Ann Thorac Surg 1998;66:2089-2091
© 1998 The Society of Thoracic Surgeons
Case Reports
Leiomyosarcoma of the pulmonary artery
Pascal Dumont, MDa,
Patrice Diot, MDb,
Michel R. Aupart, MDa,
Bernard Toumieux, MDa
a Departments of Thoracic & Cardiac Surgery, University Hospital, Tours, France
b Department of Pneumology, University Hospital, Tours, France
Accepted for publication May 20, 1998.
Address reprint requests to Dr Dumont, Service de Chirurgie Thoracique, Hôpital Trousseau, C.H.U., 37044, Tours Cedex, France
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Abstract
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Primary leiomyosarcoma of the pulmonary artery is an extremely rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. In the present case, early diagnosis and location in the left pulmonary artery permitted resection by pneumonectomy with total cardiopulmonary bypass.
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Introduction
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P rimary sarcoma of the pulmonary artery is very rare and the histologic type called leiomyosarcoma is even rarer. These tumors are frequently misdiagnosed as chronic pulmonary embolism. The majority of such tumors reported in the literature have involved the right ventricular outflow tract and the main pulmonary trunk, often extending into the main pulmonary artery branches [1, 2] and being diagnosed at postmortem examination [3, 4]. The particular features of the case reported here are early diagnosis before surgical treatment and limited extension into the left pulmonary artery. It is hoped that these factors will increase the patient’s chances of survival.
A 42-year-old woman presented at her local hospital in April 1997 with a 3-week history of chest pain, cough, fever, and asthenia. She had no previous history. Therapy by antibiotics was ineffective. Chest radiograph revealed a left pleural effusion and a number of poorly delineated opacities at the periphery of the left lung. Tracheobronchoscopy was normal. Left thoracocentesis revealed exudate with lymphocytes.
Computed tomographic scan and magnetic resonance imaging showed the presence of a left endoluminal pulmonary artery mass (Fig 1). Angiography revealed a thrombus in the left pulmonary artery without perfusion on this side and the radiologist indicated that the lesion had a firm consistency. The ventilation/perfusion scan demonstrated absence of perfusion on the left side. Echocardiography and blood gases were normal. Pelvic echocardiography showed a fibromatous uterus. The second computed tomographic scan revealed absence of response to 2 weeks’ treatment with anticoagulants. In view of the results of these investigations, surgical treatment was indicated and the possibility of tumor was already evoked.
Operation was performed on June 1997 by median sternotomy. Palpation of the left pulmonary artery revealed a mass in the lumen. Total cardiopulmonary bypass was established. Longitudinal incision of the left pulmonary artery revealed a mass that completely occluded the left branch and prolapsed into the main pulmonary artery and the right branch. Section of the left pulmonary artery was extended to the end of the main pulmonary artery and to the origin of the right pulmonary artery. A frozen section of the artery wall showed no tumor at these margins. The mass appeared to be attached only to the wall of the left pulmonary artery and a left pneumonectomy was performed. The main pulmonary artery and the right pulmonary artery were repaired using a lateral Dacron patch. The postoperative course was uncomplicated, and the patient was discharged on the ninth postoperative day.
The patient underwent adjuvant radiotherapy followed by chemotherapy: mesna and ifosfamid (Asta medica, Mérignac, France), doxorubicin (Dakota pharm, Créteil, France), and dacarbazin (Rhone-Poulenc-Roger Bellon, Neuilly sur Seine, France). Eleven months later she was asymptomatic and chest radiography revealed no evidence of recurrence.
The pathologic examination showed a solid tumor involving the media and adventitia of the pulmonary artery left branch. There were no lung metastases. Hilar, subcarinal, and intrapulmonary lymph nodes were free of tumor.
Light microscopy revealed swirling, interlaced bundles of spindle cells with hyperchromatic nuclei and numerous abnormal mitotic figures (Fig 2 ). Immunocytologic staining revealed leiomyosarcoma with a positive reaction on the membrane to vimentin, a positive reaction on the membrane and cytoplasm to desmin, and smooth muscle-specific actin.
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Fig 2. Wall of the left main pulmonary artery. Leiomyosarcoma arising from the media and adventitia. (Orcein stain, original magnification x63.)
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Comment
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Primary sarcoma of the pulmonary artery is a rare disease first described by Mandelstamm in 1923. Since then there have been few reports in the literature and the total number of pulmonary artery sarcomas approaches only 120; leiomyosarcomas of the pulmonary artery are extremely rare, with only 22 reported cases [1, 5–7]. According to the literature, there appears to be a female predominance and leiomyosarcomas are more often observed in the inferior vena cava [8].
The diagnosis of pulmonary sarcoma is difficult because the symptoms are insidious and nonspecific. Indeed, pulmonary embolism was the most common initial clinical diagnosis in a large series. However, atypical features, such as a lack of predisposing factors for thromboembolism, persistence or recurrence of symptoms despite adequate anticoagulation, and unilateral distribution of a massive perfusion defect [4], may evoke the diagnosis of tumoral obstruction. In our patient the ventilation/perfusion scan showed massive unilateral perfusion defect. This is unusual for pulmonary embolism and should suggest obstruction by tumor. However, massive unilateral defect is not entirely specific and may occur in chronic embolism. Computed tomographic scanning cannot differentiate between thrombus and tumor. Angiography has little value but it provides the opportunity to insert an endocardial biopsy catheter, with a view to establishing histologic diagnosis. This biopsy was not performed in the patient reported but firm consistency of the lesion was indicated by the radiologist.
Magnetic resonance imaging was performed but unfortunately without using gadolinium-diethylenetriamine pentaacetic acid, which usually shows a marked increase in the signal intensity of the tumor.
In our patient the tumor was located in the left pulmonary artery. This location is unusual because more than 90% of sarcomas are located in the pulmonary trunk [1, 2]. Invasion of the vessel media and adventitia is also uncommon.
It is well established that early and primary surgical resection is the best treatment [1]. The role of adjuvant therapy has not been yet clearly defined in the literature. The limited experience of any center in the treatment of these neoplasms makes it difficult to evaluate the relative importance of surgical excision and adjuvant therapy. Some investigators are in favor of adjuvant therapy and describe encouraging results [3, 7]. The prognosis remains poor but better survival has been observed when the treatment comprised radical operation, radiotherapy, and chemotherapy. It is hoped that the particular features and responses of this patient will increase the length of survival.
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Acknowledgments
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We thank Doreen Raine for editing the article in English.
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References
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Okada K., Okada M., Yamamoto S., et al. Successful resection of a recurrent leiomyosarcoma of the pulmonary trunk. Ann Thorac Surg 1993;55:1009-1012.[Abstract]
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Delany S.G., Doyle T.C.A., Bunton R.W., Hung N.A., Joblin L.U., Taylor D.R. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631-1633.[Abstract/Free Full Text]
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Head H.D., Flam M.S., John M.J., Lipnik S.S., Slater D.L., Stewart R.D. Long–term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy. Ann Thorac Surg 1992;53:332-334.[Abstract]
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Eng J., Murday A.Y. Leiomyosarcoma of the pulmonary artery. Ann Thorac Surg 1992;53:905-906.[Abstract]
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Akomea–Agyin C., Dussek J.E., Anderson D.R., Hartley R.B. Pulmonary artery sarcoma mimicking pulmonary embolism: successful surgical intervention. Ann Thorac Surg 1996;61:1536-1538.[Abstract/Free Full Text]
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Mazzucco A., Luciani G.B., Bertolini P., Faggian G., Morando G., Ghimenton C. Primary leiomyosarcoma of the pulmonary artery: diagnostic and surgical implications. Ann Thorac Surg 1994;57:222-225.[Abstract]
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Anderson M.B., Kriett J.M., Kapelanski D.P., Tarazi R., Jamieson S.W. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg 1995;59:1487-1490.[Abstract/Free Full Text]
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Burke A.P., Virmani R. Sarcoma of the great vessels; a clinicopathologic study. Cancer 1993;71:1761-1773.[Medline]
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