Abstract
Malignant peripheral nerve sheath tumor (MPNST) is rare, but its association with neurofibromatosis type 1 (NF1) is well known. A 56-year-old man with NF1 was referred to our hospital for investigation of dyspnea. Computed tomography showed a huge mass occupying the right thorax and invading the inferior vena cava. A salvage resection was performed to alleviate the developing hypoxemia. Histological examination confirmed an MPNST. The tumor regrew rapidly and the patient died 6 weeks after the resection. This case report reinforces the importance of monitoring patients with NF1 carefully because of their increased risk of the development of malignant neoplasms.
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Reynolds RM, Browning GGP, Nawroz I, Campbell IW. Von Recklinghausen’s neurofibromatosis: neurofibromatosis type1. Lancet 2003;361:1552–1554.
DeVita VT Jr, Hellman S, Rosenberg SA, editors. Cancer; principles & practice of oncology. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2001.
Miura T, Kawano Y, Chujo M, Miyawaki M, Mori H, Kawahara K. Spontaneous hemothorax in patients with von Recklinghausen’s disease. Jpn J Thorac Cardiovasc Surg 2005;53:649–652.
Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. Cancer 1986;57:2006–2021.
Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumors of the buttock and lower extremity. Cancer 1990;66:1253–65.
Evans DGR, Baser ME, McGaufhan J, Howard E, Moran A. Malignant peripheral nerve sheath tumors in neurofibromatosis 1. J Med Genet 2002;39:311–314.
Pediatric malignant peripheral nerve sheath tumor: the Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005;23:8422–8430.
Asai K, Suzuki K, Shimota H, Takahashi T, Yamashita K, Kazui T. Malignant peripheral nerve sheath tumor of the mediastinum: A temporary aortic transaction approach. J Thorac Cardiovasc Surg 2004;128:615–617.
Kodama H, Iihara M, Okamoto T, Obara T. Water-clear cell parathyroid adenoma causing primary hyperparathyroidism in a patient with neurofibromatosis type 1: report of a case. Surg Today 2007;37(10):884–887.
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Kawachi, R., Takei, H., Furuyashiki, G. et al. A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: Report of a case. Surg Today 38, 945–947 (2008). https://doi.org/10.1007/s00595-008-3764-0
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DOI: https://doi.org/10.1007/s00595-008-3764-0