Background: Pulmonary artery (PA) angiosarcomas are unusual, can present like massive pulmonary emboli, and have high rates of local recurrence and poor long-term prognoses. We sought to avoid pneumonectomy for presumed residual disease and to improve local control. Methods: A 51-year-old male presented with a pulmonary intimal angiosarcoma that filled the left PA and by extension into the right PA caused hemodynamic compromise. After macroscopic complete resection with cardiopulmonary bypass using thromboendarterectomy techniques, the patient was discharged uneventfully but tolerated adjuvant chemotherapy with four cycles of doxorubicin/ifosfamide poorly. Fifteen months later, the patient developed multiple left upper lobe nodules. In addition to planned lobectomy, regional lung chemotherapy was offered as part a clinical trial (“Suffusion”, NCT-03965234, initial dose level). Results: Using a bronchial blocker catheter, the left main PA was occluded, the left pulmonary veins snared thoracoscopically, and the isolated vascular bed partially drained by withdrawing blood and immediately reinfusing systemically. Then doxorubicin (2.8 mg/m2, 3.75 % systemic dose, 75% local effect) in 84 cc of volume was injected to refill vascular bed yielding a 30-minute suffusion dwell time. After reperfusion by snare release and PA balloon deflation, a thoracoscopic left upper lobectomy followed immediately yielding negative surgical margins. Dissection was tedious because of pleural and perivascular fibrotic adhesions from the previous surgical and medical therapies. The patient had an uncomplicated 3-day hospital stay. Using serial differential perfusion scans, 30-day lung function loss did not exceed that predicted by lobectomy. Early imaging shows no additional disease progression. By six months, additional metastases were detected in the remaining lung and chemotherapy was restarted. Conclusions: While established to control parenchymal disease in general, the intravascular origin of pulmonary intimal sarcoma makes regional vascular chemotherapy particularly attractive. It was performed safely in this patient and further research is needed to determine whether higher dose levels exceeding that from peripheral delivery will provide better local long-term controls.
