Eur J Cardiothorac Surg 2001;20:1049-1051
© 2001 Elsevier Science NL
Leiomyosarcoma of the pulmonary artery — a diagnostic chameleon
A. Hoffmeiera,
M. Semika,
E.M. Fallenbergb,
H.H. Schelda
a Klinik und Poliklinik für Thorax-, Herz- und Gefäßchirurgie, Universitätsklinik Münster, Albert Schweitzer Strasse 33, D-48149 Münster, Germany
b Institut für Klinische Radiologie-Röntgendiagnostik, Universitätsklinik Münster, Albert Schweitzer Strasse 33, D-48149 Münster, Germany
Received 26 February 2001;
received in revised form 20 July 2001;
accepted 20 July 2001.
Corresponding author. Tel.: +49-251-834-7401; fax: +49-251-834-8316
e-mail: andreas.hoffmeier{at}thgms.uni-muenster.de
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Abstract
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A 60-year-old woman suffered from right-sided pain in the back and dyspnea suspicious for chronic pulmonary thromboembolism. Since computed tomography demonstrated a progressive filling defect within the left pulmonary artery, a transvenous biopsy was taken, which demonstrated malignant sarcoma. The patient underwent left-sided pneumonectomy with the aid of cardiopulmonary bypass. In case of a suspected ‘chronic‘ pulmonary embolism with occlusion of a main pulmonary artery, as may be seen with imaging techniques, a sarcomatous disease should be ruled out, especially if there are no coagulation disorders, and the tumor obliterations progress in serial CT scans despite effective anticoagulation.
Key Words: Leiomyosarcoma • Pulmonary embolism • Pneumonectomy
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1. Introduction
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Primary malignant tumors of the pulmonary arteries are very rare with an incidence of 0.001–0.03%, and they are nearly always highly malignant sarcoma that typically obtain their origin from the intima [1]. Since its first description in 1923, a total of 148 cases of primary sarcoma of the pulmonary arteries have been reported in the literature, of which 31 were leiomyosarcomas [1–3]. The underlying pathophysiology of these tumors of the pulmonary arteries is still unclear [1,4].
We report on a case of leiomyosarcoma of a pulmonary artery, for which there were considerable difficulties in establishing the correct diagnosis.
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2. Case report
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In October 1999, a 60-year-old woman was admitted to a local hospital with chest pain radiating to the left hemi-thorax and a history of intermittent dyspnea on exertion for several months. Since her medical history revealed an overseas flight just before the onset of symptoms, thromboembolic disease with pulmonary embolism was suspected, but a body examination showed no clinical evidence of deep vein thrombosis. Pulmonary scintigraphy demonstrated reduced tracer accumulation in the mediobasal left-sided pulmonary segments, and the arterial blood gas analysis was normal. Since computed tomography demonstrated thrombosis of the left pulmonary artery and a left-sided, infarction-like pneumonia, an intravenous treatment with heparin and antibiotics was started. Pneumonic infiltrates partially resumed and the patient's condition improved. As the underlying cause of thrombus formation remained unclear, an extensive laboratory search for thrombophilic diathetic disorders was initiated, but remained negative. In the following weeks, the patient's condition did not improve. As echocardiography demonstrated progressive right ventricular dilatation, the patient was referred for pulmonary embolectomy.
Repeated computed tomography revealed a progressive vascular occlusion, which was extending up into the left upper lobe artery (Fig. 1). A transvenous biopsy revealed malignant sarcoma. The patient was scheduled for surgery in August 2000, since preoperative staging showed no further pathological manifestations. After median sternotomy and institution of extra-corporeal circulation, the left pulmonary artery was opened on the beating heart. A yellowish, shiny, partially necrotic tumor partially firmly attached to the vessel wall became visible (Fig. 2). A rapid biopsy was taken which confirmed the diagnosis, and consecutively, left-sided pneumonectomy with appropriate lymphadenectomy and resection of the adjacent pericardium was performed. The histopathological examination showed no penetration of the vessel wall, and no metastasis in the adjacent lymph nodes and the resected pericardium. Considering the limited knowledge regarding this rare tumor, the patient was postoperatively treated with six cycles of chemotherapy with Doxorubicin (75 mg/m2).
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Fig. 1. Control CT-scan, 4 months after the first CT-scan, demonstrating tumor growth extending into the main left pulmonary artery and the left upper lobe artery.
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Eleven months postoperatively, the patient is being treated on an outpatient basis. There is no evidence of recurrent tumor on computed tomography and bronchoscopy.
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3. Discussion
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Leiomyosarcoma of the pulmonary artery is a rare tumor of the lung. The patient's non-specific symptoms, including cough, dyspnea on exertion, chest pain, right ventricular failure [1], are often misinterpreted as being related to pulmonary embolism, and a sarcoma is either considered not at all or too late. Both diseases are typically seen between the ages of 40 and 60, and women are involved twice as often as men [5]. Physical examination, ECG and the chest X-ray may not reveal abnormal findings. However, cardiomegaly and radiological signs of a peripheral hypoperfusion can be present if a large tumor mass is obstructing a main pulmonary artery vessel. Pulmonary angiography may demonstrate abrupt interruption of the contrast medium flow without a visible thrombus. Usually, all of these diagnostic procedures fail to differentiate between ‘chronic’ pulmonary embolism and intravascular leiomyosarcoma, as frequently does computed tomography, since penetration of the vessel and involvement of adjacent lymph nodes has been observed in only 50% of cases [5]. Similarly, it may not be possible to differentiate the diseases by magnetic resonance imaging, as in our case, despite an assumed difference in signal intensity or contrast uptake ability between the sarcoma and thrombus. Apart from leiomyosarcoma, Takayasu arteritis, giant cell arteritis, fibrosing mediastinitis and histoplasmosis may be found as rare causes of partial or complete occlusion of pulmonary arteries, but these diseases enter the pulmonary vessels from outside, like bronchogenic and mediastinal tumors, and further pathological findings in the imaging techniques are frequent. In our opinion, a tumor biopsy is the only means to establish the proper diagnosis [1,5].
The therapy for intravascular leiomyosarcoma is based on complete tumor resection whenever possible, since this is the only way with a chance of cure. As the overall experience is limited due to the malignancy of the disease, various treatment modalities, including chemotherapy and radiation, have been enrolled in patients with extensive tumor spread. The mean survival following diagnosis without therapy is short with only 1.5 months, but can be considerably extended by total excision of the tumor. Moffat and Yamamoto reported on survival rates ranging from a few weeks up to 3.5 years [5,6]. Recent data indicated a survival of even 10 years or more following complete surgical resection, so that, from our point of view, the operative procedure should be favored whenever possible [7,8]. The usefulness of adjuvant radiotherapy or chemotherapy is difficult to estimate and cannot be assessed due to the small number of cases so far [9].
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4. Conclusions
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In conclusion, in cases of a suspected ‘chronic‘ pulmonary embolism with occlusion of a main pulmonary artery as may be seen with imaging techniques, a sarcomatous disease should be ruled out, especially if there are no coagulation disorders, and the tumor obliterations progress in serial CT scans despite effective anticoagulation.
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Abstract
1. Introduction
