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Ann Thorac Surg 1997;63:1160-1161
© 1997 The Society of Thoracic Surgeons

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Case Report

Primary Cardiac Angiosarcoma: 53 Months' Survival After Multidisciplinary Therapy

Division of Cardiovascular Surgery and Departments of Pediatrics, and Pathology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan

Accepted for publication November 6, 1996.

	Abstract

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An 8-year-old girl underwent surgical excision of a tumor arising from the right atrium immediately after admission. Because histologic and immunohistochemical examination confirmed the diagnosis of angiosarcoma, adjunctive multidisciplinary therapy was administered for the following 2 years. She is leading a normal school life 53 months after the operation. Therapeutic strategy for this highly malignant cardiac tumor is discussed.

	Introduction

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Primary cardiac angiosarcoma is a rare malignant cardiac tumor and is known to carry a high mortality rate due to its rapid local relapse and high incidence of systemic metastasis. Because of nonspecific clinical presentation, most of the diagnoses in previous reports were made either at autopsy or shortly before the patients' deaths [1]. Recent advances of diagnostic methods have allowed antemortem diagnosis to be made and the patients to be treated with a therapeutic strategy including surgical excision of the tumor [2]. Early death by invariably rapid local relapse or systemic metastasis, however, is the rule, and only a few long-term survivors have been reported, thus hindering establishment of an optimal therapeutic strategy.

An 8-year-old girl was admitted to our hospital with complaints of fever, nonproductive cough, and orthopnea. On physical examination she was severely dyspneic at rest. The liver was palpable 3 cm below the right costal margin but without evidence of dependent edema. Chest roentgenography showed a markedly enlarged cardiac silhouette, with a cardiothoracic ratio of 0.75. Electrocardiography revealed regular sinus rhythm. Two-dimensional echocardiography disclosed a space-occupying tumor in the right atrium and a large amount of fluid in the pericardial sac. Computed tomography clearly demonstrated an inhomongeneously enhanced mass, 8 cm in diameter, in the right side of the cardiac silhouette. Whole-body gallium scintigraphy showed no evidence of systemic metastasis. The patient underwent pericardiocentesis and bloody fluid was aspirated, in which no atypical cell was found. Left ventriculography revealed hypokinesis of the anterolateral wall with an ejection fraction of 0.42. Selective right coronary angiography demonstrated tumor stain in the right atrial region with feeding arteries arising from segment 2 and a cluster of small arteries near segment 4. In contrast to the cytologic findings of the pericardial fluid, all these findings suggested malignancy of the tumor, thus prompting us to proceed to surgical excision on the 6th day of admission.

When the pericardium was opened, the tumor extended from the right atrial free wall to the right anterior pericardium. With the aid of cardiopulmonary bypass and moderate hypothermia, we resected the tumor mass including the tightly adhered pericardium and surrounding atrial free wall, and reconstructed the atrial wall defect with a bovine pericardial patch. Excision was inevitably incomplete near the sinoatrial node and right atrioventricular groove. Postoperative recovery was uneventful, and the electrocardiogram returned to regular sinus rhythm after a brief period of second-degree atrioventricular block.

The excised tumor was round, measured 90 x 50 x 40 mm, and weighed 115 g. The cut surface was dark red and revealed variously sized foci of hemorrhage and necrosis (Fig 1). Histologic examination showed abnormal proliferation of atypical plump endothelial cells that formed sinusoidal arrangements of various sizes. In some sites the stroma was scanty and dominated by spindle-shaped cells, and in the other sites solid sheets of anaplastic pleomorphic cells, with numerous mitotic figures, were observed. Immunohistochemical stainings of the cells were positive for factor VIII and negative for myoglobin and desmin, which reconfirmed the histologic diagnosis of angiosarcoma. Systemic immunochemotherapy was initiated immediately after the operation. The therapeutic regimen was a combination of chemotherapy that comprised dacarbazine, THP-doxorubicin, and ifosfamide for a week and immunotherapy with interleukin II for the following 2 weeks (Table 1). This regimen was repeated monthly. Irradiation therapy was also added, with a total dose of 50 Gy. At completion of nine cycles of immunochemotherapy, ie, when the accumulated dose of THP-doxorubicin reached 390 mg/m², however, the patient became intolerant to the therapy due to impaired cardiac function, with an ejection fraction of 0.61 and appearance of grade 2 tricuspid regurgitation. After discontinuation of THP-doxorubicin administration cardiac function reverted to normal, with grade 1 tricuspid regurgitation, thereby allowing two more cycles of immunochemotherapy excluding THP-doxorubicin. At completion of 11 cycles of immunochemotherapy and 1 year after the operation, the patient was discharged and received seven more cycles every other month for the following 1-year period. Repeat cardiac catheterization, performed a year after completion of the entire therapeutic regimen, revealed normal pressures of the four cardiac chambers and normal ventricular function. The patient is now leading a normal school life 53 months after the operation, without any evidence of local relapse or systemic metastasis.

View larger version (175K): [in this window] [in a new window]   Fig 1. . The excised tumor was round, measured 90 x 50 x 40 mm, and weighed 115 g. The cut surface was dark red and revealed variously sized foci of hemorrhage and necrosis.

	Comment

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	Footnotes

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Address reprint requests to Dr Nakamichi, Division of Cardiovascular Surgery, Department of Surgery, Keio University, 35 Shinanomachi, Shinjuku, Tokyo 160, Japan.

	References

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1. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer 1986;57:852–9.[Medline]
2. Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992;103:655–64.[Abstract]
3. Baay P, Karwande SV, Kushner JP, Olsen S, Renlund DG. Successful treatment of a cardiac angiosarcoma with combined modality therapy. J Heart Lung Transplant 1994;13:923–5.[Medline]
4. Stein M, Deitling F, Cantor A, Perner Y, Bezwoda W. Primary cardiac angiosarcoma: a case report and review of therapeutic options. Med Pediatr Oncol 1994;23:149–52.[Medline]
5. Sorlie D, Myhre ESP, Stalsberg H. Angiosarcoma of the heart: unusual presentation and survival after treatment. Br Heart J 1984;51:94–7.[Abstract/Free Full Text]
6. Percy RF, Perryman RA, Amornman R, et al. Prolonged survival in a patient with primary angiosarcoma of the heart. Am Heart J 1987;113:1228–30.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Takaaki Suzuki Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Nakamichi, T. Articles by Morikawa, Y. Search for Related Content PubMed PubMed Citation Articles by Nakamichi, T. Articles by Morikawa, Y.