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J Thorac Cardiovasc Surg 2004;128:615-617
© 2004 The American Association for Thoracic Surgery
Brief communication |
a First Department of Surgery, Hamamatsu University School of Medicine, Hamamatsu, Japan
Received for publication January 5, 2004; accepted for publication January 27, 2004.
* Address for reprints: Katsuyuki Asai, MD, Department of Thoracic Surgery, Numazu City Hospital, 550 Harunoki Higashi-shiiji, Numazu, Shizuoka 410-0302, Japan
asaik@msc.biglobe.ne.jp
| The first 20% of the full text of this article appears below. |
We report a case of mediastinal malignant peripheral nerve sheath tumor (MPNST) in a patient with neurofibromatosis type 1 (NF-1, von Recklinghausen disease). The tumor had a complex involvement with mediastinal structures and was resected by a temporary aortic transection approach under cardiopulmonary bypass (CPB).
Clinical summary
A 40-year-old man with NF-1 was referred to our hospital because of hoarseness and enlargement of an abnormal shadow on his chest radiograph. On admission to our hospital, chest computed tomographic (CT) scan showed multiple masses in the mediastinum and chest wall. The largest one, measuring 12 x 9 x 8 cm, was centered in the aortopulmonary window, encasing the ascending aorta and compressing the right pulmonary artery, left atrium, and left main bronchus (Figure 1). Compared with his chest CT 4 years previously, this mediastinal lesion showed marked growth, whereas the other masses had remained unchanged in size. A CT-guided needle biopsy revealed MPNST immunohistochemically positive for S-100 protein. We initially chose
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