of 81/20 mm Hg, pulmonary artery pressures of 76/46 mm Hg, and a pulmonary artery wedge pressure of 40 mm Hg. During the procedure she became bradycardic, requiring pacemaker insertion. Arteriography was not performed, and she was taken to the operating room for exploration through a median sternotomy. A 3 x 3.5-cm pedunculated tumor was found in the left atrium. A second tumor mass measuring approximately 1.5 x 1.5 cm arose from the commissure along the lateral aspect of the posterior leaflet of the mitral valve. This mass prolapsed across the annulus but did not enter the ventricle. A third tumor mass arising from the lateral free wall of the left atrium was incompletely excised from the area of the right inferior pulmonary vein. A fourth mass (2 x 2 cm), which was incompletely excised, appeared to be organized into the wall of the left superior pulmonary vein, causing complete occlusion. There were 10 smaller tumor implants along the lateral and posterior free walls of the atrium. Because of the extent of tumor spread, residual tumor remained at the end of the operation. The left ventricle, right atrium, and right ventricle were examined and found to be free of tumor. The pathologic diagnosis was myxosarcoma. The mitral valve commissure to commissure distance was approximately 1.8 cm; however, a commissurotomy was not performed because it was thought that severe insufficiency would result. The valve was not replaced due to the high concentration of tumor in the posterior aspect of the annulus. The postoperative course was uneventful. Because of the irregular intracardiac surface caused by residual tumor, anticoagulation was initiated.
565
One month later congestive heart failure developed; echocardiography revealed recurrence of the left atrial mass. Multiple treatment modalities, including intracavitary irradiation, external irradiation, chemotherapy, and heart-lung transplantation were considered; it was thought that none would alter the prognosis. She died of progressive disease 2 months after the diagnosis had been made. Permanent sections of the tumor showed necrosis with cellular areas showing pleomorphism and high mitotic activity-changes characteristic of malignant myxosarcoma. The areas without necrosis showed greater cellularity as well as pleomorphic and hyperchromatic cells containing a moderate number of mitotic figures (4 to 5 per high power field). These cells had moderately clumped chromatin, irregular nuclear membranes, and medium-sized nucleoli (Fig 1).Special stains revealed that the myxoid areas were composed of acid mucopolysaccharide. These areas also stained positive with the Alcian blue and colloidal iron stains. Immunoperoxidase stains showed abundant smooth muscle actin and vimentin in the cytoplasm of the tumor cells. They were negative for desmin, factor VIII-related antigen, and cytokeratin (AEUAE3). Electron microscopy showed evidence of smooth muscle differentiation characterized by cytoplasmic filaments with associated dense bodies (Fig 2), thin basement membrane-like material adjacent to the cytoplasmic membrane, occasional pinocytic vesicles, and mitochondria1 aggregates with closely opposed rough endoplasmic reticulum. The stroma contained fine stippled and fibrillar material characteristic of mucopolysaccharide as well as a few collagen fibers.
Comment The rarity of this tumor is underscored by the fact that Whorton’s review [3] in 1949 identified only six myxosar-
Fig 2. Electron micrograph of tumor cells shows fine cytoplasmic filaments associated with dense bodies farrow). The swollen mitochondria are closely associated with the elongated, dark, rough endoplasmic reticulum. (X22,OOO before 51 % reduction.)
566
CASE REPORT JONES ET AL LOW-HEPARIN BYPASS
comas reported up to that time. Since then several institutions have reported their experience with primary malignancies of the heart, further underscoring the rarity of this tumor. A review of 35 patients with primary cardiac tumors presenting over a 6-year period at ithe Mayo Clinic revealed only one atrial myxosarcoma [4]. Only three primary intracardiac sarcomas have been identified at the Texas Heart Institute [ 5 ] .Furthermore, a review from the Armed Forces Institute of Pathology identified only 3 patients with primary cardiac myxosarconna [6]. The histologic findings in the presented case are consistent with the generally accepted belief that cardiac myxoid tumors are true neoplasms derived from undifferentiated or embryonal rests found in the endocardium [7, 81. The pathologic material shows variable areas of dedifferentiation as well as differentiated areas that resemble benign atrial myxoma [9]. The cells that are clearly malignant show areas of pleomorphism and hyperchromicity with high mitotic activity. The immunocytochemical and ultrastructural findings indicate a tendency toward smooth muscle differentiation although undifferentiated cells, best classified as primitive mesenchymal cells, were present. These most likely represent the equivalent of the myxoma cell in the benign lesion. Previous ultrastructural studies have demonstrated evidence of :smooth muscle differentiation in the benign counterpart of this tumor [lo]. The undifferentiated cells have the capacity to differentiate into mesenchymal cell lines including endothelial cells, fibroblasts, myofibroblasts, smooth muscle cells, and chondroid cells, suggesting that other histologic variants of atrial sarcoma may be derived from the same group of undifferentiated cells [ll-131. These tumors may have originated from the same embryonal cell as the myxoma cell and, after differentiation, assumed the histologic picture of fibromyxosarcoma, malignant fibrous histiocytoma, or even chondrosarcoma. In conclusion, confirmation of a primary cardiac neoplasm is relatively easy if the clinician has a high degree of suspicion. Median sternotomy is indicated for tissue diagnosis as well as curative resection of benign lesions and debulking of malignant neoplasms. Although malignancy most likely develops from mesenchymal cells, cardiac malignancies are difficult to treat with any modality (operation, chemotherapy, radiation therapy, transplantation) because the tumor has usually undergone extensive spread by the time the diagnosis is made. Palliation remains the mainstay of therapy.
References 1. Straus R, Merliss R. Primary tumor of the heart. Arch Pathol 1945;39:74-8. 2. Prichard RW. Tumors of the heart. Arch Pathol 1951;51: 98-128. 3. Whorton CM. Primary malignant tumors of the heart. Cancer 1949;2:24560. 4. Fyke FE 111, Seward JB, Edwards WD, et al. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography. J Am Coll Cardiol 1985;5:1465-73. 5. Klima T, Milam JD, Bossart MI, Cooley DA. Rare primary sarcomas of the heart. Arch Pathol Lab Med 1986;110:1155-9.
Ann Thorac Surg 1993;56566-8
6 . Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387-95. 7. Lie JT. The identity and histogenesis of cardiac myxomas: a controversy put to rest. Arch Pathol Lab Med 1989;113:724-6. 8. Johansson L. Histogenesis of cardiac myxomas: an immunohistochemical study of 19 cases, including one with glandular structures, and review of the literature. Arch Pathol Lab Med 1989;113:73541. 9. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:21940. 10. Feldman PS, Horvath E, Kovacs K. An ultrastructural study of seven cardiac myxomas. Cancer 1977;40:2216-32. 11. Ravid JM, Sachs J. Tumors of the heart with a report of a primary fibromyxosarcoma of the left auricle and the pulmonary vein, associated with multiple tumors of the mesentery and alimentary tract. Am Heart J 1943;26:385-97. 12. Gabelman C, Al-Sadir J, Lamberti J, et al. Surgical treatment of recurrent primary malignant tumor of the left atrium. J Thorac Cardiovasc Surg 1979;77914-21. 13. Hammond GL, Strong WW, Cohen LS, et al. Chondrosarcoma simulating malignant atrial myxoma. J Thorac Cardiovasc Surg 1976;72:575-80.
Use of Heparin-Coated Cardiopulmonary Bypass David R. Jones, MD, Ronald C. Hill, MD, Michael J. Hollingsed, CCP, Edward Stullken, MD, Geoffrey M. Graeber, MD, Robert A. Gustafson, MD, and Gordon F. Murray, MD Department of Surgery, West Virginia University School of Medicine, Morgantown, West Virginia
A 49-year-old man with unstable angina and a history of severe anaphylaxis to seafood and intravenous iodine needed myocardial revascularization.Because of concern of an intraoperative protamine reaction, preoperative treatment was instituted with steroids and with HI and H, blockers. Revascularization was accomplished using a heparin-coated cardiopulmonary bypass circuit. Complement activation and postoperative bleeding were minimal. Heparin-coated cardiopulmonary bypass is a safe, effective technique of bypass in select patients. (Ann Thoruc Surg 1993;56:566-8) A 48-year-old man was referred for coronary artery bypass grafting for unstable angina. The patient had a prior cardiac history of an inferior wall myocardial infarction. Cardiac catheterization at that time showed no flow significant lesions, but the patient did have an anaphylactic reaction to the intravenous iodine. The patient remained asymptomatic with medical management until recently, when worsening angina developed. He was premedicated with steroids and with HI and H, blockers and underwent repeat cardiac catheterization with no anaphylaxis. The coronary anatomy demonstrated normal left Accepted for publication Nov 3, 1992 Address reprint requests to Dr Hill, Department of Surgery, West Virginia University, Health Sciences Center North, Morgantown, WV 26506.
CASE REPORT HARRIS ET AL PRIMARY LEFT ATRIAL MYXOSARCOMA
centers with some modifications [3, 41. 1Jntil recently, lung transplantation has not been considered an option for patients with ventilator-dependent respiratory failure by most transplant programs. Recent successful transplantations in 4 patients with short-term ventilator dependence have been reported by the Washington University Lung Transplantation Group [ 11. Respiratory failure developed in their patients while on the active waiting list or when graft failure occurred. They state that preoperative short-term ventilator dependence (up to 1 2 days) of patients without major complications or other organ dysfunction is not associated with increased morbidity or mortality [l]. When our patient was evaluated for transplantation there were no contraindications other than chronic ventilator dependence. To rescue our patient from life-long ventilator-dependence we initiated a program of daily bicycle training. Once a good level of physical conditioning was achieved we accepted her for transplantation. To provide excellent respiratory function foir this young, active individual we chose double-lung transplantation rather than the alternative single-lung transplantation. This case demonstrates that selected long-term ventilator-dependent patients may be considered good candidates for lung transplantation provided that they are well trained and no concurrent contraindications such as severe organ dysfunction or inanition exist.
Addendum Fifteen months postoperatively the patient is in good physical condition without any infection or rejection problems. At the moment she is on holidays in the Philippines. Lung function parameters measured in June 1993 had not changed significantly (vital capacity, 2.11 L [58% predicted]; forced expiratory volume in 1second, 1.9 L [59%predicted]; arterial oxygen tension, 85 mm
Hg).
Special acknowledgment is made to the members of the Vienna Lung Transplantation Group: Thoracic Surgery-Walter Klepetko, MD, Michael Grimm, MD, Adelheid End, MD, Gunther Laufer, MD; Pulmonary Medicine-Otto C. Burghuber, MD, Werner Schlick, MD; Anaesthesiology and Intensive CareMichael Hiesmayr, MD, Werner Moosbauer, MD, Peter Mares, MD; Pathology-Gerhard Dekan, MD; Physical TherapyGabriela Michalek, RPT; Transplant Coordinator-Thomas Inhauser, MD; Administration-Helga Koller.
References Low DE, Trulock EP, Kaiser LR, et al. Lung transplantation of ventilator-dependent patients. Chest 1992;101:%11. Klepetko W, Grimm M, Laufer G, et al. One and one-half year experience with unilateral and bilateral lung transplantation. J Cardiac Surg 1992;712&33. Marshall SE, Kramer MR, Lewiston NJ, Starneis VA, Theodore J. Selection and evaluation of recipients for heart-lung and lung transplantation. Chest 1990;98:1488-94. Cooper JD. The evaluation of technique and indications for lung transplantation. Ann Surg 1990;212:249-56.
Ann Thorac Surg 1993;56:5646
Primary Left Atrial Myxosarcoma Gary J. Harris, MD, Fermin 0. Tio, MD, and Frederick L. Grover, MD Division of Cardiothoracic Surgery and Department of Pathology, The University of Texas Health Science Center at San Antonio, San Antonio, Texas
A patient in whom rapidly progressive congestive heart failure developed due to the presence of a left atrial myxosarcoma is described. The histologic picture is consistent with the hypothesis that cardiac myxoid tumors are neoplasms derived from undifferentiated cells found in the endocardium. Unfortunately, most cardiac malignancies are detected too late for curative resection, and palliation remains the mainstay of therapy. (Ann Thorac Surg 1993;56:5646)
P
rimaiy neoplasms of the heart are rare. Autopsy series report a combined incidences of 0.0017% to 0.1% [l, 21 for both benign and malignant primary cardiac tumors. Malignant tumors, the majority of which are sarcomas, represent up to 25% of the cardiac neoplasms. Therefore, the incidence of primary cardiac malignancies is between 0.0004% and 0.025%. Patients with cardiac neoplasms may present with a spectrum of cardiovascular symptoms; however, because the symptoms are often vague, a high index of suspicion is required to make the diagnosis. Fortunately, echocardiography can provide the diagnosis as well as information regarding preoperative cardiac function. We present the case of a patient with progressive congestive heart failure caused by a left atrial myxosarcoma. The clinical course and pathologic findings are described in an attempt to obtain a better understanding of this rare neoplasm. A 26-year-old white woman with a 3-week history of ventricular tachycardia resistant to digoxin and quinidine therapy underwent transthoracic echocardiography after pulmonary edema developed. Echocardiography revealed a large, pedunculated, left atrial tumor extending through the mitral orifice. The patient was in moderate respiratory distress. The blood pressure was 132/84 mm Hg; pulse, 112 beats/min; and respirations, 24/min. Significant physical findings included jugular venous distention to the angle of the jaw, bilateral rales, and trace pretibial edema. Cardiac examination revealed an early decrescendo, diastolic murmur, and a left third heart sound. The electrocardiogram revealed right axis deviation; no Accepted for publication Nov 19, 1992. No reprints are available. Address correspondence to Dr Hams, Surgical Service (112), Veterans Administration Medical Center, 800 Zom Ave, Louisville, KY 40206-1499.
CASE REPORT HARRIS ET AL PRIMARY LEFT ATRIAL MYXOSARCOMA
Ann Thorac Surg 1993;56:564-6
Fig 1 . Photomicrograph of the solid area of the atrial mass showing prominent pleomorphism and hyperchromicity of tumor cells. Clear cytoplasm with distinct cytoplasmic membranes appearing as perinuclear halo (arrow) is seen in many of the cells. (Hematoxylin and eosin, X280 before 51 % reduction.)
acute changes were noted. Chest roentgenogram revealed increased pulmonary vascular markings and mild cardiomegaly. Admission laboratory studies were normal. Cardiac catheterization revealed a right atrial pressure of 12 mm Hg, right ventricular pressures of 81/20 mm Hg, pulmonary artery pressures of 76/46 mm Hg, and a pulmonary artery wedge pressure of 40 mm Hg. During the procedure she became bradycardic, requiring pacemaker insertion. Arteriography was not performed, and she was taken to the operating room for exploration through a median sternotomy. A 3 x 3.5-cm pedunculated tumor was found in the left atrium. A second tumor mass measuring approximately 1.5 x 1.5 cm arose from the commissure along the lateral aspect of the posterior leaflet of the mitral valve. This mass prolapsed across the annulus but did not enter the ventricle. A third tumor mass arising from the lateral free wall of the left atrium was incompletely excised from the area of the right inferior pulmonary vein. A fourth mass (2 x 2 cm), which was incompletely excised, appeared to be organized into the wall of the left superior pulmonary vein, causing complete occlusion. There were 10 smaller tumor implants along the lateral and posterior free walls of the atrium. Because of the extent of tumor spread, residual tumor remained at the end of the operation. The left ventricle, right atrium, and right ventricle were examined and found to be free of tumor. The pathologic diagnosis was myxosarcoma. The mitral valve commissure to commissure distance was approximately 1.8 cm; however, a commissurotomy was not performed because it was thought that severe insufficiency would result. The valve was not replaced due to the high concentration of tumor in the posterior aspect of the annulus. The postoperative course was uneventful. Because of the irregular intracardiac surface caused by residual tumor, anticoagulation was initiated.
565
One month later congestive heart failure developed; echocardiography revealed recurrence of the left atrial mass. Multiple treatment modalities, including intracavitary irradiation, external irradiation, chemotherapy, and heart-lung transplantation were considered; it was thought that none would alter the prognosis. She died of progressive disease 2 months after the diagnosis had been made. Permanent sections of the tumor showed necrosis with cellular areas showing pleomorphism and high mitotic activity-changes characteristic of malignant myxosarcoma. The areas without necrosis showed greater cellularity as well as pleomorphic and hyperchromatic cells containing a moderate number of mitotic figures (4 to 5 per high power field). These cells had moderately clumped chromatin, irregular nuclear membranes, and medium-sized nucleoli (Fig 1).Special stains revealed that the myxoid areas were composed of acid mucopolysaccharide. These areas also stained positive with the Alcian blue and colloidal iron stains. Immunoperoxidase stains showed abundant smooth muscle actin and vimentin in the cytoplasm of the tumor cells. They were negative for desmin, factor VIII-related antigen, and cytokeratin (AEUAE3). Electron microscopy showed evidence of smooth muscle differentiation characterized by cytoplasmic filaments with associated dense bodies (Fig 2), thin basement membrane-like material adjacent to the cytoplasmic membrane, occasional pinocytic vesicles, and mitochondria1 aggregates with closely opposed rough endoplasmic reticulum. The stroma contained fine stippled and fibrillar material characteristic of mucopolysaccharide as well as a few collagen fibers.
Comment The rarity of this tumor is underscored by the fact that Whorton’s review [3] in 1949 identified only six myxosar-
Fig 2. Electron micrograph of tumor cells shows fine cytoplasmic filaments associated with dense bodies farrow). The swollen mitochondria are closely associated with the elongated, dark, rough endoplasmic reticulum. (X22,OOO before 51 % reduction.)
566
CASE REPORT JONES ET AL LOW-HEPARIN BYPASS
comas reported up to that time. Since then several institutions have reported their experience with primary malignancies of the heart, further underscoring the rarity of this tumor. A review of 35 patients with primary cardiac tumors presenting over a 6-year period at ithe Mayo Clinic revealed only one atrial myxosarcoma [4]. Only three primary intracardiac sarcomas have been identified at the Texas Heart Institute [ 5 ] .Furthermore, a review from the Armed Forces Institute of Pathology identified only 3 patients with primary cardiac myxosarconna [6]. The histologic findings in the presented case are consistent with the generally accepted belief that cardiac myxoid tumors are true neoplasms derived from undifferentiated or embryonal rests found in the endocardium [7, 81. The pathologic material shows variable areas of dedifferentiation as well as differentiated areas that resemble benign atrial myxoma [9]. The cells that are clearly malignant show areas of pleomorphism and hyperchromicity with high mitotic activity. The immunocytochemical and ultrastructural findings indicate a tendency toward smooth muscle differentiation although undifferentiated cells, best classified as primitive mesenchymal cells, were present. These most likely represent the equivalent of the myxoma cell in the benign lesion. Previous ultrastructural studies have demonstrated evidence of :smooth muscle differentiation in the benign counterpart of this tumor [lo]. The undifferentiated cells have the capacity to differentiate into mesenchymal cell lines including endothelial cells, fibroblasts, myofibroblasts, smooth muscle cells, and chondroid cells, suggesting that other histologic variants of atrial sarcoma may be derived from the same group of undifferentiated cells [ll-131. These tumors may have originated from the same embryonal cell as the myxoma cell and, after differentiation, assumed the histologic picture of fibromyxosarcoma, malignant fibrous histiocytoma, or even chondrosarcoma. In conclusion, confirmation of a primary cardiac neoplasm is relatively easy if the clinician has a high degree of suspicion. Median sternotomy is indicated for tissue diagnosis as well as curative resection of benign lesions and debulking of malignant neoplasms. Although malignancy most likely develops from mesenchymal cells, cardiac malignancies are difficult to treat with any modality (operation, chemotherapy, radiation therapy, transplantation) because the tumor has usually undergone extensive spread by the time the diagnosis is made. Palliation remains the mainstay of therapy.
References 1. Straus R, Merliss R. Primary tumor of the heart. Arch Pathol 1945;39:74-8. 2. Prichard RW. Tumors of the heart. Arch Pathol 1951;51: 98-128. 3. Whorton CM. Primary malignant tumors of the heart. Cancer 1949;2:24560. 4. Fyke FE 111, Seward JB, Edwards WD, et al. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography. J Am Coll Cardiol 1985;5:1465-73. 5. Klima T, Milam JD, Bossart MI, Cooley DA. Rare primary sarcomas of the heart. Arch Pathol Lab Med 1986;110:1155-9.
Ann Thorac Surg 1993;56566-8
6 . Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387-95. 7. Lie JT. The identity and histogenesis of cardiac myxomas: a controversy put to rest. Arch Pathol Lab Med 1989;113:724-6. 8. Johansson L. Histogenesis of cardiac myxomas: an immunohistochemical study of 19 cases, including one with glandular structures, and review of the literature. Arch Pathol Lab Med 1989;113:73541. 9. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:21940. 10. Feldman PS, Horvath E, Kovacs K. An ultrastructural study of seven cardiac myxomas. Cancer 1977;40:2216-32. 11. Ravid JM, Sachs J. Tumors of the heart with a report of a primary fibromyxosarcoma of the left auricle and the pulmonary vein, associated with multiple tumors of the mesentery and alimentary tract. Am Heart J 1943;26:385-97. 12. Gabelman C, Al-Sadir J, Lamberti J, et al. Surgical treatment of recurrent primary malignant tumor of the left atrium. J Thorac Cardiovasc Surg 1979;77914-21. 13. Hammond GL, Strong WW, Cohen LS, et al. Chondrosarcoma simulating malignant atrial myxoma. J Thorac Cardiovasc Surg 1976;72:575-80.
Use of Heparin-Coated Cardiopulmonary Bypass David R. Jones, MD, Ronald C. Hill, MD, Michael J. Hollingsed, CCP, Edward Stullken, MD, Geoffrey M. Graeber, MD, Robert A. Gustafson, MD, and Gordon F. Murray, MD Department of Surgery, West Virginia University School of Medicine, Morgantown, West Virginia
A 49-year-old man with unstable angina and a history of severe anaphylaxis to seafood and intravenous iodine needed myocardial revascularization.Because of concern of an intraoperative protamine reaction, preoperative treatment was instituted with steroids and with HI and H, blockers. Revascularization was accomplished using a heparin-coated cardiopulmonary bypass circuit. Complement activation and postoperative bleeding were minimal. Heparin-coated cardiopulmonary bypass is a safe, effective technique of bypass in select patients. (Ann Thoruc Surg 1993;56:566-8) A 48-year-old man was referred for coronary artery bypass grafting for unstable angina. The patient had a prior cardiac history of an inferior wall myocardial infarction. Cardiac catheterization at that time showed no flow significant lesions, but the patient did have an anaphylactic reaction to the intravenous iodine. The patient remained asymptomatic with medical management until recently, when worsening angina developed. He was premedicated with steroids and with HI and H, blockers and underwent repeat cardiac catheterization with no anaphylaxis. The coronary anatomy demonstrated normal left Accepted for publication Nov 3, 1992 Address reprint requests to Dr Hill, Department of Surgery, West Virginia University, Health Sciences Center North, Morgantown, WV 26506.