Ann Thorac Surg 2004;77:331-334
© 2004 The Society of Thoracic Surgeons
Case report
Primary chondromatous osteosarcoma of the pulmonary artery
Yoshio Tsunezuka, MD, PhDa*,
Makoto Oda, MD, PhDa,
Masao Takahashi, MD, PhDa,
Hiroshi Minato, MD, PhDb,
Go Watanabe, MD, PhDa
a Department of General and Cardiothoracic Surgery, Kanazawa, Japan
b Department of Pathology, Kanazawa University School of Medicine, Kanazawa, Japan
Accepted for publication April 29, 2003.
* Address reprint requests to Dr Shiraishi, Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan 602-8566.
e-mail: isao{at}koto.kpu-m.ac.jp
 |
Abstract
|
|---|
Primary pulmonary sarcoma is an extremely rare malignancy, pulmonary osteogenic sarcoma is even more rare and has a poor prognosis. We present a report of a 58-year-old woman with primary pulmonary osteosarcoma occupying the right main pulmonary artery and extending to the main pulmonary artery. Right pneumonectomy was performed with total right main pulmonary artery resection under cardiopulmonary bypass. Microscopic examination demonstrated that tumor cells contained mainly chondromatous components and were atypical cells that became osteoid. There is no sign of recurrence 24 months postoperatively. The present case is the only case yet reported with even an intermediate-term survival without recurrence.
|
Introduction
|
|---|
Primary pulmonary sarcoma is an extremely rare malignancy and osteogenic sarcoma is even more rare. Extraskeletal osteosarcoma usually arises in somatic soft tissues and paraenchymal organs such as soft tissue, kidney, and thyroid gland. We report here a surgically respectable case of primary chondromatous osteosarcoma of the pulmonary artery, and discuss the case and previous case reports.
A 58-year-old woman was admitted to Kanazawa University Hospital on February 19, 2001 for evaluation and treatment of dyspnea on effort and transient chest pain that began 5 months before admission. She had been treated with heparin at another hospital because the diagnosis of pulmonary emboli was made by chest computed tomography (CT) in September 2000, but the emboli gradually enlarged in spite of treatment. She underwent total resection of the uterus for myoma uteri in 1991, and had a history of hypertension since 1993. Physical examination revealed vital signs within normal limits and bilateral breath sounds were equal without adventitious sounds. Significant laboratory findings included C-reactive protein 0.6 U and serum alkaline phosphatase 330 U/L. Arterial blood gas analysis indicated hypoxemia at room air; pH 7.397, PaCO2 44.6 mm Hg, PaO2 72.5 mm Hg, HCO3 -26.8 mmol/L, BE 1.6 mmol/L, and SaO2 96.1%. Tumor markers, CEA, SCC, CYFRA, NSE, and proGRP were within normal limits. Chest roentgenogram did not reveal congestion or abnormal mass shadows. Dynamic enhanced CT revealed complete occlusion of the proximal right pulmonary artery by a solid mass, which was not enhanced or accompanied by calcification. A tumorlike shadow was revealed in right upper lobe (S2). The top of the solid intravascular mass was lobular and smooth, and jutted out into the pulmonary trunk. The right peripheral pulmonary artery was slightly dilated but not beaded. Magnetic resonance imaging revealed T1-low and T2-high intensity filling lesions in the pulmonary artery (Fig 1).
The pulmonary angiogram demonstrated complete occlusion of the right pulmonary artery, but no filling defect in the left pulmonary artery. Pulmonary artery pressure was 46/11 mm Hg, right ventricular pressure was 35/4 mm Hg, and pulmonary artery resistance was 187 dyne · s · cm. Scans from a ventilation perfusion study indicated that ventilation was normal but there was almost no perfusion to the right lung.
View larger version (110K):
[in this window]
[in a new window]
|
Fig 1. (Top) Magnetic resonance imaging of the chest. High intensity filling lesions in right main pulmonary artery, and the top of the solid intravascular mass was lobular and smooth and jutted out into the pulmonary trunk. (Bottom) Surgical specimens; the tumor filled the lumen of the right main pulmonary to a peripheral segmental artery.
|
|
The patient underwent median sternotomy under separate ventilation. Partial resection of the right upper lobe region containing the mass was performed first, and the mass was diagnosed as pulmonary infarction with focal pneumonia. She was then placed on cardiopulmonary bypass, the peripheral right pulmonary artery was opened, and biopsy of the material was performed. The intraoperative immediate histologic study indicated a frozen section diagnosis of chondrosarcoma or chondromatous osteosarcoma. The pulmonary trunk was opened and a balloon catheter was placed into the left main pulmonary artery to inhibit migration of the tumor to the left lung. The top of the tumor was a multilobular cyst and the edge was smooth. The pulmonary trunk was opened and incised apart from the tumor adhesion to the wall of the pulmonary artery. The stump was closed with 3–0 Prolene (Ethicon, Somerville, NJ). The right main pulmonary artery was ablated and a right pneumonectomy was performed. In the gross pathologic specimen the tumor filled the lumen of the right main pulmonary to the peripheral segmental artery, but did not invade lung tissues (Fig 1). The tumor's origin in the pulmonary artery was unknown. Microscopic examination demonstrated that tumor cells in the artery had mainly chondromatous components and atypical cells that became osteoid. On the edge of the chondromatous islet, the major findings were immature mesenchymal spindle cells and partial cells. Some atypical cells transmigrated to osteoid. These pathologic findings were compatible with chondromatous osteosarcoma, which originated primarily from the pulmonary artery. Postoperative bone scan was normal. Adjuvant chemotherapy has not been performed at the patient's request. The patient is now in good health without tumor recurrence 24-months after surgery.
|
Comment
|
|---|
Primary osteosarcoma of the pulmonary artery is extremely rare. Thirteen cases of primary pulmonary osteosarcoma and 7 cases of primary pulmonary artery osteosarcoma have been reported (Table 1).
Our patient is the eighth reported case of pulmonary artery osteosarcoma. Clinically there is no gender predilection of primary pulmonary artery sarcoma, but 7 of 8 patients (87.5%) were women. The clinical symptoms are usually exertional dyspnea, cough, and chest pain. Many of the patients were diagnosed at postmortem examination. Ramp and coworkers [1] reported that the diagnosis of primary pulmonary sarcoma was made at postmortem examination in 60% of patients. The reasons are that the disease is difficult to diagnose before surgical exploration and most patients were late staged with distal metastasis, or had progressive sized tumors at admission. The diagnosis of pulmonary artery tumor is difficult to establish with conventional radiologic studies because plain chest roentgenograms are normal in the majority patients [2]. Shepard and colleagues [3] reported that dilated and beaded peripheral pulmonary arteries on CT are highly suggestive of pulmonary metastatic intravascular tumor emboli, but beaded peripheral findings were not observed in the present case. It is difficult to distinguish pulmonary artery tumor or pulmonary artery thrombus by CT. Including the present patient, anticoagulant therapy with heparin was performed in 3 patients as an initial therapy for the diagnosis of pulmonary artery thrombus. Interestingly, peripheral wedge-shaped opacities were thought to represent small infarcts in 2 patients. The present patient had a peripheral round opacity in the right upper lobe and we diagnosed it as a pulmonary infarction based on intraoperative histologic study.
Before surgery we considered four disease hypotheses of the two abnormal shadows in our patient: the first was that both pulmonary artery emboli and the peripheral opacity were tumors; the second was that neither one was a tumor, but rather one was a thrombus and the other an infarction; the third was that one was a malignancy; and the fourth was a infarction or thrombus. The CT indicated rapid growth of the pulmonary artery emboli, and given that progression to the left main pulmonary artery would lead to sudden death whether or not the emboli was a tumor, we selected surgical extirpation. If neither were determined to be malignant during the intraoperative histologic study, the surgical procedure would be to cut it and then suture at the bifurcation of the right pulmonary artery without pneumonectomy.
In general, the prognosis of primary pulmonary osteosarcoma is poor and the recurrence rate is high. No patients who had chemotherapy or radiation alone without surgical therapy survived longer than 1 year. Of those 7 previously reported patients, 5 patients died within 1 year after admission. Four patients underwent surgery, but 2 patients died less than 1 year after surgery, 1 patient had recurrence 9 months postoperatively, and the outcome was not reported for 1 patient.
Even if the surgical procedure was complete resection of tumor, distal metastasis or local recurrence could occur and careful follow-up is essential [4]. We performed pneumonectomy and resection of total right pulmonary artery. The patient had no recurrence 24 months after operation. The present report is the only patient yet described with even an intermediate-term survival without recurrence. Pneumonectomy was performed in 1 patient other than our patient [5], which described a right pneumonectomy and a left atrium resection that were performed. The operation was radical because the stump of the resected specimen was negative for malignancy, but recurrence occurred 3 months after the operation, and the patient died from superior vena cava syndrome due to mediastinal lymph node metastasis. Similar to that report, in our patient the operation was radical because the tumor existed only in the pulmonary artery, and the stump of the right main pulmonary artery was negative for malignancy. We think that the prognosis is excellent because the pathologic chondroblastic component was contained in the resected tumor in the present patient [6].
Tumor embolectomy was performed in 2 patients, but residual tumor recurred at the same location in 1 patient and endobronchial reperfusion hemorrhage was observed in the other, indicating a poor prognosis. Tumor embolectomy has two severe demerits, one is the incompleteness of resection of tumor and the other is reperfusion injury. The relationship between the tumor and intima of the pulmonary artery is very intimate, therefore complete resection of tumor cannot be achieved with embolectomy. Even if a tumor exists within narrow limits, such as main pulmonary artery or trunchus in preoperative diagnosis, micrometastasis could exist in peripheral vessels. Also, to make matters worse, embolectomy may mechanically promote metastasis to peripheral sites. Secondly, ischemic reperfusion injury is also a severe complication after embolectomy. It is characterized by pulmonary edema caused by endothelial dysfunction, platelet aggregation, and neutrophil activation and sequestration because free radicals are produced in the endothelial cell membranes and in neutrophils, causing an increase in cell membrane permeability. Embolectomy causes massive hemoptysis and severe hemosputum clinically as symptoms of ischemic reperfusion injury, which leads to death at the worst. Therefore, we think that embolectomy is contraindicated for the treatment of pulmonary artery tumor and that total resection of the tumor region with careful follow-up is recommended.
This report has discussed the treatment and outcome of a patient with primary pulmonary osteosarcoma, which has resulted in good prognosis confirmed 24-months postoperatively after performing pneumonectomy and resection of total right pulmonary artery. We believe the procedures reported here can be a useful example of the treatment of this rare malignancy.
|
References
|
|---|
- Ramp U.G., Ramp U., Gerharz C.D., et al. Sarcoma of the pulmonary artery: report of two cases and a review of the literature. J Cancer Res Clin Oncol 1992;118:551-556.[Medline]
- Kane R.D., Hawkins K.H., Miller J.A., Noce P.S. Microscopic pulmonary tumor emboli associated with dyspnea. Cancer 1975;36:1473-1482.[Medline]
- Shepard J.O., Moore E.H., Templeton P.A., McLoud T.C. Pulmonary intravascular tumor emboli: dilated and beated peripheral pulmonary arteries at CT. Radiology 1993;187:797-801.[Abstract/Free Full Text]
- Lyerly H.K., Reves J.G., Sabiston D.C., Jr Management of pulmonary sarcomas of the pulmonary artery and reperfusion intrabronchial hemorrhage. Surg Gynecol Obstet 1986;163:291-301.[Medline]
- Kimura I., Kiuchi H., Sakamoto Y., Yamamoto K., Dohi Y., Takahama M. Primary osteogenic sarcoma of pulmonary artery. Jpn J Med 1990;29:32-37.[Medline]
- Polypoid sarcoma of the pulmonary trunk. analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma. Cancer 1980;46:314–24
- McConnell T.H. Bony and cartilabinous tumors of the heart and great vessels. Cancer 1970;25:611.[Medline]
- Murthy M.S.N., Meckstroth C.V., Merkle B.H., Huston J.T., Cattaneo S.M. Primary intimal sarcoma of pulmonary valve and trunk with osteogenic sarcomatous elements. Arch Pathol Lab Med 1976;100:649-651.[Medline]
- Shimizu M., Murata T., Doi Y., Kitamura K. An autopsy case of the osteosarcoma originating from the pulmonary artery. Nichinan Kaishi 1983;72:1041.
- Madu E.C., Taylor D.C., Durzinsky D.S., Fraker T.D., Jr. Primary intimal sarcoma of the pulmonary trunk simulating pulmonary embolism. Am Heart J 1993;125:1790-1792.[Medline]
- Fujii H., Osako M., Otani H. Primary pulmonary artery sarcoma. Jpn Circ J 1998;62:379-381.[Medline]
This article has been cited by other articles:
|
|
|
|
 
S. H. Blackmon, D. C. Rice, A. M. Correa, R. Mehran, J. B. Putnam, W. R. Smythe, J.-C. Walkes, G. L. Walsh, C. Moran, H. Singh, et al.
Management of Primary Pulmonary Artery Sarcomas.
Ann. Thorac. Surg.,
March 1, 2009;
87(3):
977 - 984.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
