Ann Thorac Surg 2006;82:2014-2016
© 2006 The Society of Thoracic Surgeons
Original Articles: General Thoracic
Surgical Experience for the Pulmonary Artery Sarcoma
Ayako Maruo, MDa,*,
Yutaka Okita, MDa,
Kenji Okada, MDa,
Teruo Yamashita, MDa,
Satoshi Tobe, MDb,
Nobuhiro Tanimura, MDc
a Department of Cardiovascular Surgery, Kobe University School of Medicine, Kobe, Hyogo
b Department of Cardiovascular Surgery, Akashi Medical Center, Akashi, Hyogo
c Department of Cardiovascular Surgery, Takatsuki General Hospital, Takatsuki, Osaka, Japan
Accepted for publication July 12, 2006.
* Address correspondence to Dr Maruo, Hyogo Brain and Heart Center, 520 Saisho Ko, Himeji, 670-0981, Japan (Email: ayakom{at}hbhc.jp).
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Abstract
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BACKGROUND: Pulmonary sarcoma arising from the pulmonary artery is a rare disease and its prognosis is disastrous.
METHODS: Five patients who underwent surgery for pulmonary artery sarcoma were reviewed.
RESULTS: All patients except one were initially diagnosed with pulmonary embolism. One patient with preoperative profound shock could not wean from cardiopulmonary bypass. Two patients are still surviving for 36 and 30 months, respectively.
CONCLUSIONS: Early diagnosis and complete surgical resection is perhaps the best way to improve patients with pulmonary artery sarcoma.
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Introduction
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Pulmonary sarcoma arising from the pulmonary artery is an extremely rare disease. The presumptive diagnosis before surgery or autopsy is difficult because the clinical manifestations are similar to pulmonary thromboembolism. The prognosis is disastrous because of the high recurrence ratio of the tumor and an optimal therapeutic strategy is still uncertain. We reviewed 5 cases of pulmonary artery sarcoma treated with surgery.
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Patients and Methods
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Between March 1990 and May 2002, five patients with pulmonary sarcoma underwent surgery. Two of this series (patient 1 and patient 4) have been reported previously [1, 2]. The mean age was 69 ± 11 (56 to 84) years old. There were 4 female patients and 1 male. Clinical features are summarized in Table 1. Only one patient (patient 3) was correctly diagnosed preoperatively. She had been pointed out to have a metastatic tumor in the right lung on routine check-up after surgery for colon cancer. The histologic study of the resected specimen was sarcoma and the pulmonary arterial lesion was found as the primary focus of the sarcoma. Magnetic resonance imaging demonstrated an inhomogeneous enhancing filling defect of the pulmonary artery, consistent with a pulmonary artery sarcoma (Fig 1). The other patients had a preoperative diagnosis of pulmonary thromboembolism and final diagnosis was established during surgery. Three patients had exertional dyspnea and one patient had cardiogenic shock due to pulmonary artery obstruction.
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Fig 1. Computed tomography of patient 2 demonstrates a massive space-occupying mass in the pulmonary artery, which was first misdiagnosed as thromboembolism (left). Preoperative gadolinium-diethylenetriamine-pentaacetic acid magnetic resonance study of patient 3 showed an intraluminal inhomogeneous enhanced tumor in the main and proximal right pulmonary artery (right).
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Results
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In all patients surgery was performed under cardiopulmonary bypass and cardiac arrest except patient 2, who had resection of the tumor and endarterectomy under ventricular fibrillation. Circulatory arrest with deep hypothermia was not utilized in this series. In patient 1 the tumor involved the pulmonary valve and right ventricular muscle. Resection of all involved sites followed right ventricular outflow reconstruction utilizing a stented pericardial prosthesis (Carpentier Edwards Perimount bioprosthesis (Edwards Lifesciences, Irvine, CA) with equine pericardium (Xenomedica; Baxter, Chicago, IL) roll graft was performed [2]. This patient is alive at 36 months after surgery without any sign of tumor recurrence. In patient 2, tumor resection and endarterectomy of the main pulmonary to the right pulmonary artery were performed. Tumor recurrence was found 4 months after surgery and she died one month later. In patient 3, resection of the tumor with pulmonary endarterectomy was performed. An intraoperative frozen section showed positive sarcoma cells from the margin of the specimen and additional endarterectomy was extended to the left pulmonary artery. He had adjuvant chemotherapy after the surgery and is still surviving 30 months later. In patient 4, local tumor recurrence occurred 7 months after surgery and resection of the pulmonary valve and the pulmonary artery trunk, with reconstruction of the pulmonary artery with an equine pericardial patch, was performed. She died of the tumor 10 months after a second surgery. In patient 5, with profound shock, a partial resection of the tumor was done but the patient could not be weaned from cardiopulmonary bypass.
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Comment
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Pulmonary artery sarcoma is a very rare tumor. However, as the number of patients with pulmonary thromboembolism has increased, the more chances to encounter this malignant entity have increased. The tumors usually arise from the main pulmonary artery and sometimes extend distally beyond the bifurcation or proximally into the right ventricular outflow.
There have been no specific symptoms to the neoplasm. Clinical presentations (dyspnea, chest pain, syncope, and palpitations) are similar to pulmonary thromboembolism and are usually related to obstruction of the pulmonary artery [3]. Although preoperative diagnosis is difficult, suspicion of the tumor and careful observation might lead to an accurate diagnosis. Computed tomography could help differentiate the pulmonary artery sarcoma from a pulmonary embolism by a filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery, expansion of the involving arteries, or extraluminal tumor extension [4]. There are reports [5, 6] of a potential usefulness of the gadolinium-enhanced magnetic resonance image or the (2-[18F]-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET).
Optimal therapeutic strategy is still unknown. Surgery can ameliorate symptoms in patients presenting with dyspnea and has the potential to provide late survival. According to Kruger and colleagues [7] prognosis for survival without surgery is 1.5 months and surgery could extend survival to 10 months. There are only a few reports of collecting surgical experiences. Anderson and colleagues [8] presented 6 cases and, even with the largest experience of surgical treatment of chronic pulmonary thromboembolism, preoperative diagnosis was seldom possible and only one patient who had a homograft reconstruction of right ventricular outflow construction could survive. The median survival of their 6 patients was 7.5 months after surgery. Mayer and colleagues [9] reported 7 cases and emphasized an aggressive resection rather than simple endarterectomy. The longest survival was 62 months, in a patient who underwent resection of the tumor and adjuvant chemotherapy. Another proposed procedure is heart and lung transplantation [10]. Even with total removal of all cardiopulmonary structures, however, recurrent metastatic tumors in the extracardiac organs occurred in half of the patients. Considering the results of reported cases, survival after surgical treatment seems to be mainly determined by the sarcoma extension rather than the type of procedure. Although the role of adjuvant therapies is still unclear, several reports demonstrate prolonged survival with adjuvant chemotherapy after surgery [7]. Uchida and colleagues [11] demonstrated a regression of the residual tumor after chemotherapy.
Inconclusion, early diagnosis by suspecting the possibility of a pulmonary sarcoma and surgical resection including pulmonary endarterectomy or the pulmonary trunk itself, combined with the adjuvant chemotherapy, is the most hopeful way to improve patients’ survival.
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References
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Abstract
Introduction
