Ann Thorac Surg 2004;78:2161-2163
© 2004 The Society of Thoracic Surgeons
Case report
Anterior Mediastinal Presentation of a Giant Angiomyolipoma
Afzal M.I. Amir, MDa,
Clark J. Zeebregts, MD*,a,
H. Jan Mulder, MD, PhDa
a Department of Surgery, Medisch Spectrum Twente, Enschede, The Netherlands
Accepted for publication August 1, 2003.
* Address reprint requests to Dr Zeebregts, Department of Vascular Surgery, University Hospital Groningen, PO Box 30.001, Groningen 9700 RB, The Netherlands
czeebregts{at}hotmail.com
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Abstract
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Angiomyolipomas are benign, solitary, noninvasive lesions that most often arise in the kidney. Extrarenal manifestations of these tumors include the skin, oropharynx, the abdominal wall, retroperitoneum, gastrointestinal tract, heart, lung, liver, uterus, penis, and spinal cord. We report a patient with a giant angiomyolipoma located in the anterior mediastinum. We believe this is the seventh reported case of mediastinal angiomyolipoma and the largest reported by size. It is the second reported lesion to arise in the anterior mediastinum. Distinction from other pulmonary or thoracic masses relies on the appreciation of the unique and characteristic histologic features of these mediastinal angiomyolipomas. We conclude that, although rare, angiomyolipoma should be considered in the differential diagnosis of a mediastinal tumor.
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Introduction
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Angiomyolipomas are mesenchymal tumors that are composed of an admixture of fat, smooth muscle cells, and tortuous, thick-walled, small- to medium-sized blood vessels [1]. They may arise in the setting of tuberous sclerosis, comprising half of all cases (Bourneville's disease), or otherwise normal individuals [1]. Angiomyolipomas most often affect renal tissue, but they also occasionally arise at extrarenal locations, among which the liver has been the most frequently reported site. At the level of the mediastinum, only six of these tumors have been previously reported in world literature [2–7].
We describe the occurrence of a giant angiomyolipoma within the upper anterior mediastinum of a 22-year-old man. We believe this is the seventh report of a mediastinal angiomyolipoma. Consistencies and differences of the other cases are discussed.
A 22-year-old man with psychosis was admitted to the psychiatric department of our hospital. The psychosocial stressful moment leading to his behavior was the impending results of his school examination. There was no relevant previous medical history. At admission the patient complained of dry coughing and suffered dyspnea on exercise. He had neither fever nor excessive night sweating. On physical examination we found diminished auscultation and percussion sounds noted at the level of the left ventral hemithorax. His blood pressure was 110/70 mm Hg. He had normal heart sounds. Laboratory examination was completely normal. Plain chest radiograph showed a large mass in the upper part of the mediastinum (Fig 1). Spirometry and flexible bronchoscopy revealed no abnormalities. Computed tomography scanning of the thorax showed a large circumscribed tumor with focal calcification in the antero-ventral mediastinum extending into the upper left hemithorax (Fig 2). Computed tomographic scanning of the cerebrum was normal. To exclude germ cell tumor, thyroidal disorder, and thymoma, further blood examination was conducted. The additional tests included 
-fetoprotein, ß-human chorion gonadotrophin, thyroid function screening, and antibodies against acetylcholine, which were all within normal levels. It was decided not to perform a biopsy because of possible dissemination of cancer cells in case of malignancy and because the patient's complaints necessitated a surgical resection anyway.
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Fig 2. Computed tomographic scan of the thorax showing a large circumscribed tumor with focal calcification in the antero-ventral mediastinum extending into the upper left hemithorax.
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The patient electively underwent a left thoracotomy. The tumor was easily dissected free from the surrounding structures (ie, the aorta, pulmonary vessels, vagal nerve, trachea, thymus, and pericardium) and was subsequently resected. Macroscopically, the tumor seemed radically removed. The postoperative course was uneventful and the patient was discharged home 6 days after the operation.
Macroscopic examination revealed a spotty brownish tumor with a rubbery consistency and fibrous structure (Fig 3). Its size was 19 x 15 x 9 cm. The tumor was well circumscribed with fatty tissue and a thin fibrous coat. Microscopically, conglomerations of confluencing vessel lumina partly filled with blood were seen (Fig 4). The interstitial stroma mostly contained smooth muscle cells and focal fat cells. In addition, some lymphocyte foci and diffuse plasma cells were detected in the stroma. No signs of malignancy were noted. These findings were consistent with angiomyolipoma.
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Fig 4. Photomicrograph of the angiomyolipoma with conglomerations of confluencing vessel lumina partly filled with blood (right) and interstitial stroma containing mostly smooth muscle cells and focal fat cells (left). (Hematoxylin & eosin; x100 original magnification.)
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Comment
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The anterior mediastinal compartment is located anterior to the pericardium and includes lymphatic tissue, the thymus, the extrapericardial aorta and its branches, as well as the greater veins. Masses in the anterior compartment are more likely to be malignant than those found in the other mediastinal compartments. Lesions most commonly found in the former location are thymomas, germ cell tumors, lymphomas, intrathoracic thyroid tissue, and parathyroid lesions [8].
Angiomyolipomas are rare, benign mesenchymal tumors usually found in the kidney. Extrarenal manifestation of angiomyolipoma is rare. The most frequently reported extrarenal location is the liver, but sporadic presentations in other tracts have also been described, such as the skin, oral cavity, the abdominal wall, retroperitoneum, heart, lung, gastrointestinal tract, gynecologic tract, penis, and spinal vertebrae. Only 6 patients with mediastinal localization of angiomyolipoma have appeared in the literature so far (Table 1). Only one of these six tumors was located in the anterior mediastinum, as was the case in the patient described in this article. Including our patient, the male to female ratio was 2:5, with a mean age of 45 years. Four patients were asymptomatic on admission, two suffered exertional dyspnea and intermittent cough, and one had intermittent palpitations and dysrhythmias. Diagnostic tools that were used included plain chest radiograph (100%), computed tomographic scanning (86%), magnetic resonance imaging (57%), and angiography (29%). Six of 7 patients were treated by thoracotomy and resection of the tumor. In 1 patient, interruption of blood flow in the tumor was achieved by repeated arterial embolization after which the tumor became organized and was diminished in size [5]. All patients survived the procedure, but no long-term outcome was given for any of the patients.
In conclusion, although rare, angiomyolipoma should be considered in the differential diagnosis of a mediastinal tumor.
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References
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- Dickinson M, Ruckle H, Beaghler M, Hadley HR. Renal angiomyolipoma: optimal treatment based on size and symptoms. Clin Nephrol. 1998;49:281–286[Medline]
- Bertrand G, Bidabe MC, George P, Dubin P, Touzard C. Angiomyolipoma of the central mediastinum: an apparently undescribed entity. Ann Chir. 1984;38:679–681[Medline]
- Fukuzawa J, Shimizu T, Sakai E, et al. Case report of angiomyolipoma of the posterior upper mediastinum. Nihon Kyobu Shikkan Gakkai Zasshi. 1992;30:464–467[Medline]
- Hayashi K, Yamamoto M, Nishimura H, Inou N. Angiomyolipoma of the anterior mediastinum: a case report. Nippon Kyobu Geka Gakkai Zasshi. 1994;42:584–587[Medline]
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Abstract
Introduction
