Ann Thorac Surg 2006;82:1504-1506
© 2006 The Society of Thoracic Surgeons
Case Reports
Pulmonary Angiomyolipoma
Bertrand Marcheix, MDa,
Laurent Brouchet, MDa,
Yoan Lamarche, MDb,
Claire Renaud, MDa,
Anne Gomez-Brouchet, MDc,
Lucy Hollington, MDe,
Valérie Chabbert, MDd,
John Berjaud, MDa,
Marcel Dahan, MDa,*
a Department of Thoracic Surgery, Rangueil-Larrey University Hospital, Toulouse, France
b Department of Cardiovascular Surgery, Montreal Heart Institute, Montreal, Quebec, Canada
c Department of Pathology, Rangueil University Hospital, Toulouse, France
d Department of Radiology, Rangueil University Hospital, Toulouse, France
e Department of Cardiology, Montauban General Hospital, Montauban, France
Accepted for publication February 3, 2006.
* Address correspondence to Dr Dahan, Department of Thoracic Surgery, Rangueil-Larrey University Hospital, TSA 300 30, 24, chemin de Pouvourville, Toulouse, Cedex 9, 31059 France (Email: dahan.m{at}chu-toulouse.fr).
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Abstract
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Extrarenal angiomyolipoma are benign lesions that have rarely been described in the thorax. We present the clinical, radiographic, and pathologic findings of a pulmonary angiomyolipoma in a 63-year-old woman who had no diagnosis of tuberous sclerosis or lymphangioleiomyomatosis. We believe that this report is one of the first descriptions of angiomyolipoma of the lung.
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Introduction
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Solitary pulmonary nodules are frequently identified on routine chest roentgenogram. Despite technical advances, such as fine-needle aspiration and fluorodeoxyglucose–positron emission tomographic scan, identifying a solitary, pulmonary nodule remains a diagnostic challenge to the thoracic surgeon. We present the clinical, radiographic, and pathologic findings of a pulmonary angiomyolipoma. We believe that only four other cases have been reported in the literature.
A 63-year-old woman was referred to our institution because of an abnormal chest roentgenogram that revealed a nodule in the right lower lobe of the lung (Fig 1a). A computed tomographic scan confirmed the presence of a proximal nonenhancing nodule measuring 23 x 20 mm, with fat-like density and no calcifications (Fig1b–1e). Review of prior chest roentgenograms and computed tomographic scans from April 2004 to June 2005 showed a progressive increase in size. An fluorodeoxyglucose–positron emission tomographic scan was performed that showed an isolated fixation at the level of the pulmonary nodule (Fig 1f–1h). Our patient refused fine-needle aspiration. As spirometry and pulmonary scintigraphy were compatible with surgical modalities, the patient was electively operated on using a posterolateral thoracotomy. Because of the proximal location of the nodule, which prevented the use of a wedge resection or enucleation, a right lower lobectomy was performed. The postoperative course was uneventful. The patient was discharged on postoperative day 5. Six months later the patient was asymptomatic.
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Fig 1. (a) Anteroposterior chest roentgenogram demonstrating a nodular opacity in the inferior region without any calcifications (arrow). Computed tomographic scan of the axial view with (b) parenchymal settings and (c–e) mediastinal settings. (e) Magnified view 1 minute after the injection of iodine contrast. The lesion (28 x 23 mm) appears composite and heterogeneous before and after the contrast media injection. Fatty densities (–40 Hounsfield Unit) were measured in the macronodule. The other component showed strong enhancement 1 minute after the injection. (f) coronal, (g) sagittal, and (h) axial positron emission tomographic–computed tomographic scan showing a solitary focus of intense tracer location in the right lower lobe.
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The pathologic findings (Fig 2a–2d) include an uncapsuled nodule that was composed of mature fat associated with numerous thick-walled blood vessels and smooth-muscle cells. All vessels lacked an internal elastic lamina. Mitotic figures and necrosis were absent. Diagnosis was confirmed by immunohistochemistry studies. Most cells expressed H Caldesmone, desmine, and HMB45. Other markers including CD 34, CD 31, cytokeratine, EMA, and PS 100 were negative.
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Fig 2. (a) Pulmonary tumor, which is well-delineated with a thin fibrous pseudocapsule. (Hematoxylin and eosin; x20.) (b) Three components: mature adipose tissue, mature smooth muscle arranged in short fascicles, and medium caliber thick-walled blood vessels. No appreciable mitotic activity. No necrosis. (Hematoxylin and eosin; x150.) (c) Identical immunopositivity for H Caldesmone and Desmine on tumor cells. (Hematoxylin and eosin; x150.) (D) Focal staining of tumor cells with the HMB45 antibody. (Hematoxylin and eosin; x150.)
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Comment
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In the United States, approximately 150,000 solitary pulmonary nodules are identified each year [1, 2]. Most patients facing this situation expect a surgeon to provide them with a clear diagnosis. In some cases, technical advances such as fine-needle aspiration, cytologic diagnosis, or a positron emission tomographic scan may help the practitioner to clarify the diagnosis. However, in some cases as with the patient described herein, these modalities may have limitations and surgery may remain the only way to make a safe and precise diagnosis.
Angiomyolipomas are rare, mesenchymal tumors that are composed of fat, smooth muscle and thick-walled vessels. Most angiomyolipomas occur in the kidney. Approximately 50% of cases occur in patients with tuberous sclerosis [3–6]. Occurrence in extrarenal sites has rarely been reported. Other locations include the skin [7], liver, abdominal wall, retroperitoneum, uterus, oral cavity, penis, spermatic cord, fallopian tube, vagina, and mediastinum [8]. In this report we identify one case of angiomyolipoma of the lung. The patient had no history of prior angiomyolipomas and did not present any stigmata of tuberous sclerosis. We believe that only four pulmonary angiomyolipomas have been previously reported [9–12]. In contrast to renal angiomyolipomas, this case and a review of previous reports, reveal that extrarenal angiomyolipomas seem to be well-delineated, easily resectable, and not associated with tuberous sclerosis. Distinction from other benign and malignant pulmonary, mesenchymal lesions depends on traditional histologic criteria.
In conclusion, according to the literature, in contrast to renal angiomyolipomas, which are often invasive and may involve regional nodes that may recur, extrarenal angiomyolipomas are most often solitary, noninvasive, and rarely associated with tuberous sclerosis, and are most often easily resectable lesions. In cases of a proximal pulmonary location, a lobectomy has to be performed.
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References
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- Lillington G. Managment of solitary pulmonary nodules Dis Mon 1991;37:271-318.[Medline]
- Stoller JK, Ahmad M, Tice TW. Solitary pulmonary nodule Cleve Clin J Med 1988;55:68-74.[Medline]
- Farrow GM, Harrison EG, Utz DC, Jones DR. Renal angiomyolipomaA clinicopathologic study of 32 cases. Cancer 1968;22:564-570.[Medline]
- Lack EE, Dolan MF, Finisio J, Grover G, Singh M, Triche TJ. Pulmonary and extrapulmonary lymphangioleiomyomatosisReport of a case with bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis, and glial polyp of the endocervix. Am J Surg Pathol 1986;10:650-657.[Medline]
- Monteforte WJ, Kohnen PW. Angiomyolipomas in a case of lymphangiomyomatosis syndrome: relationships to tuberous sclerosis Cancer 1974;34:317-321.[Medline]
- Taylor RS, Joseph DB, Kohaut EC, Wilson ER, Bueschen AJ. Renal angiomyolipoma associated with lymph node involvment and renal cell carcinoma in patients with tuberous sclerosis J Urol 1989;141:930-932.[Medline]
- Argenyi EB, Piette WW, Goeken JA. Cutaneous angiomyolipomaA light-microscopic, immunohistochemical, and electron-microscopic study. Am J Dermatopathol 1991;13:497-502.[Medline]
- Amir AMI, Zeebregts CJ, Mulder HJ. Anterior mediastinal presentation of a giant angiomyolipoma Ann Thorac Surg 2004;78:2161-2163.[Abstract/Free Full Text]
- Papla B, Malinowski E. Angiomyolipoma of the lung Pol J Pathol 1999;50:47-50.[Medline]
- Guinee DG, Thornberry DS, Azumi N, Przygodzki RM, Koss MN, Travis WD. Unique pulmonary presentation of an angiomyolipoma Am J Surg Pathol 1995;19:476-480.[Medline]
- Gloeckner-Hofmann K, Krismann M, Feller AC. Angiomyolipom der Lunge Pathologe 2000;21:260-263.[Medline]
- Ito M, Sugamura Y, Ikari H, Sekine I. Angiomyolipoma of the lung Arch Pathol Lab Med 1998;122:1023-1025.[Medline]
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Abstract
Introduction
