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Case Reports
. 2014 Oct 1;41(5):515-7.
doi: 10.14503/THIJ-13-3735. eCollection 2014 Oct.

Pulmonary artery sarcoma mimicking pulmonary embolism

Magdy M El-Sayed Ahmed, Muhammad Aftab, Raed M Al-Najjar, Kim I de la Cruz, Robert S Benjamin, Charles H Hallman
Case Reports

Pulmonary artery sarcoma mimicking pulmonary embolism

Magdy M El-Sayed Ahmed et al. Tex Heart Inst J. .

Abstract

Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.

Keywords: Diagnosis, differential; diagnostic errors; dyspnea; pulmonary artery sarcoma; pulmonary embolism; pulmonary valve; sarcoma/diagnosis/pathology/surgery; tomography, x-ray computed; vascular neoplasms/diagnosis/surgery.

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Figures

Fig. 1.
Fig. 1.
Computed tomogram of the chest shows the tumor mass within the pulmonary trunk and its extensions into the pulmonary artery main branches (arrows).
Fig. 2.
Fig. 2.
Transesophageal echocardiograms show an echodense structure A) at the root (arrow) of the pulmonary trunk and B) in the right ventricular outflow tract (arrow). Supplemental motion images are available for Figures 2A and 2B.
Fig. 3.
Fig. 3.
Photomicrograph of the resected specimen shows a proliferation of spindle cells in a vague storiform pattern (white arrows). The cells have pleomorphic nuclei with readily identifiable mitotic figures (including atypical forms), and an eosinophilic cytoplasm. Present as well in the collagenous stroma are scattered inflammatory cells, predominantly lymphocytes (black arrows) (H & E, orig. ×200).
Fig. 4.
Fig. 4.
Computed tomograms of the chest show the tumor (arrows) A) before and B) after postoperative chemotherapy and radiation therapy.
See this image and copyright information in PMC

Comment in

  • More on pulmonary artery sarcoma.
    Fred HL. Fred HL. Tex Heart Inst J. 2014 Oct 1;41(5):514. doi: 10.14503/THIJ-14-4635. eCollection 2014 Oct. Tex Heart Inst J. 2014. PMID: 25425985 Free PMC article. No abstract available.

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