Ann Thorac Surg 2009;87:1284-1286. doi:10.1016/j.athoracsur.2008.08.048
© 2009 The Society of Thoracic Surgeons
Case Reports
Aorticopulmonary Paraganglioma With Severe Obstruction of the Pulmonary Artery: Successful Combined Treatment by Stenting and Surgery
Naser Qedra, MDa,*,
Mahdi Kadry, MDa,
Semih Buz, MDa,
Rudolf Meyer, MD, PhDa,
Peter Ewert, MD, PhDb,
Roland Hetzer, MD, PhDa
a Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
b Department of Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany
Accepted for publication August 6, 2008.
* Address corresondence to Dr Qedra, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, Berlin, 13353, Germany (Email: qedra{at}gmx.de).
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Abstract
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Aorticopulmonary paraganglioma is a rare tumor of the middle mediastinum. Complete surgical resection is the only effective treatment, even when it may pose a surgical challenge due to the proximity of the tumor to the heart and great vessels, often rendering complete resection difficult to achieve. We report the case of a 30-year-old woman with an aorticopulmonary paraganglioma who presented with severe pulmonary hypertension due to obstruction of the pulmonary artery. In the first step, stenting of the pulmonary artery was performed and 2 months later a radical resection of the tumor using cardiopulmonary bypass under circulatory arrest and deep hypothermia was carried out. In addition, the ascending aorta and aortic arch were replaced by a prosthesis. The patient is in optimal condition and has now been disease-free for almost 7 years. We believe that this is the first description in the English literature of a successful combined management strategy in view of such an unusual manifestation of aorticopulmonary paraganglioma.
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Introduction
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Mediastinal paragangliomas are rare, arising from the paraganglia in the region of the cardiac plexus (middle mediastinum) or along the aorticosympathetic chain in the costovertebral sulcus (posterior mediastinum) [1, 2]. Histologically identical to pheochromocytomas, they may be benign or malignant. An aorticopulmonary paraganglioma is either asymptomatic or it presents with compression or invasion of the local structures or with hormonal activity. Because of the close location to the great vessels and sometimes the trachea, complete resection is very difficult. Paragangliomas are invasive and have a high local recurrence and metastatic rate of 55.7% and 26.6%, respectively [1]. Because radiation and chemotherapy are ineffective, surgery is the therapy of choice, striving whenever possible for complete resection.
A 30-year-old woman was admitted to our institution, having had dyspnea, chest pain, and epigastric pain for 3 weeks. The patient reported slight stress-induced dyspnea in the past 3 years, which had increased in the last 3 weeks during an infection. Physical examination revealed normal blood pressure, a known systolic murmur, and normal breath sounds. Signs of stress of the right ventricle (RV) were observed by echocardiogram. This showed severe regurgitation of the tricuspid valve, a circular pericardial effusion (1.3 to 1.6 cm), elevation of the end-systolic pressure in the RV (115 mm Hg), and dilatation of the RV (end-diastolic diameter, 42 mm) and of the inferior caval vein and hepatic vein.
On the first day after admission, a computed tomographic scan showed a mass located in the aortopulmonary window around the ascending aorta and extending to the aortic arch and supra-aortic vessels, causing stenosis of the pulmonary artery (Figs 1A,
1B). On the following day, we performed a left-sided mini-thoracotomy to obtain tissue biopsies and a sample of the effusion. Histologic investigation of the mass yielded the diagnosis of paraganglioma. A three-dimensional angio-magnetic resonance image performed on day 8 showed compression of the right pulmonary artery (PA) to a diameter of 6 mm and of the left PA to a diameter of 4 mm (Fig 2A). During the heart catheterization performed on hospital day 10, the following systolic pressure values were measured: proximal to the stenosis in the pulmonary artery trunk, 85 mm Hg; distal to the stenosis in the right PA, 9 mm Hg, and in the left PA, 13 mm Hg. The first therapeutic step was to relieve the RV by stenting both PAs during catheterization (Fig 2B). Four days later the patient was able to leave the hospital with significant clinical improvement.
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Fig 1. Computed tomographic (CT) scan of the chest showing (A) the tumor mass surrounding the ascending aorta and (B) the compression of the pulmonary artery. (C, D) The CT scan 7 years later shows freedom from local recurrence. (D) Also shows the stent in the pulmonary artery.
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Fig 2. (A) Three-dimensional angio-magnetic resonance imaging of the pulmonary arteries (PAs) showing the severity of the stenosis. (B) Angiography of the PAs after stenting.
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Two months later the RV function had improved and the tricuspid regurgitation lessened (grade I). The patient underwent a median sternotomy. The pericardium and left pleura were opened to allow adequate exposure of the tumor, which surrounded the ascending aorta and extended to the anterior wall of the aortic arch and the supra-aortic vessels. Using cardiopulmonary bypass, deep hypothermia (16°C), temporary circulatory arrest, and retrograde cerebral perfusion, the tumor mass was carefully mobilized en-bloc off the ascending aorta; it had infiltrated the adventitia, whereas the media was free of tumor. The ascending aorta and aortic arch were opened and a tumor extension into the left carotid artery was identified. The tumor mass was radically resected. The tumor mass around the PA could also be mobilized and totally resected, removing as much tissue as possible, including the adventitia of the PA. Opening or reconstruction of the PA (or both of these together) was not necessary. The stent in the PA was left in situ. Prosthetic replacement of the ascending aorta and aortic arch up to the descending aorta with a tube prosthesis (Hemashield tube, 22 mm; Boston Scientific, Natick, MA) was performed. The patient was weaned from cardiopulmonary bypass. The thin, fragile aortic tissue and temporary right ventricular failure were challenging. Due to long hemostasis time, the operation lasted a total of 16 hours (total circulatory arrest time, 64 minutes; aortic clamp time, 120 minutes; cardiopulmonary bypass time, 556 minutes). On the first postoperative day, a re-thoracotomy was necessary twice due to bleeding. The patient was weaned from the respirator on postoperative day 11 and she left the intensive care unit on postoperative day 18.
The histology of the surgical specimens was characteristic of a paraganglioma, because the tumor cells were moderately polymorphic with a low proliferation rate. Almost 7 years later there has been no recurrence of the tumor and the patient's heart and lung functions at this follow-up were very good (Figs 1C and 1D).
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Comment
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According to the classification by Glenner and Grimley [3], aorticopulmonary paraganglioma is one of the branchiomeric paragangliomas located around the root of the ascending aorta and pulmonary trunk, and it is derived from the parasympathetic nervous system as an aortic or aorticopulmonary paraganglion.
The aorticopulmonary paraganglioma is such a rare tumor that only approximately 100 cases have been reported in the English literature [1]. It has a slight female predominance, and the average age at time of diagnosis is 49 years [4]. The aorticopulmonary paraganglioma is often difficult to resect because of the anatomical position and high vascularity of the tumor, and due to severe bleeding. Incomplete resection and high operative mortality (5.3% to 9%) have been reported [1, 4]. To prevent or control operative bleeding, the artery feeding the tumor has been embolized or the pleural cavity has been packed for 48 hours postoperatively [5, 6].
In our case, echocardiography showed the first evidence of increasing pressure in the RV and PA. By means of the biopsies taken during the diagnostic operation from the lung, the pericardium, and the pericardial effusion, pathologic findings in the adjacent organs could be excluded. To achieve rapid improvement of the patient's clinical condition and to relieve the RV, the stenoses of the PAs were eliminated by stenting. This step created a good basis for the planned tumor resection. Despite the infiltrative character and the excessive extension of the tumor mass, the aim of the operation was the radical resection of the tumor, taking into consideration its low proliferation in the histologic findings and the young age of the patient. The expected bleeding in the tumor bed made long hemostasis after cardiopulmonary bypass necessary.
In conclusion, in this unusual case of paraganglioma, treatment by initial stenting of the pulmonary artery followed later by surgical tumor resection was difficult, but helpful, and entirely successful. The careful preoperative assessment and the multi-disciplinary management were crucial, making the resection of this complex mediastinal paraganglioma feasible, with good long-term result.
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Acknowledgments
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The authors thank Franziska Hintz for data collection, Astrid Benhennour for literature research, and Anne Gale for editorial assistance.
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References
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- Herrera MF, Van Heerden JA, Puga FJ, Hogan MJ, Carney JA. Mediastinal paraganglioma: a surgical experience Ann Thorac Surg 1993;56:1096-1100.[Abstract/Free Full Text]
- Glenner GG, Grimley PM. Tumors of the extraadrenal paraganglion systems Atlas of tumor pathology, 2nd series, fasc 9. Washington, DC: Armed Forces Institute of Pathology; 1974.
- Lack EE, Stillinger RA, Colvin DB, Groves RM, Burnette DG. Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature Cancer 1979;43:269-278.[Medline]
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- Rakovich G, Ferraro P, Therasse E, Duranceau A. Preoperative embolization in the management of a mediastinal paraganglioma Ann Thorac Surg 2001;72:601-603.[Abstract/Free Full Text]
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Abstract
Introduction
