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J Thorac Cardiovasc Surg 2008;136:1093-1095
© 2008 The American Association for Thoracic Surgery
Brief Communication |
a Department of Cardiac, Thoracic, Transplant, and Vascular Surgery, Hannover Medical School, Hannover, Germany
b Department of Pathology, Hannover Medical School, Hannover, Germany
c Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany
Received for publication November 21, 2007; accepted for publication February 5, 2008. * Address for reprints: Stefan Fischer, MD, MSc, Director, Hannover Thoracic Surgery and Lung Support Program Department of Cardiac, Thoracic, Transplant, and Vascular Surgery, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany. (Email: fischer.stefan{at}mh-hannover.de).
Sarcoma originating from the pulmonary artery (PA) is a rare tumor. In 1923, Mandelstamm1
was the first to describe this disease in an autopsy. Since then, fewer than 200 cases overall have been reported. Because of its rarity and insidious growth characteristics, PA sarcoma is often mistaken for pulmonary embolism, leading to inappropriate therapy such as prolonged anticoagulation or thrombolysis.2
Prognosis is usually poor with a median survival of approximately 12 months with surgical resection of the tumor and 1.5 months without surgical resection of the tumor. Several reports have shown that chemotherapy and radiotherapy may lead to prolonged survival. Only a few cases of complete remission after radical surgical resection have been reported. The longest survival times reported are 3.5 years and 5.5 years.3,4
We report the case of a 48-year-old female patient with recurrent sarcoma of the main PA mimicking fulminant PA embolism 6 years after extensive resection of the primary tumor.
A previously healthy 42-year-old patient developed progressive respiratory distress over a period of several weeks as the result of pleomorphic sarcoma of the mediastinum with infiltration of the left atrium, both pulmonary arteries, the superior vena cava, right main stem bronchus with total occlusion of the right upper lobe, and lung parenchymal infiltration. After initial chemotherapy, surgical resection was performed, including right pneumectomy, resection of the PA bifurcation, and partial resection of the left atrium. The left atrium was patched with pericardial tissue, and the PA was reestablished in an end-to-end technique.
Because of perioperative acute pulmonary embolism, oral anticoagulation was initiated. The patient recovered with mild to moderately reduced physical resilience and without the need for oxygen application.
Six years later, the patient presented with a sudden onset of dyspnea, cyanosis, and recurrent syncopal attacks. Despite oral anticoagulation with phenprocoumon, there was a large filling defect in the left PA, as depicted by computed tomography (CT) scan (
Figure 1, A). Cardiac magnetic resonance imaging and magnetic resonance angiography of the chest showed no signs of tumor recurrence. There was no hypermetabolic activity observed by fluorodeoxyglucose positron emission tomography scan, and bronchoscopy findings were unremarkable. Echocardiography demonstrated moderate impairment of the right side of the heart with tricuspid regurgitation grade II and systolic PA pressure of 55 mm Hg. A presumptive diagnosis of pulmonary embolism was made, and thrombolysis was performed with 40 mg of recombinant tissue plasminogen activator. A follow-up CT scan of the chest failed to show any dissolution of the thrombus.
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A redo median sternotomy was performed, and cardiopulmonary bypass was established through the ascending aorta and the right atrium (2-stage). In mild hypothermia, the main PA trunk was dissected and incised. A thrombus-like structure was found, which almost completely obstructed the PA extending from the commissural level of the pulmonary valve to the trifurcation of the first left upper lobe branch. An intraoperative frozen section revealed a thrombus with portions of sarcoma (
Figure 2, A and B). A supracommissural replacement of the PA main trunk using a PA homograft was performed, which was anastomosed with the left PA at the level of the first branch. The patient was weaned from bypass uneventfully and extubated within 6 hours after surgery. Her recovery was uneventful. She was discharged on postoperative day 7 with oral anticoagulation (international normalized ratio 2.5) and without oxygen substitution. A follow-up CT scan showed normal perfusion of the homograft (Figure 1, B). Eight months after the resection, the patient is recurrence-free.
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The diagnosis of PA sarcoma is often not correctly established before surgery,4 as described in our case. Even though magnetic resonance imaging and positron emission tomography did not demonstrate any signs of malignancy, intraoperative and postoperative histologic examination showed relapse of the sarcoma. It is without doubt that even advanced and unique diagnostics, such as magnetic resonance imaging and positron emission tomography scanning once, again failed to distinguish pulmonary embolism from intravascular sarcoma. A definitive diagnosis of PA sarcoma requires the pathologic examination of tissue obtained by intravascular, percutaneous, or surgical biopsies.5
In this emergency situation, we were able to resect a sarcoma of the PA, which was mimicking fulminant acute pulmonary embolism with tumor-free resection margins, and to solve the acute problem. Improvements are required to establish the diagnosis of this disease correctly. Nevertheless, this case is rare and it shows the feasibility of resection of recurrent sarcomas in areas such as the great vessels even after extensive previous resection.
References
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