Ann Thorac Surg 2007;83:302-304
© 2007 The Society of Thoracic Surgeons
Case Reports
Preoperative Embolization Followed by Surgical Excision of an Intrapericardial Pheochromocytoma
Ased M. Ali, MRCSa,
Mohan Devbhandari, MRCSa,
Anand Sastry, MRCSa,
Raymond Joel Ashleigh, FRCRb,
Mark Trevor Jones, FRCSa,*
a Department of Cardiothoracic Surgery, Manchester, United Kingdom
b Department of Radiology, Wythenshawe Hospital, Manchester, United Kingdom
Accepted for publication May 31, 2006.
* Address correspondence to Dr Jones, Wythenshawe Hospital, Department of Cardiothoracic Surgery, Manchester, UK M23 9LT (Email: mark.jones{at}smuht.nwest.nhs.uk).
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Abstract
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A teenager with palpitations and hypertension was found to have an intrapericardial pheochromocytoma of the left atrium with multiple feeding collaterals. Radiologic embolization was carried out on the major feeding arteries to the tumor preoperatively with good angiographic result. Surgery was carried out the following day through a median sternotomy approach and cardiopulmonary bypass. Minimal bleeding was observed due to prior embolization. The patient made a rapid postoperative recovery and was discharged 7 days later.
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Introduction
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We demonstrate that preoperative embolization is useful in decreasing pheochromocytoma vascularity, facilitating complete and safe excision.
Paragangliomas are rare neoplasms of neural crest origin, which when they secrete catecholamines, are called pheochromocytomas. We report the case of a teenager presenting with intrapericardial pheochromocytoma, which was resected successfully after preoperative embolization.
A 16-year-old boy, with a previous history of excision of a neck chemodectoma at age 10, presented with palpitations, tiredness, and hypertension. Raised urinary norepinephrine (2.96 mg/24 h) and serum norepinephrine (24.3 nmol/L) indicated a functional paraganglioma. Chest roentgenograms revealed a prominent left hilum, and echocardiography showed irregularity in the superior mediastinum. Iodine-131-meta-iodobenzylguanidine scans revealed an avid area in the left mediastinum. Contrast-enhanced cardiac computed tomographic scan confirmed a 5-cm highly vascular intrapericardial tumor with multiple collaterals at the roof of the left atrium, posterior to the right ventricular outflow tract and lateral to the ascending aorta (Fig 1). On the basis of the clinical picture and imaging, pheochromocytoma was diagnosed.
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Fig 1. Cardiac-gated computed tomographic scan of the tumor (anteroposterior [AP] view, maximum intensity projection reconstruction). Relationships of the tumor to the left ventricular outflow tract (LVOT) and ascending aorta (AA) can be seen. (T = tumor.)
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The patient was prepared by instituting 2 weeks of aggressive blood pressure control with a standard regime for preoperative treatment of phaeochromocytomas [1]. This used 
-adrenoreceptors blockers for 10 to 14 days prior to surgery (doxazosin) and ß-adrenoreceptor blockade (atenolol) was started after several days to avoid tachyarrhythmia. A calcium antagonist was added as a further agent (amlodipine). A blood pressure of approximately 120/60 mm Hg was achieved preoperatively. Embolization was carried out through a right-femoral approach using a 5-French sheath and various pre-shaped catheters and microcatheters. Diagnostic angiography of the thoracic branches of the descending thoracic aorta and both internal thoracic arteries were carried out (Fig 2). The major branches supplying the tumor were the left bronchial artery, superior intercostals, and small branches from both internal thoracic arteries. The appropriate branches were embolized with polyvinyl alcohol (350–500 micron size), which were believed to be the most efficacious agent to maximally occlude blood supply and reduce risk of collateralization. A good angiographic result was achieved from embolization (Fig 3) with preservation of parent vessels including the internal thoracic arteries. After embolization, the patient’s blood pressure remained at approximately 120/60 mm Hg.
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Fig 2. Super selective angiogram of the branch of the intercostobronchial trunk showing tumor circulation.
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At surgery the next day the chest was opened through a median sternotomy. Phentolamine (3 mg) was used for blood pressure control on opening the pericardium, and no other agent was required. A 4-cm vascular fleshy tumor was found over the roof of the left atrium behind the main pulmonary artery. Routine aorto-atrial cannulation was used to establish cardiopulmonary bypass. The main pulmonary artery was divided to access the tumor. Despite the vascularity of the tumor, little bleeding was observed due to embolization. A segment of the proximal left anterior descending coronary artery, which was completely encased by tumor, was sacrificed, and continuity was restored with an interposition saphenous vein graft. With meticulous dissection the tumor was excised completely without major fluctuations in blood pressure during manipulation and dissection. The patient was weaned off the cardiopulmonary bypass onto a high dose of norepinephrine due to the loss of vasoconstrictive drive. The patient was electively sedated and ventilated overnight for optimum hemodynamic control. He was extubated the following morning with a normal ECG. He was transferred to the ward 4 days later and was discharged after 7 days. At his 6-month follow-up he remains normotensive without medication. Histology confirmed a completely excised intrapericardial pheochromocytoma.
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Comment
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Cardiac pheochromocytomas are rare tumors [2, 3]. They arise from coronary or visceral autonomic paraganglia of the atria. Based on clinical history they are diagnosed on biochemical evidence of hypercatecholaminemia. To locate all tumor foci, scanning with radio-iodinated iodine-131-meta-iodobenzylguanidine is reported to be the best modality with alternatives being octreotide and technetium (99mTc). Additional computed tomography or magnetic resonance imaging assists in showing precise anatomic location and relationships.
Typical features include a soft fleshy texture, high vascularity, adhesion to surrounding structures, and local invasion making resection challenging [3]. Cardiac pheochromocytomas lack clear planes of dissection and therefore excision of surrounding myocardium and vasculature with reconstruction through pericardial patches and grafts may be required for complete resection. Completeness of resection relates to increased survival at follow-up [4].
These tumors are highly vascular and prone to fatal hemorrhagic complications [3]. Preoperative embolization has a proven role in minimizing bleeding and facilitating dissection in vascularized tumors. Though well described in neck and abdominal pheochromocytomas, there are no previous reports of this procedure used for intrapericardial pheochromocytomas. The only two previously reported cases of preoperative embolization were nonsecretory mediastinal paragangliomas [5, 6]. In both cases embolization was only carried out after unexpected severe hemorrhage forcing the operation to be abandoned at first attempt.
For pheochromocytomas, surgery continues to be first line treatment [7] and is based on surgical principles of adequate exposure, meticulous hemostasis, minimal tumor manipulation, and complete resection with intact capsule. The surgical approach to the tumor and use of cardiopulmonary bypass varies, depending on anatomical location, surgeon preference, and experience. The most popular approach is the median sternotomy with cardiopulmonary bypass, which allows adequate vascular control and manipulation [2, 4–7]. Thoracotomy with or without cardiopulmonary bypass has also been used.
Preoperative embolization has a valuable role in the management of cardiac pheochromocytoma. Our experience shows that it reduces hemorrhage, results in greater intraoperative hemodynamic stability, and therefore facilitates handling of the tumor and resection off the surrounding structures. We believe that this multimodality approach helps create a safer operation with reduced risk of complications for both the surgeon and the anesthesiologist.
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References
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- Lenders JWM, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma Lancet 2005;366:665-675.[Medline]
- Besterman E, Bromley LL, Peart WS. An intrapericardial pheochromocytoma Br Heart J 1974;36:318-320.[Free Full Text]
- Orringer MB, Sisson JC, Glazer G, et al. Surgical treatment of cardiac pheochromocytomas J Thorac Cardiovasc Surg 1985;89:753-757.[Abstract]
- Lamy AL, Fradet GJ, Luoma A, Nelems B. Anterior and middle mediastinum paraganglioma: complete resection is the treatment of choice Ann Thorac Surg 1994;57:249-252.[Abstract]
- Drucker EA, McLoud TC, Dedrick CG, Hilgenberg AD, Geller SC, Shepard JA. Mediastinal paraganglioma: radiologic evaluation of an unusual vascular tumor AJR 1987;148:521-522.[Free Full Text]
- 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]
- Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Duffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma): a case report and review of the literature J Thorac Cardiovasc Surg 1978;76:183-189.[Abstract]
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Abstract
Introduction
