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Ann Thorac Surg 2004;77:1841-1843
© 2004 The Society of Thoracic Surgeons


Case report

Primary lung tumor mimicking acute type A aortic dissection

Uday Sonker, FRCS(I), FRCS(Ed)a*, Dirk F. P. M. Peek, MDa, Jacoba J. C. van Der Meij, MDb, Robin H. Heijmen, MD, PhDa, Cornelis A. Seldenrijk, MD, PhDb, Paul J. Knaepen, MDa

a Cardiothoracic Surgery, St. Antonius Hospital, Nieuwegein, The Netherlands,
b Department of Pathology, St. Antonius Hospital, Nieuwegein, The Netherlands

Accepted for publication May 12, 2003.

* Address reprint requests to Dr Sonker, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands
e-mail: usonker{at}hotmail.com


    Abstract
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A 64-year-old man presented with clinical features and echocardiographic diagnosis of an acute type A dissection. He underwent median sternotomy for definitive surgical treatment. On external examination of the aorta, other intrapericardial structures, and the right lung, it was evident that the patient had an advanced lung tumor. This was confirmed by frozen-section and histopathologic examinations. Epiaortic scanning showed beyond doubt the presence of a mobile intraaortic mass that had misled us in making the preoperative diagnosis of an acute type A dissection.


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Acute type A aortic dissection is a life-threatening condition that necessitates immediate surgical intervention. Clinical features of acute type A dissection are acute-onset interscapular pain, shock of varying degrees due to cardiac tamponade or massive aortic insufficiency, and neurologic deficits [1, 2]. A plain chest roentgenogram may demonstrate a widened mediastinum. Transthoracic or transesophageal ultrasound, computed tomography, or magnetic resonance imaging (MRI) can identify an intraluminal dissection flap [3]. Surgical treatment consists of ascending aortic replacement with or without aortic valve replacement, depending on the extent of the dissection. We present a case of a patient referred to us with a high suspicion of acute type A dissection in whom, unexpectedly, a primary pulmonary tumor with invasion of the pericardium and superior vena cava and a mass (probably tumor) inside the ascending aorta were seen during operation.

A 64-year-old man was admitted to the emergency room of a peripheral center in shock with a 1-day history of increased temperature, sweating, and diminished consciousness. His medical history included a nonproductive cough of 6 weeks' duration and depression. Physical examination revealed signs of shock, absent right radial artery pulsations, congested neck veins, and right hemiplegia. The chest roentgenogram showed cardiomegaly, widened mediastinum, and patchy infiltrates in the right mid and lower zones (Fig 1). Laboratory test showed normal hemoglobin, mild leucocytosis, and mildly increased creatinine. A transthoracic echocardiogram revealed massive pericardial effusion and a mobile intraaortic flap. Blood-stained fluid was obtained on pericardiocentesis. The patient was thus transferred to our hospital with a diagnosis of an acute type A aortic dissection. In accordance with our protocol the patient was transferred to the operating theater, and a transesophageal echocardiogram was performed while he was under anesthesia; it revealed pericardial fluid (1.5 cm), a swinging heart, and an intraaortic mobile mass located 15 mm distal to the aortic valve (Fig 2). No insufficiency of the aortic valve or dilatation of the ascending aortic artery was observed. During the operation we found a large amount of hemorrhagic pericardial fluid, which was sent for cytologic evaluation. There was no dilatation or hemorrhagic discoloration of the ascending aorta. In the region of the aortic root multiple reddish granular areas were visible that extended laterally toward the superior vena cava. Samples were obtained from these areas for frozen-section examinations. Because of these conflicting intraoperative findings we decided to perform an intraoperative epiaortic ultrasound by using a 9-MHz probe (Site Rite II; Dymax Corporation, Pittsburgh, PA), which showed an abnormally thickened aortic wall and a mobile mass of 1 to 1.5 cm length in the aortic lumen (Fig 3). Frozen section results were reported as a malignant tumor, probably non–small cell carcinoma. The right pleura was opened and the right lung inspected. This showed multiple firm nodular areas of tumor that involved all lobes of the right lung. All frozen-section sample results were reported as malignant tumor, probably non–small cell carcinoma. Therefore it was decided to abandon further surgical treatment. A fenestration was created between the pericardium and the right pleural cavity for adequate drainage.



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Fig 1. Chest roentgenogram showing a broadened mediastinum and patchy infiltrates in the right mid and lower zones.

 


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Fig 2. Transesophageal echocardiogram. (Ao = aortic lumen; Ma = intraluminal mass; PE = pericardial effusion.)

 


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Fig 3. Epiaortic echocardiogram showing an intraluminal mass that mimicked a dissection flap. (Ao = aortic lumen; Ma = intraluminal mass.)

 
Cytologic analysis of the pleural fluid and histologic examination of the periaortic tissue, lung, and pericardium revealed an adenocarcinoma. Thyroid transcription factor-1 (TTF-1) immunohistochemical staining was positive. TTF-1 can be used to distinguish a primary thyroid or lung adenocarcinoma from an adenocarcinoma originating from other organs (positivity for TTF-1 is 98% to 100%, 75% to 80%, and 0% to 5%, respectively). Tests for specific markers of thyroid origin (calcitonin and thyroglobulin) were negative. The histologic appearance of the tumor, predominance of tumor growth in the thorax, presence of malignant pleural effusion, expression of TTF-1, and negative markers for thyroid made a primary adenocarcinoma of the lung the most likely diagnosis.

After operation, the patient was transferred to the referring hospital and died a few days later. No postmortem examination was conducted.


    Comment
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 Abstract
 Introduction
 Comment
 References
 
We present a previously unreported case of a lung tumor in an advanced stage that mimicked an acute type A aortic dissection. Despite the absence of interscapular pain, clinical signs and investigations suggested the presence of a type A dissection. The decision to perform emergency operation was made because of a visible dissection flap and pericardial effusion on the transthoracic and transesophageal echocardiogram. Unfortunately, neither computed tomography nor MRI was performed because of the unstable clinical condition of the patient [4]. At operation we found a pulmonary tumor with invasion of the pericardium and the vena cava and a mass in the aortic lumen, which mimicked a dissection flap. Tumor markers confirmed that this was a primary lung tumor [5]. Preoperative diagnostic modalities could be misleading. Spiral computed tomography, multiplanar transesophageal echocardiography, and MRI scan, which are the main diagnostic modalities, have reported specificities of 100%, 94%, and 94%, respectively. They are all 100% sensitive [3]. Esophageal sarcoma, torn aortic valve commissure, anomalous inferior vena cava, tortuous descending thoracic aorta, haustration of the ascending aorta, and superior pericardial recess have been reported as causes of misdiagnosis of type A dissection of the aorta. The origin of the aortic arch vessels, superior intercostal and pulmonary veins, residual thymus, atelectasis, pleural thickening, periaortic soft-tissue masses such as periaortic fibrosis or lymphoma, normal aortic sinuses, and congenital aortic diverticula may be difficult to distinguish from aortic dissection. These pitfalls in accurate diagnosis of type A dissection can be avoided by more extensive preoperative investigation with more than 1 diagnostic modality. The acute critical condition of the patient does not allow this very often. Therefore, the use of intraoperative epiaortic scanning in cases in which clear signs of dissection are absent may have potential value in distinguishing between intraluminal flap and linear artifacts, which are difficult to diagnose before operation [6].


    References
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 Abstract
 Introduction
 Comment
 References
 

  1. Kirklin J.W., Barratt Boyes B.G. Cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. , 2nd ed New York: Churchill Livingstone, 1993.
  2. Armstrong W.F., Bach D.S., Carey L.M., Froehlich J., Lowell M., Kazerooni E.A. Clinical and echocardiographic findings in patients with suspected acute aortic dissection. Am Heart J 1998;136:1051-1060.[Medline]
  3. Sommer T., Fehske W., Holzknecht N., et al. Aortic dissection: a comparative study of diagnosis with spiral CT, multiplanar transesophageal echocardiography and MRI imaging. Radiology 1996;199:347-352.[Abstract/Free Full Text]
  4. Nienaber C.A., von Kodolitsch Y., Nicolas V., et al. The diagnosis of thoracic aortic dissection by non-invasive imaging procedures. N Engl J Med 1993;328:1-9.[Abstract/Free Full Text]
  5. Lau S.K., Luthringer D.L., Eisen R.N. Thyroid transcription factor-1: a review. Appl Immunohistochem Mol Morphol 2002;10:97-102.[Medline]
  6. Vignon P., Spencer K.T., Rambaud G., et al. Differential transesophageal echocardiographic diagnosis between linear artifacts and intraluminal flap of aortic dissection or disruption. Chest 2001;119:1778-1790.[Abstract/Free Full Text]




This Article
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Robin H. Heijmen
Paul J. Knaepen
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