Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

  • Takehiko Shimoyama
  • Katsuo Yoshiya
  • Yasushi Yamato
  • Teruaki Koike
  • Keiichi Honma
Case Report


Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.

Key words

Malignant peripheral nerve sheath tumor Mediastinum Metastasis 


  1. 1.
    Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 1986;57:2006–2021.PubMedCrossRefGoogle Scholar
  2. 2.
    Lai RS, Lin SL, Hsu SS, Wu MT. Intrathoracic paraspinal malignant peripheral nerve sheath tumor. J Chin Med Assoc 2006;69:37–41.PubMedCrossRefGoogle Scholar
  3. 3.
    Kourea HP, Bilsky MH, Leung DH, Lewis JJ, Woodruff JM. Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: a clinicopathologic study of 25 patients and 26 tumors. Cancer 1998;11:2191–2203.CrossRefGoogle Scholar
  4. 4.
    Zisis C, Fragoulis S, Rontogianni D, Stratakos G, Bellenis I. Malignant triton tumour of the anterior mediastinum as incidental finding. Monaldi Arch Chest Dis 2006;65:222–224.PubMedGoogle Scholar
  5. 5.
    Shijubo N, Sugaya F, Imada A, Fujishima T, Nakata H, Satoh M, et al. Malignant peripheral nerve sheath tumor of the mediastinum. Respiration 2000;67:346–347.PubMedCrossRefGoogle Scholar
  6. 6.
    Fukai I, Masaoka A, Yamakawa Y, Niwa H, Eimoto T. Mediastinal malignant epithelioid schwannoma. Chest 1995; 108:574–575.PubMedCrossRefGoogle Scholar
  7. 7.
    Lang-Lazdunski L, Pons F, Jancovici R. Malignant “triton” tumor of the posterior mediastinum: prolonged survival after staged resection. Ann Thorac Surg 2003;75:1645164–1645168.Google Scholar
  8. 8.
    Shoji F, Maruyama R, Okamoto T, Wataya H, Nishiyama K, Ichinose Y. Malignant schwannoma of the upper mediastinum originating from the vagus nerve. World J Surg Oncol 2005;65: online.Google Scholar
  9. 9.
    Asai K, Suzuki K, Shimota H, Takahashi T, Yamashita K, Kazui T. Malignant peripheral nerve sheath tumor of the mediastinum: a temporary aortic transection approach. J Thorac Cardiovasc Surg 2004;128:615–617.PubMedCrossRefGoogle Scholar
  10. 10.
    Ingels GW, Campbell DC, Giampetro AM, Kozub RE, Bentlage CH. Malignant schwannomas of the mediastinum: report of two cases and review of the literature. Cancer 1971; 27:1190–1201.PubMedCrossRefGoogle Scholar

Copyright information

© The Japanese Association for Thoracic Surgery 2009

Authors and Affiliations

  • Takehiko Shimoyama
    • 1
  • Katsuo Yoshiya
    • 1
  • Yasushi Yamato
    • 1
  • Teruaki Koike
    • 1
  • Keiichi Honma
    • 2
  1. 1.Division of Chest SurgeryCancer Center of Niigata HospitalNiigataJapan
  2. 2.Division of PathologyCancer Center of Niigata HospitalNiigataJapan

Personalised recommendations