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Primary undifferentiated sarcoma of the pericardium
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Primary Undifferentiated Sarcoma of the Pericardium Ammu Sivaraman,
MD, Jaya Deshpande, MD, and Pradeep Vaideeshwar, MD
Department of Pathology, Seth G. S. Medical College, Bombay, India
A 60-year-old woman was found to have a pericardial masson two-dimensional echocardiography and computerizedtomography.At autopsy,therewasa largetumor arising from the epicardial surface of the left ventricle. The tumor encircled the great arteries and invadedthe wall of the pulmonary artery. Microscopic examination showedfeaturesof an undifferentiated sarcoma.
Primary malignant neoplasms of the heart are extremely uncommon and are generally sarcomas. Most of them arise from the cardiac chambers, and only a few are pericardial in origin. Pericardial tumors present with features of effusion, constriction, or cardiac failure and can be diagnosed by echocardiography, computed tomography, and magnetic resonance imaging. We report the findings in a malignant pericardial tumor that was diagnosed by imaging techniques during life and identified histologically at postmortem examination.
Case Report A 60-year-old housewife was admitted to the hospital for anorexia, weight loss, and evening rise of temperature of three months’ duration. A chest x-ray taken a month earlier revealed a left pleural effusion, cardiomegaly, and a left paracardiac shadow. A diagnostic pleural tap was done that showed an exudate, but no malignant cells were seen. Following this, she was given steroids and antituberculous drugs, but there was no improvement. Physical examination revealed an emaciated woman (body weight 29 kg) with pallor and edema of both feet. The pulse rate was 96 bpm and blood pressure 90/60 mm Hg. Jugular venous pressure was not elevated. Heart sounds were muffled. Air entry was diminished in the left mid and lower zone. Routine hematological and biochemical investigations showed mild
Manuscript received August 5, 1994; accepted December 23, 1994. Address for reprints: Dr. A. Sivaraman, Department of Pathology, Seth G. S. Medical College, Bombay 400 012, India. Cardiovascular Pathology Vol. 4, No. 2, April-June 1995:147-148 o 1995 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
anemia, raised erythrocyte sedimentation rate, and hypoproteinemia. A pericardial mass and vegetations on the pulmonary valve were seen on two-dimensional echocardiography. Computerized tomography showed a pericardial mass suggestive of lymphoma. The condition of the patient deteriorated rapidly, and she died of cardiac failure. A limited autopsy was done.
Postmortem Findings Emaciation, pallor, and pedal edema were observed on external examination. There was no fluid in the serous cavities. The heart was markedly enlarged and weighed 920 gm. A mass measuring 15 X 12 X 11 cm was present on the anterolateral aspect of the left ventricle, just above the apex. It extended over the great arteries and was densely adherent to the pericardium. The cut surface showed a well-circumscribed, fleshy, yellow mass with areas of hemorrhage and cystic degeneration. It compressed the myocardium of the anterior wall of the left ventricle but appeared to be separated from it by an ill-defined capsule (Fig. 1). The mass surrounded the aorta and pulmonary artery and projected into the lumen of the pulmonary artery as polypoid masses.It also infiltrated the anterior wall of the left atrium but did not extend into the cavity. The left lung showed infarcts. There were no distant metastases. No primary tumor was found in the thorax or abdomen. Permission to examine the skull was not obtained. Microscopic sections from the tumor showed extensive necrosis with only small areas of viable tumor. The tumor cells were noncohesive, large, and round to elongated with abundant cytoplasm and large vesicular nuclei. Intracytoplas-
1054-8807/95/$9.50 SSDI 1054-8807(94)00064-X
14x
SIVARAMAN ET AL PARICARDIAL SARCOMA
Figure 1. Tumor (T) arising from the epicardium over the left ventricle (LV), encircling the aorta (A) and infiltrating the wall of the left atrium (LA).
mic glycogen, mucin, and cross-striations were not demonstrated. Immunohistochemistry stains for cytokeratin, desmin, myoglobin, carcinoembryonic antigen, S-100 protein, B 72.3 antigen, and Leu MI were negative. A diagnosis of undifferentiated sarcoma was made.
dium. Mesotheliomas generally cover the visceral and parieta1pericardium, encasing the heart. Rarely they are seen as localized lesions. They grow by contiguous spread, coating rather than infiltrating the underlying myocardium. It has been stressed that when a pericardial tumor invades the myocar. dium and forms an intracavitary mass. a diagnosis of meso.. thelioma is unlikely (3). The diagnosis on gross examination of the heart in this case was mesothelioma. The extensive lrrcal spread with little myocardial invasion, lack of an intracavitary mass, and absence of distant metastases supported this diagnosis. The unusual feature was the polypoid masses 01 tumor projecting into the lumen of the pulmonary artery lt would appear from the subsequent microscopic diagnosis 01 sarcoma that growth into the iumen-arterial or cardiac chamber-speaks in favor of sarcoma rather than mei;othc’ lioma. Although the use of immunohistochemistry and electron microscopy has made it possible to classify sarcomas mom precisely, a fairly large group still remains unclassified. It has been observed that neither the histological type nor the dcgree of differentiation affects the outcome (4). Necrosis, which was a prominent feature in our tumor. is indicative of a poor prognosis. Improvements in imaging modalities have enhanced the detection of all cardiac tumors and the early diagnosis ofmalignant lesions. Total or palliative resection of sarcoma has been achieved by refinements in surgical technique (56). However, the long-term survival is poor because these are extremely aggressive tumors that can be controlled only by effective adjuvant therapy. The authors gratefully acknowledge the valuable opinion of A. P. Burke and R. Virmani (AFIP, Washington, DC.) and their assistance in providing the immunochcmical studies.
Discussion The reported autopsy incidence of cardiac tumors is 0.0017% and 0.03 % (1,2). Approximately 25 % of them are malignant (3). Sarcomas of varying histological types constitute 78.4% of the malignant tumors. In descending order of frequency, they arise from the cardiac chambers, pericardium, and valves (4). Primary malignancies originating in the pericardium are mesothelioma or one of the following types of sarcomas: angiosarcoma, undifferentiated sarcoma, neurofibrosarcoma, leiomyosarcoma, and synovial sarcoma (4). The other cardiac sarcomas- rhabdomyosarcoma, osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, myxosarcoma, and lymphoma - arise murally and secondarily involve the pericar-
References 1 Straus R, Merliss R. Primary tumor of the heart Arch Pathol 1945. 39:74-78. 2. Benjamin HG. Primary fibromyxoma of the heart. Arch Pathol 1939: 27:950. 3. McAllister HA, Jr. Primary tumours of the heart and pericardium. in. Sommers SC and Rosen PP. eds. Pathology Annual. East Norwalk, Cl Appleton-Century-Croft, 1979:325-X5. 4. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Can cer 1992;69:387 -395. 5. Murphy MC, Sweeney MS. Putman JB, Jr.. et al. Surgical treatment of cardiac tumours. Ann Thorac Surg 1990;49:612-617. 6. Dein JR, Frist WH, Stinson EB, et al. Primary cardiac neoplasma: cariy and late results of surgical treatment in 42 patients. J Thorac Cardiovasc Surg 1987;93:502-511.
Primary Undifferentiated Sarcoma of the Pericardium Ammu Sivaraman,
MD, Jaya Deshpande, MD, and Pradeep Vaideeshwar, MD
Department of Pathology, Seth G. S. Medical College, Bombay, India
A 60-year-old woman was found to have a pericardial masson two-dimensional echocardiography and computerizedtomography.At autopsy,therewasa largetumor arising from the epicardial surface of the left ventricle. The tumor encircled the great arteries and invadedthe wall of the pulmonary artery. Microscopic examination showedfeaturesof an undifferentiated sarcoma.
Primary malignant neoplasms of the heart are extremely uncommon and are generally sarcomas. Most of them arise from the cardiac chambers, and only a few are pericardial in origin. Pericardial tumors present with features of effusion, constriction, or cardiac failure and can be diagnosed by echocardiography, computed tomography, and magnetic resonance imaging. We report the findings in a malignant pericardial tumor that was diagnosed by imaging techniques during life and identified histologically at postmortem examination.
Case Report A 60-year-old housewife was admitted to the hospital for anorexia, weight loss, and evening rise of temperature of three months’ duration. A chest x-ray taken a month earlier revealed a left pleural effusion, cardiomegaly, and a left paracardiac shadow. A diagnostic pleural tap was done that showed an exudate, but no malignant cells were seen. Following this, she was given steroids and antituberculous drugs, but there was no improvement. Physical examination revealed an emaciated woman (body weight 29 kg) with pallor and edema of both feet. The pulse rate was 96 bpm and blood pressure 90/60 mm Hg. Jugular venous pressure was not elevated. Heart sounds were muffled. Air entry was diminished in the left mid and lower zone. Routine hematological and biochemical investigations showed mild
Manuscript received August 5, 1994; accepted December 23, 1994. Address for reprints: Dr. A. Sivaraman, Department of Pathology, Seth G. S. Medical College, Bombay 400 012, India. Cardiovascular Pathology Vol. 4, No. 2, April-June 1995:147-148 o 1995 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
anemia, raised erythrocyte sedimentation rate, and hypoproteinemia. A pericardial mass and vegetations on the pulmonary valve were seen on two-dimensional echocardiography. Computerized tomography showed a pericardial mass suggestive of lymphoma. The condition of the patient deteriorated rapidly, and she died of cardiac failure. A limited autopsy was done.
Postmortem Findings Emaciation, pallor, and pedal edema were observed on external examination. There was no fluid in the serous cavities. The heart was markedly enlarged and weighed 920 gm. A mass measuring 15 X 12 X 11 cm was present on the anterolateral aspect of the left ventricle, just above the apex. It extended over the great arteries and was densely adherent to the pericardium. The cut surface showed a well-circumscribed, fleshy, yellow mass with areas of hemorrhage and cystic degeneration. It compressed the myocardium of the anterior wall of the left ventricle but appeared to be separated from it by an ill-defined capsule (Fig. 1). The mass surrounded the aorta and pulmonary artery and projected into the lumen of the pulmonary artery as polypoid masses.It also infiltrated the anterior wall of the left atrium but did not extend into the cavity. The left lung showed infarcts. There were no distant metastases. No primary tumor was found in the thorax or abdomen. Permission to examine the skull was not obtained. Microscopic sections from the tumor showed extensive necrosis with only small areas of viable tumor. The tumor cells were noncohesive, large, and round to elongated with abundant cytoplasm and large vesicular nuclei. Intracytoplas-
1054-8807/95/$9.50 SSDI 1054-8807(94)00064-X
14x
SIVARAMAN ET AL PARICARDIAL SARCOMA
Figure 1. Tumor (T) arising from the epicardium over the left ventricle (LV), encircling the aorta (A) and infiltrating the wall of the left atrium (LA).
mic glycogen, mucin, and cross-striations were not demonstrated. Immunohistochemistry stains for cytokeratin, desmin, myoglobin, carcinoembryonic antigen, S-100 protein, B 72.3 antigen, and Leu MI were negative. A diagnosis of undifferentiated sarcoma was made.
dium. Mesotheliomas generally cover the visceral and parieta1pericardium, encasing the heart. Rarely they are seen as localized lesions. They grow by contiguous spread, coating rather than infiltrating the underlying myocardium. It has been stressed that when a pericardial tumor invades the myocar. dium and forms an intracavitary mass. a diagnosis of meso.. thelioma is unlikely (3). The diagnosis on gross examination of the heart in this case was mesothelioma. The extensive lrrcal spread with little myocardial invasion, lack of an intracavitary mass, and absence of distant metastases supported this diagnosis. The unusual feature was the polypoid masses 01 tumor projecting into the lumen of the pulmonary artery lt would appear from the subsequent microscopic diagnosis 01 sarcoma that growth into the iumen-arterial or cardiac chamber-speaks in favor of sarcoma rather than mei;othc’ lioma. Although the use of immunohistochemistry and electron microscopy has made it possible to classify sarcomas mom precisely, a fairly large group still remains unclassified. It has been observed that neither the histological type nor the dcgree of differentiation affects the outcome (4). Necrosis, which was a prominent feature in our tumor. is indicative of a poor prognosis. Improvements in imaging modalities have enhanced the detection of all cardiac tumors and the early diagnosis ofmalignant lesions. Total or palliative resection of sarcoma has been achieved by refinements in surgical technique (56). However, the long-term survival is poor because these are extremely aggressive tumors that can be controlled only by effective adjuvant therapy. The authors gratefully acknowledge the valuable opinion of A. P. Burke and R. Virmani (AFIP, Washington, DC.) and their assistance in providing the immunochcmical studies.
Discussion The reported autopsy incidence of cardiac tumors is 0.0017% and 0.03 % (1,2). Approximately 25 % of them are malignant (3). Sarcomas of varying histological types constitute 78.4% of the malignant tumors. In descending order of frequency, they arise from the cardiac chambers, pericardium, and valves (4). Primary malignancies originating in the pericardium are mesothelioma or one of the following types of sarcomas: angiosarcoma, undifferentiated sarcoma, neurofibrosarcoma, leiomyosarcoma, and synovial sarcoma (4). The other cardiac sarcomas- rhabdomyosarcoma, osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, myxosarcoma, and lymphoma - arise murally and secondarily involve the pericar-
References 1 Straus R, Merliss R. Primary tumor of the heart Arch Pathol 1945. 39:74-78. 2. Benjamin HG. Primary fibromyxoma of the heart. Arch Pathol 1939: 27:950. 3. McAllister HA, Jr. Primary tumours of the heart and pericardium. in. Sommers SC and Rosen PP. eds. Pathology Annual. East Norwalk, Cl Appleton-Century-Croft, 1979:325-X5. 4. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Can cer 1992;69:387 -395. 5. Murphy MC, Sweeney MS. Putman JB, Jr.. et al. Surgical treatment of cardiac tumours. Ann Thorac Surg 1990;49:612-617. 6. Dein JR, Frist WH, Stinson EB, et al. Primary cardiac neoplasma: cariy and late results of surgical treatment in 42 patients. J Thorac Cardiovasc Surg 1987;93:502-511.