Pulmonary artery sarcoma is a rare tumor of the cardiovascular system, first described by Mandelstramm in 1923. Here, we present a case of metastatic pulmonary artery intimal sarcoma mimicking saddle pulmonary embolism.
A 38-year-old male presented to his PCP with a six week history of progressive dyspnea on exertion, decreased exercise tolerance, frequent palpitations, and pleuritic chest pain. Chest radiography revealed two masses in the left upper lung. Chest CT confirmed a large mass in the left upper lobe, and smaller mass in the lingula. PET scan revealed uptake in the mediastinum. The patient underwent fine needle aspiration of the left upper lobe lesion, revealing low grade spindle cell tumor. Subsequently, diagnostic mediastinoscopy with lymph node dissection was performed. Pathology was negative for malignant cells. Five days later, the patient reported persistent dyspnea and pleuritic chest pain while in his pulmonologist’s office. Vitals revealed a significant tachycardia with a heart rate of 120. An emergent CT Angiogram of the chest revealed bilateral pulmonary emboli and an apparent saddle embolism within the main pulmonary artery. He was hospitalized and initiated on anticoagulation therapy with Heparin, and an IVC filter was placed. Despite a presumed large clot burden, the patient remained hemodynamically stable, and transthoracic echocardiogram revealed normal right and left heart function with normal pulmonary artery pressures. His symptoms improved and he was discharged home on Lovenox. One week later, he again developed worsening dyspnea and recurrent chest pain. Repeat CT angiogram of the chest did not reveal any significant changes. A VQ Scan was then obtained, showing multiple segmental and subsegmental abnormalities consistent with pulmonary emboli. The greatest perfusion defects were noted in the lingula, right middle lobe and right lower lobe. Given his lack of response to anticoagulation therapy, the diagnosis of possible pulmonary artery sarcoma was entertained. He was transferred to a facility specializing in pulmonary endarterectomy. An MRI of the chest confirmed suspicion of a mass in the pulmonary artery. He eventually underwent right and left pulmonary endarterectomy. Pathology revealed thrombus as well as intimal sarcoma. He is currently undergoing chemotherapy with Sunitinib 50mg daily, which he is tolerating well.
Pulmonary artery sarcomas usually arise from the dorsal pulmonary trunk, growing along the intima of the arterial wall in the direction of blood flow. They can also arise from the right and left pulmonary arteries, the pulmonary valve, and the right ventricular outflow tract. Histology most often reveals angiosarcoma. Our patient’s histology showed intimal sarcoma, which is rare and distinct from angiosarcomas in that it is highly cellular, radiographically dense, and poorly responsive to chemotherapy.Review of the literature reveals five case reports of pulmonary artery sarcoma initially presenting as pulmonary embolism. Common symptoms include hemoptysis, dyspnea (75%), and chest pain or cough (50%). Patients may also develop weight loss, fever, anemia, and digital clubbing. Other subtle findings may include an elevated erythrocyte sedimentation rate, the absence of a procoagulant state, and lack of prior deep vein thrombosis history. Historically, mean survival is 18 months, and 5-year survival is 18.5%. Surgical resection with curative intent portends the best survival. Multi-modality therapy, involving surgical resection with neoadjuvent chemotherapy, is currently recommended.
Pulmonary artery intimal sarcoma is a rare tumor which carries a poor prognosis. Diagnosis is often delayed, as symptoms are often subtle and easily mistaken for thromboembolic disease. Failure to appropriately respond to anticoagulation should prompt consideration of this diagnosis.
Alison Kole, No Financial Disclosure Information; No Product/Research Disclosure Information