Ann Thorac Surg 2007;83:2201-2203
© 2007 The Society of Thoracic Surgeons
Case Reports
Surgical Management of a Giant Thoracic Angiomyolipoma
William L. Holman, MDa,*,
Lisa Diethelm, MDd,
Audrey J. Lazenby, MDc,
Thomas S. Winokur, MDc,
Ralph T. Lyerly, III, MDb,
Robert J. Cerfolio, MDa
a Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama
b Department of Anesthesiology, University of Alabama at Birmingham, Birmingham, Alabama
c Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama
d Department of Radiology; Ochsner Health System, New Orleans, Louisiana
Accepted for publication December 28, 2006.
* Address correspondence to Dr Holman, Room 719, 703 19th St South, Birmingham, AL 35294-0007 (Email: wholman{at}uab.edu).
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Abstract
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This report describes the surgical management of a tumor that filled the left chest of a 58-year-old man. Histopathologic examination showed that this was an angiomyolipoma, a tumor that most commonly occurs in the kidney. The preoperative evaluation and intraoperative management are presented, along with a brief review of this unusual neoplasm.
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Introduction
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Angiomyolipomas are rare tumors that most commonly occur in the kidneys. This patient had a 4.9-kg angiomyolipoma that filled the left hemithorax, displaced the heart, and crushed the left lung. The tumor was removed through a median sternotomy approach.
The patient is a 58-year-old man who presented to his local physician with a chief complaint of worsening shortness of breath. A chest roentgenogram (Fig 1) showed opacification of the left hemithorax with rightward shifting of the trachea and heart. The chest computed tomography scan (Fig 2 and 3)
and magnetic resonance imaging studies confirmed the presence of a large mass having mixed characteristics consistent with whorls of adipose and fibrous tissue. There was no compression of the right ventricle or left atrium seen in these views, suggesting a slowly growing mass. The left hemidiaphragm was inverted by the tumor, and there was no evidence for local or distant spread of the tumor. Preoperative bronchoscopy showed complete occlusion of the left main stem bronchus by external compression.
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Fig 1. This posteroanterior chest roentgenogram reveals considerable displacement of mediastinal structures into the right hemithorax, with nearly complete opacification of the left hemithorax. Minimal aeration is maintained subsegmentally in the apex of the left lung.
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Fig 2. This chest computed tomography image shows rightward mediastinal shift without compression of the left pulmonary artery or superior vena cava. Compressed airless lung is adjacent to the tumors, which themselves contain whorls of fat within the soft tissue.
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Fig 3. A computed tomography image at the level of the right pulmonary artery demonstrates the marked rightward shift of the great vessels. This view suggests that a lateral thoracotomy approach for removal of the mass would further compress the contents of the right chest and compromise cardiac and pulmonary function.
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A median sternotomy was chosen for exposure. Dissection of the tumor started with the most accessible areas that were between the anterior surface of the tumor and the chest wall, and the inferior surface of the tumor and the diaphragm. During this dissection, there was no evidence for invasion of the tumor into surrounding structures. The tumor was intraparenchymal in the left lung, and tissue that was consistent with chronically compressed lung was identified. It did not inflate with positive pressure ventilation.
The initial dissection was continued until limited exposure prevented advancement, and at this point the pericardium was opened. The left pulmonary artery and pulmonary veins were stapled and divided from within the pericardium, and then the dissection was resumed outside the pericardium to identify the left main stem bronchus at the hilum. After the left main stem bronchus was stapled and divided, the medial portions of the tumor were dissected free of their mediastinal attachments. The posterior adhesions of the tumor mass to the chest wall and diaphragm could then be divided. The tumor was too large to deliver en bloc from the chest, so it was cleaved along planes between the three lobes of the tumor. The masses were removed along with the left lung.
The tumor masses and lung weighed 4.9 kg in total. Visual inspection of the cut tumor surface showed homogenous yellow-red whorls of tissue. Interlacing fascicles of spindle cells and bland adipose tissue were seen on histologic examination. The spindle cells stained positive for smooth muscle actin. The final diagnosis was angiomyolipoma. The patient remains well 14 months after surgery. No evidence of recurrent chest masses have been found on posteroanterior and lateral chest roentgenograms.
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Comment
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Angiomyolipomas are rare and typically occur in the kidney, although a few mediastinal and pulmonary angiomyolipomas have been reported [1–4]. Most renal angiomyolipomas occur in association with tuberous sclerosis, but this association is uncommon for extrarenal angiomyolipomas [5].
Thoracic angiomyolipomas may be an incidental finding at autopsy, or they can cause symptoms that result from their location and size [2, 6]. Indications for resection of these tumors include diagnosis of a thoracic mass lesion and relief of symptoms caused by compression by the tumor. Removal of a thoracic angiomyolipoma eliminates the small possibility that this tumor could become malignant, but it should be recognized that only renal angiomyolipomas have been shown to undergo malignant transformation [7, 8].
A median sternotomy approach was chosen for this case, which allowed safer removal of the large noncompressible mass than a lateral thoracotomy approach. Opening the pericardium with a median sternotomy incision provided good exposure for dissection of the pulmonary artery and pulmonary veins. Moreover, the heart and great vessels were immediately available for cannulation or defibrillation in the event of severe bleeding or arrhythmias.
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References
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- Lowe BA, Brewer J, Houghton DC, Jacobson E, Pitre T. Malignant transformation of angiomyolipoma J Urol 1992;147:1356-1358.[Medline]
- Martignoni G, Pea M, Rigaud G, et al. Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions Am J Surg Pathol 2000;24:889-894.[Medline]
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Abstract
Introduction
