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Journal of Clinical Oncology, Vol 24, No 21 (July 20), 2006: pp. 3502-3504 © 2006 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.04.9171
Ventricular Tachycardia Indicating Cardiac Involvement in Metastatic LeiomyosarcomaDepartment of Cardiology and Pulmonology, Charité-Campus Benjamin Franklin, Berlin, Germany
Department of Radiology, Charité-Campus Virchow-Klinikum, Berlin, Germany
Departments of Hematology, Oncology and Transfusion Medicine; General, Vascular, and Thoracic Surgery; and Cardiology and Pulmonology, Charité-Campus Benjamin Franklin, Berlin, Germany A 64-year-old white man presented in our emergency room with a first episode of left-sided chest pain and shortness of breath in April 2004. He had been in good physical condition and had not taken any medication regularly. His history had been unremarkable thus far. Specifically, he denied having any weight loss, fever, or night sweats. No cardiovascular risk factors were known. The ECG on admission showed inferiorly directed sustained right ventricular tachycardia with 180 beats per minute and left bundle-branch block configuration (Fig 1). The arrhythmia converted to stable sinus rhythm after intravenous administration of 50 mg of ajmaline. Chest x-ray, echocardiography, and coronary angiography were unremarkable. An electrophysiological study reproduced the right ventricular outflow tract tachycardia seen on admission without hemodynamic compromise. Oral treatment with 150 mg of propafenone bid was initiated and the patient remained in stable sinus rhythm as documented by Holter ECG. The patient underwent magnetic resonance imaging to rule out arrythmogenic right ventricular dysplasia. T1-weighted spin-echo images showed a 4 cm x 3 cm x 3 cm irregular mass isointense to the myocardium. This structure originated from the anterolateral pericardium and extended into the left ventricular wall (Fig 2; arrow; AoR, aortic root; DAo, descending aorta; LA, left atrium, RVOT, right ventricular outflow tract). There was no evidence of arrhythmogenic right ventricular dysplasia. Marginal gadolinium enhancement was demonstrated. The mass exhibited higher signal intensity on the T2-weighted images. A chest computed tomography (CT) scan confirmed the cardiac mass and also revealed multiple pulmonary lesions. Video-assisted thoracoscopy with atypical pulmonary wedge excision was performed, and the histologic examination of the left superior pulmonary segment S6 (Fig 3; arrow) showed three foci of a grade 2 vascular leiomyosarcoma. Clinical examination and CT staging yielded no evidence of another tumor localization. Chemotherapy with ifosfamide (5g/m2) and liposomal doxorubicin (75 mg/m2) was initiated. In May 2005, restaging after five therapy cycles showed stable disease on magnetic resonance imaging (Fig 2, inset) and CT. Our patient has been free of symptoms for 25 months.
Cardiac leiomyosarcoma with local tumor growth in the ventricular outflow tract was the structural substrate for right ventricular outflow tract tachycardia in our patient. In 2004, soft tissue and bone sarcomas accounted for about 0.8% of 1,368,030 newly diagnosed malignant tumors in the United States.1 Soft tissue sarcomas represent 78% of all sarcomas2 and may originate from mesenchymal tissue at all anatomic sites of the body. Approximately 18% are located within the body trunk.3 Most sarcomas cause nonspecific symptoms only and are therefore diagnosed in a late stage when metastatic disease is already present. Early hematogenic, predominantly pulmonary metastatic spread of soft-tissue sarcomas is commonly seen and is blamed for the poor prognosis associated with this disease.4 The absence of metastases at the time of diagnosis is associated with a better prognosis. Primary cardiac leiomyosarcomas, as found in our patient, are rare and account for 8% to 9% of all malignant primary cardiac tumors.5 Progressive local tumor growth may lead to congestive heart failure and death. Soft tissue sarcomas are resected surgically whenever feasible. In our patient, even metastasectomy of the multiple pulmonary lesions was considered. This approach was abandoned, however, since the cardiac mass detected on magnetic resonance imaging seemed to infiltrate the left ventricular wall and was therefore felt to be not completely excisable. Standard therapy for a metastasized and inoperable soft tissue sarcoma consists of combined chemotherapy with doxorubicin and ifosfamide, as administered in our patient.6 To reduce the well-documented cardiotoxicity of anthracycline, this drug was applied in a liposomal formulation. Clinical studies on patients with advanced soft tissue sarcomas achieved tumor remission in 30% to 45% of the cases with combined doxorubicin and ifosfamide treatment. Unfortunately, the recurrence-free interval is usually short. The overall mean survival time in patients with metastasizing soft tissue sarcoma is approximately 10 months despite therapy.7 Patients who show no progress after four or five cycles of chemotherapy seem to have a better prognosis. In our patient leiomyosarcoma was detected incidentally due to thorough evaluation of cardiac arrhythmia. Combined chemotherapy could be initiated immediately. Stable disease and a survival better than average could be achieved. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Jemal A, Tiwari RC, Murray T, et al: Cancer statistics, 2004. Cancer J Clin 54:8-29, 2004 2. Enzinger FM, Weiss SW: Soft Tissue Tumors. St Louis, MO, CV Mosby, 1983 3. Lawrence W Jr, Donegan WL, Natarajan N, et al: Adult soft tissue sarcomas: A pattern of care survey for the American College of Surgeons. Ann Surg 205:349-359, 1987[Medline] 4. Bear PA, Moodie DS: Malignant primary cardiac tumours: Cleveland Clinic experience, 1956-1986. Chest 92:860-870, 1987 5. Burk A, Virmani R: Tumors of the heart and great vessels, in Atlas of Tumor Pathology (vol 16). Washington, DC, Armed Forces Institute of Pathology, 1996, 127-170 6. Antunes MJ, Vanderdonck KM, Andrade CM, et al: Primary cardiac leiomyosarcomas. Ann Thorac Surg 51:999-1001, 1991[Abstract] 7. Agrawal Y, Agrawal SK, Srivastava AK, et al: Primary cardiac tumors: Surgical experience and follow-up. Indian Heart J 55:632-636, 2003[Medline]
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Copyright © 2006 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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