Eur J Cardiothorac Surg 2007;32:388-390. doi:10.1016/j.ejcts.2007.04.019
Copyright © 2007, European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved
Long-term survival of a pulmonary artery sarcoma produced by aggressive surgical resection and adjuvant chemoradiotherapy
Atsushi Nakahiraa,b,
Hitoshi Oginoa,*,
Hiroaki Sasakia,
Nobuyuki Katakamic
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Cardiovascular Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
c Department of Pulmonary Medicine, Kobe City General Hospital, Hyogo, Japan
Received 21 February 2007;
received in revised form 3 April 2007;
accepted 5 April 2007.
* Corresponding author. Address: Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka 565-8565, Japan. Tel.: +81 6 6833 5012; fax: +81 6 6872 7486. (Email: hogino{at}hsp.ncvc.go.jp).
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Abstract
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Malignant pulmonary artery tumors represented by sarcomas are rare, but fatal. Early diagnosis and radical surgical resection offer the only chance for survival. However, surgical intervention has some challenging aspects, and prognosis is poor even after tumor resection. We report a case of a pulmonary artery sarcoma between the right ventricular outflow tract and the pulmonary artery branches. The tumor was aggressively extracted with reconstruction using a cryopreserved pulmonary valved allograft, followed by adjuvant chemoradiotherapy. At 56 months after surgery, the patient is well without any evidence of recurrence, demonstrating that aggressive surgical resection with adjuvant chemoradiotherapy can prolong survival.
Key Words: Pulmonary artery • Tumor • Allograft • Adjuvant therapy
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1. Introduction
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Malignant pulmonary artery tumors represented by sarcomas, although rare, are fatal. They are often misdiagnosed as pulmonary thromboembolic disease because clinical manifestations are similar. Prognosis for survival without surgical resection is 1.5 months; with surgical resection it can be up to 10 months [1]. The only chance for longer survival is by early diagnosis and radical resection [2–4]. However, complete resection of malignant pulmonary artery tumors is challenging because they usually originate from the pulmonary artery trunk and grow intraluminally both proximally into the right ventricular outflow tract and distally beyond the pulmonary artery bifurcation [2–5]. They also have a tendency to embolize to peripheral trees of the pulmonary artery as a recurrence, which worsens the prognosis [4,5]. We report a case with 56-month survival without any sign of recurrence – the result of aggressive surgical resection and adjuvant chemoradiotherapy.
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2. Case report
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A 38-year-old male complained of progressive dyspnea on effort. Echocardiography showed severe pulmonary artery stenosis with serious dilatation of the right ventricle. Catheterization revealed pulmonary artery stenosis with a maximum pressure gradient of 88 mmHg (Fig. 1A). Computed tomographic scans (CT-scans) showed a main pulmonary artery mass extending from the right ventricular outflow tract to the pulmonary artery branches (Fig. 1B). Magnetic resonance imaging (MRI) using gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) showed enhancement of a thickened main pulmonary artery wall, suggesting the existence of a vascularized tumor. Surgical intervention was undertaken.
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Fig. 1. Preoperative pulmonary angiography (A) and computed tomographic scan with three-dimensional reconstruction (B) show a pulmonary artery sarcoma invading the right ventricular outflow tract and pulmonary artery trunk along the wall. It extends into the bilateral pulmonary arteries, especially into the right upper and interlobar branches. An operative drawing (C) and postoperative computed tomographic scan with three-dimensional reconstruction (D) clearly show the replaced cryopreserved pulmonary valved allograft between the right ventricular outflow tract and distal pulmonary artery branches, with anastomotic sites indexed by black arrowheads.
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Under a cardiopulmonary bypass, the hard and severely thickened main pulmonary artery was incised. An intraoperative frozen section of the inside white fibrous mass showed that the tumor was a primary pulmonary artery sarcoma. The tumor showed fast-growing behavior and had grown more diffusely and intraluminally than shown on the angiography and CT-scans taken 2 months previously (Fig. 1A and B). It had extended to the more proximal right ventricular outflow tract with invasion to all structures of pulmonary valve (Fig. 2C) and the more distal pulmonary artery branches (Fig. 2D). The tumor distally extended into the right upper and interlobar pulmonary arteries (Fig. 2A) and the left pulmonary arteries along the wall. The pulmonary valve, the right ventricular outflow tract, the pulmonary artery trunk, and the bilateral pulmonary arteries, including all visible tumor components, were extracted (Fig. 2B). Reconstruction using a cryopreserved pulmonary valved allograft, containing parts of right ventricular outflow tract and bilateral pulmonary artery branches, was carried out (Fig. 1C and D). The proximal anastomosis was performed in the remnant of the transected right ventricular outflow tract (Fig. 1C). Regarding the distal anastomoses, the native bilateral pulmonary artery branches were trimmed as distally as possible in the bifurcation of upper and interlobar branches after removal of tumor components, where end-to-end anastomoses with the allograft were undertaken (Fig. 1C and D). Histological examination revealed an epithelioid hemangioendothelioma with undifferentiated spindle cell proliferations that had a similar malignant potential to that of a sarcoma. Although there was no invasion of the adventitia, the distal margin of the right pulmonary artery unfortunately had tumor component. Adjuvant concurrent chemoradiotherapy was also instituted. Chemotherapy included 168 mg carboplatin and 80 mg paclitaxel weekly for 6 weeks. A total dose of 60 Gray irradiation was performed by 2 Gray/day for 30 days in the margin just beside the distal right pulmonary artery anastomosis where there were residual tumor components. At 56 months after surgery, the patient was well without any evidence of recurrence.
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Fig. 2. (A) Right pulmonary artery was opened just before the bifurcation of upper and interlobar branches and stuffed tumor component is clearly seen. (B) A part of the excised tumor inside the pulmonary artery trunk and bilateral branches. (C) Pulmonary valve leaflets are severely thickened and destroyed by the tumor invasion. (D) This operative drawing shows the opened right pulmonary artery and opened pulmonary artery trunk. It clearly shows the tumor components stuffed in the right pulmonary artery and the invasion to the pulmonary valves.
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3. Comment
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Malignant pulmonary artery tumors represented by sarcomas are rare and fatal. They usually originate from a wall of the pulmonary artery trunk and grow intraluminally without remarkable symptoms until the end stage [2–5]. As their clinical features as well as radiological findings resemble pulmonary thromboembolic disease [2–6], diagnosis tends to be mistaken or delayed. Early diagnosis and radical surgical resection offer the only chance for longer survival [2–4], with only three cases reported to have survival of longer than 3 years [2,3,5]. Our case has survived for 56 months after aggressive surgical resection with the adjuvant chemoradiotherapy. MRI using Gd-DTPA [3–5], which was suggestive of a pulmonary artery vascularized tumor, enabled the early surgical intervention, although the biopsy did not provide for a correct diagnosis. The sensitivity of the biopsy was thought to be influenced by appositional thrombotic materials covering the tumor [6].
Malignant pulmonary artery tumors originate from pluripotential mesenchymal cells of the intima of the pulmonary artery trunk or the bulbus cordi, with a variety of histopathologic patterns [4]. They usually extend intraluminally to the right ventricular outflow tract and the pulmonary artery branches [2–6] and rarely invade through the adventitia to the surrounding structures. Considering the embryological relationship and the tumor growth behavior, central pulmonary artery resection including the right ventricular outflow tract is essential. For reconstruction of the right ventricular outflow tract, the pulmonary artery trunk, and its branches, a cryopreserved pulmonary valved allograft is useful as it will enable a much wider resection with greater flexible adaptability in distal anastomoses than other materials [2–5]. Heart-lung resection followed by heart-lung transplantation might be another option; however, this can show poor survival outcomes because of the development of recurrences and metastatic diseases with prolonged administration of immunosuppressive drugs [7].
Malignant pulmonary artery tumors have a tendency to embolize to peripheral trees of the pulmonary artery in the direction of blood stream, resulting in poor survival outcomes even after radical resection of tumors [4,5]. Considering the trend for distal microembolization of tumors, adjuvant therapy should be added for radical cure [4], though this has not been well proven because of the limited reports [2–5,8]. Among the chemotherapy trials that have been reported, there is none of a definite effect from chemotherapy except for that of Uchida et al. [8]. They demonstrated that a combination of ifosfamide and epirubicin was effective for radiological regression of the residual tumor after surgical removal of a pulmonary artery sarcoma, done like a pulmonary thrombectomy. In our case the residual tumor in the distal margin seemed to be controlled by the adjuvant chemoradiotherapy, including a combination of carboplatin and paclitaxel. There are many varieties of histopathologic patterns; thus more case reports should help determine for which histopathologic types adjuvant therapy is effective and what is the most beneficial adjuvant protocol combined with surgical resection.
In conclusion, aggressive resection of a malignant pulmonary artery tumor with reconstruction using a cryopreserved pulmonary valved allograft was successful. Adjuvant chemoradiotherapy is also worth undertaking to improve the prognosis.
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References
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Abstract
1. Introduction
