Pulmonary Artery Sarcoma Masquerading as Pulmonary Embolism

1Mayo Clinic, Rochester, MN

Meeting: Hospital Medicine 2007, May 23-25, Dallas, Texas

Abstract number: 147

Case Presentation:

A 39‐year‐old woman with a medical history of polycystic ovarian syndrome presented with pleuritic chest pain, fever, progressive dyspnea on exertion for 1 month, and right upper quadrant abdominal pain. Her physical exam was significant for a temperature of 38.8°C and a positive Murphy's sign. Laboratory findings included elevated total and direct bilirubin, alkaline phosphatase, and ALT. D‐dimer was within normal limits. CXR, ECG, and echocardiogram results were unremarkable. Acute cholecystitis was diagnosed by ultrasound and MRCP; a laparoscopic cholecystectomy was performed. Herfeverand abdominal pain resolved, but her chest pain and ex‐ertional dyspnea progressed. Within 6 weeks she was unable to climb a flight of stairs and was referred to a cardiologist. The cardiac exam revealed a loud P2, a split S2, and a holosystolic murmur. A CT angiogram revealed a lobulated mass involving the main and left pulmonary arteries extending into the left segmental arteries. A thrombophilia workup was negative. A pulmonary embolism (PE) was diagnosed, and heparin was initiated. An echocardiogram showed an elevated right ventricular systolic pressure of 71 mm Hg with right ventricular dilation. Direct administration of tPA to the lesion for 41 hours resulted in no improvement. She was then transferred to a tertiary‐care center. A bilateral lower extremity ultrasound was negative. A repeat CT angiogram showed a large nodular filling defect extending from the main pulmonary artery to the proximal left main pulmonary artery. Cardiothoracic surgery was consulted, and an endarterectomy was performed. Surgical pathology and histopathology findings were consistent with poorly differentiated leiomyosarcoma.

Discussion:

Pulmonary artery sarcomas are rare tumors, with approximately 200 cases described in the literature. The mean age of patients diagnosed with a pulmonary artery sarcoma is 49.3 years with neither sex predominant. As in this case, the condition is often confused with PE. Common presenting symptoms are dyspnea, chest pain, cough, and hemoptysis. CT findings are generally nonspecific; but a filling defect occupying the entire luminal diameter of the artery and/or expansion of any segment of the artery containing the filling defect are both highly suspicious of angiosarcoma. Treatment consists of local excision, pneumonectomy, or endarterectomy. These tumors tend to embolize, with frequent recurrence and metastasis. Prognosis is dismal, with a mean survival of 1.5 months without surgery and 14‐18 months with therapy.

Conclusions:

Rarity and nonspecific clinical presentation make pulmonary artery sarcoma a diagnostic challenge. As illustrated by this case, a suspected pulmonary embolus that fails to improve despite adequate anticoagulation should heighten suspicion of pulmonary artery sarcoma. Failure to considerthis diagnosis may lead to unnecessary and potentially harmful interventions such as prolonged administration of thrombolytics.

Author Disclosure:

K. Ravi, None.

To cite this abstract:

Ravi K. Pulmonary Artery Sarcoma Masquerading as Pulmonary Embolism. Abstract published at Hospital Medicine 2007, May 23-25, Dallas, Texas Abstract 147. Journal of Hospital Medicine. 2007; 2 (suppl 2). https://www.shmabstracts.com/abstract/pulmonary-artery-sarcoma-masquerading-as-pulmonary-embolism/. Accessed April 25, 2019.

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