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© 2002 American Society for Clinical Oncology
Unusual Pulmonary LesionsCase 3. Pulmonary Vein Leiomyosarcoma Presenting as a Left Atrial MassUniversity of North Dakota, Veterans’ Affairs Medical Center, and MeritCare Medical Center, Fargo, ND CASE 3. PULMONARY VEIN LEIOMYOSARCOMA PRESENTING AS A LEFT ATRIAL MASS A 23-year-old man presented to his primary care physician with productive cough and shortness of breath of 5 days’ duration. Review of systems disclosed low-grade fever with night sweats for 2 weeks and generalized weakness for 1 year. Past medical history and family history were unremarkable. Physical examination revealed an afebrile, normotensive individual with a regular pulse of 109 beats per minute. He was in mild respiratory distress and was diaphoretic. Cardiovascular examination revealed a right parasternal heave, 2/6 diastolic murmur over the apex radiating to the axilla. The murmur was accentuated by a change in posture from supine to sitting position. All the routine laboratory tests were within normal limits. Troponin-I and creatine phosphokinase levels were not elevated. ECG showed sinus tachycardia with accentuation of T waves. No ST segment changes were seen. Arterial blood gas analysis revealed a pH of 7.45, pO2 of 36 mmHg, pCO2 of 68 mmHg, and HCO3 of 24 mEq/dL. Oxygen saturation on room air was 92%. Chest radiography demonstrated cardiomegaly and central pulmonary vascular congestion with pulmonary edema (Fig 1). Two-dimensional echocardiogram showed a left atrial mass (Fig 2, arrows; LA, left atrium; LV, left ventricle) highly suggestive of an atrial myxoma. The patient subsequently underwent cardiovascular surgery, which revealed a mass attached to superior and inferior pulmonary veins. The pedunculated mass measured 6.5 x 5.5 x 4.3 cm (Fig 3) and was found prolapsing into the mitral orifice, almost occluding it. It was completely resected. Histopathologic examination (hematoxylin and eosin staining) showed grade 3 leiomyosarcoma arising from the superior pulmonary veins (Fig 4, arrows) that was positive for actin, desmin, and vimentin but negative for factor VIII.
Pulmonary vein leiomyosarcoma is extremely rare. We found only 11 cases previously reported worldwide.1-5 Also, to our knowledge, this is only the second case report in a patient younger than 30 years.5 Experience from the Armed Forces Institute of Pathology6 shows that the preferred site of most cardiac leiomyosarcomas is the left atrium. The subendocardial lining of the atrium normally contains bundles of smooth muscles. Pulmonary vein sarcomas reveal high cellular proliferation of spindle cells with varying degrees of mitotic activity and necrosis. Immunohistochemically, these tumors tend to show reactivity with antibodies to vimentin, desmin, and actin, thereby confirming the presence of smooth muscle differentiation. Aberrant keratin reactivity may be observed, as seen in 40% of Mayo clinic cases.5 There are important differences with other great vessel sarcomas. Aortic sarcomas present in the seventh decade and show no sex predilection. Presentation is usually because of embolic phenomenon. Most patients die within 16 months.6 Sarcomas of the pulmonary artery tend to present in the fifth decade and are more common in men. The symptoms are usually of recurrent pulmonary emboli, and survival varies from months to years; median survival is 23 months.6 Inferior vena cava sarcomas tend to occur in women in the fifth to sixth decades of life. Symptoms are usually of venous thrombosis and inferior vena caval syndrome, and these patients tend to have a more favorable prognosis among vascular sarcomas.5 Pulmonary vein sarcomas occur mostly in women, although our patient is male. Patients tend to present with dyspnea, hemoptysis, and chest pain. All great vessel sarcomas show a tendency to react with antibodies to actin, desmin, and vimentin. Not much is known about the best therapeutic modality for these patients. Chemotherapy and radiation therapy have not been shown to be effective.7,8 Surgical resection with wide margins seems to offer the only chance of cure. REFERENCES 1. Gonzalez-Campora R, Rubin-Uria J, Mora-Marin J, et al: Pulmonary vein myxoid leiomyosarcoma. Pathol Res Pract 185: 900-904, 1989[Medline]
2.
Gyhra AS, Santander CK, Alarcon EC, et al: Leiomyosarcoma of the pulmonary veins with extension to the left atrium. Ann Thorac Surg 61: 1840-1841, 1996 3. Takatsugu S, Hiroshi Y, Uhito Y, et al: Pulmonary leiomyosarcoma extending into left atrium or pulmonary trunk: Complete resection with cardiopulmonary bypass. 4. Tsutsumi Y, Deng YL: Alveolar soft part sarcoma of the pulmonary vein. Acta Pathol Jpn 41: 771-777, 1991[Medline] 5. Oliai BR, Taxelarr HD, Lloyd RV, et al: Leiomyosarcoma of the pulmonary veins. Am J Surg Pathol 23: 1082-1088, 1999[Medline] 6. Burke AP, Virmani R: Sarcomas of the great vessels: A clinicopathologic study. Cancer 71: 1761-1773, 1993[CrossRef][Medline] 7. Burke A, Virmani R: Primary cardiac sarcomas, in Tumors of the Heart and Great Vessels, Vol 16. Atlas of Tumor Pathology, Third Series. Washington, DC, Armed Forces Institute of Pathology; 1996, pp 127-169 8. Mingoli A, Cavallereo A, Sapienza P, et al: International registry of inferior vena cava leiomyosarcoma: Analysis of a world series on 218 patients. Anticancer Res 16: 3201-3206, 1996[Medline]
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Copyright © 2002 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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