Surgery Today

, Volume 38, Issue 10, pp 945–947 | Cite as

A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: Report of a case

  • Riken Kawachi
  • Hidefumi Takei
  • Goh Furuyashiki
  • Yoshihiko Koshi-Ishi
  • Tomoyuki Goya
Case Report

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.

Key words

Malignant peripheral nerve sheath tumor Neurofibromatosis type 1 Von Recklinghausen’s disease Mediastinal tumor 

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References

  1. 1.
    Reynolds RM, Browning GGP, Nawroz I, Campbell IW. Von Recklinghausen’s neurofibromatosis: neurofibromatosis type1. Lancet 2003;361:1552–1554.PubMedCrossRefGoogle Scholar
  2. 2.
    DeVita VT Jr, Hellman S, Rosenberg SA, editors. Cancer; principles & practice of oncology. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2001.Google Scholar
  3. 3.
    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.PubMedCrossRefGoogle Scholar
  4. 4.
    Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. Cancer 1986;57:2006–2021.PubMedCrossRefGoogle Scholar
  5. 5.
    Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumors of the buttock and lower extremity. Cancer 1990;66:1253–65.PubMedCrossRefGoogle Scholar
  6. 6.
    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.PubMedCrossRefGoogle Scholar
  7. 7.
    Pediatric malignant peripheral nerve sheath tumor: the Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005;23:8422–8430.Google Scholar
  8. 8.
    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.PubMedCrossRefGoogle Scholar
  9. 9.
    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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2008

Authors and Affiliations

  • Riken Kawachi
    • 1
  • Hidefumi Takei
    • 1
  • Goh Furuyashiki
    • 1
  • Yoshihiko Koshi-Ishi
    • 1
  • Tomoyuki Goya
    • 1
  1. 1.General Thoracic Surgery Division, Department of SurgeryKyorin University School of MedicineTokyoJapan

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