Primary sarcoma of pulmonary artery and valve: Multimodality treatment by chemotherapy and homograft replacement

Abstract

Primary pulmonary sarcomas are extremely rare tumors. Since the first description by Mandelstamm 1 in 1923, only about 100 case reports have been published, and only a few have dealt with combined treatment of primary sarcoma. We report on a woman with a metastasizing pulmonary sarcoma of the pulmonary valve with infiltration of the pulmonary artery. After complete remission of the pulmonary metastases with polychemotherapy, the patient underwent curative resection of the tumor and insertion of a cryopreserved homograft. A 49-year-old woman was admitted because of increasing short of breath with exercise. She had pericarditis about 6 months previously. Since then, both chest and back pain occurred frequently, associated with tachycardia and dyspnea. Auscultation revealed a 3/6 systolic ejection murmur in the second left intercostal space and a markedly split second heart sound. There were signs of right ventricular overload on the electrocardiogram. The initial chest radiograph showed several bilateral pulmonary nodular lesions, confirmed by computed tomographic scan. Two-dimensional and transesophageal echocardiography revealed an enlarged right ventricle and an abnormal structure in the main and right pulmonary arteries. Right heart catheterization showed elevated right ventricular and pulmonary arterial pressures, with a ventricular-distal pulmonary artery gradient of 40 mmHg. The main and right pulmonary arteries were seen by cineangiography to be incompletely perfused. Needle biopsy was carried out for cytologic and histologic examination of the pulmonary nodular lesions, which we had suspected and later confirmed to be hemangiosarcoma. Elective resection of the right sided lung metastases was incomplete as a result of life-threatening hemodynamic instability. Histologic examination of one of these tumors revealed granulation and scar tissue without evidence of malignancy. Because of progression of right heart failure and growth of the pulmonary lesions seen on the computed tomographic scan, chemotherapy was initiated. In the subsequent 7 months, polychemotherapy (etoposide, vincristine, ifosfamide, adriamycin) was administered for six