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Olusola Oduntan
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J Thorac Cardiovasc Surg 2006;131:736-737
© 2006 The American Association for Thoracic Surgery

Brief Communication

Intrathoracic peripheral nerve sheath tumors in patients with neurofibromatosis type 1 (von Recklinghausen disease)

Vijay S. Patel, MD * , James D. St Louis, MD, Olusola Oduntan, MD, Kevin P. Landolfo, MD

Department of Cardiothoracic and Vascular Surgery, the Medical College of Georgia, Augusta, Ga

Received for publication August 1, 2005; revisions received November 7, 2005; accepted for publication November 14, 2005.

* Address for reprints: Vijay S. Patel, MD, Medical College of Georgia, Department of Cardiothoracic and Vascular Surgery, 1120 15th St, BA-4300, Augusta, GA 30912 (Email: vijay_patel_us@yahoo.com).

The first 20% of the full text of this article appears below.

Intrathoracic neurogenic tumors comprise 15% to 25% of all primary mediastinal tumors in adults, and 95% originate within the posterior mediastinum. 1 Go In most, these are benign slow-growing schwannomas and neurofibromas of peripheral nerve origin. However, patients with neurofibromatosis type 1 (NF1) are particularly at risk for malignant transformation of these tumors and have a poor long-term prognosis. 2 Go

Clinical Summaries

We present 3 cases of intrathoracic peripheral nerve sheath tumors in patients with NF1 disease.

Patient 1
A 55-year-old man presented with a recent onset of dyspnea and a known posterior mediastinal mass diagnosed 20 years earlier. The mass had increased in size over the past 5 years. Chest radiography revealed a smooth shadow adjacent to the right upper thoracic spine, and computed tomographic (CT) scan confirmed a 4.3 x 3.4-cm low-attenuation mass occupying the right posterior mediastinum. Intraoperative bronchoscopy results were negative. He underwent a posterolateral thoracotomy with resection of a well-circumscribed 5 x 4 x 3-cm mass. The histopathologic diagnosis was neurofibroma. His postoperative course was uneventful, and he is doing well 15 . . . [Full Text of this Article]






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