Annals of Surgical Oncology

, Volume 2, Issue 6, pp 524–529 | Cite as

Malignant peripheral nerve sheath tumors: The St. Jude Children's Research Hospital experience

  • James M. DeCou
  • Bhaskar N. Rao
  • David M. Parham
  • Thom E Lobe
  • Laura Bowman
  • Alberto S. Pappo
  • James Fontanesi
Original Articles

Abstract

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in young patients. To contribute to the understanding of these tumors, we reviewed the records of all patients treated for PNSTs at one institution over a 30-year period.

Methods: We reviewed the records of eight patients treated for benign PNSTs and 28 patients treated for 29 MPNSTs. We focused on the latter group, statistically testing several clinical factors for their significance in affecting survival.

Results: Five-year survival in patients with MPNSTs was 39%. The most significant prognostic factor was gross tumor resectability (p=0.0004). Five-year survival for patients with resectable tumors was 65%, whereas no patient with unresectable disease survived >25 months. Tumor grade, site, and patient race were also significant factors by univariate analysis but were not significant when adjusted for resectability.

Conclusion: Gross tumor resection is crucial in treating malignant PNSTs. Supplemental radiation therapy is recommended for positive microscopic margins. More effective treatment is still being sought for unresectable disease.

Key Words

Malignant peripheral nerve sheath tumors Pediatric 

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Copyright information

© The Society of Surgical Oncology, Inc 1995

Authors and Affiliations

  • James M. DeCou
    • 8
    • 4
  • Bhaskar N. Rao
    • 8
    • 4
  • David M. Parham
    • 1
    • 5
  • Thom E Lobe
    • 8
    • 4
  • Laura Bowman
    • 2
    • 6
  • Alberto S. Pappo
    • 2
    • 6
  • James Fontanesi
    • 3
    • 7
  1. 1.the Department of PathologySt. Jude Children's Research HospitalUSA
  2. 2.the Department of Hematology-OncologySt. Jude Children's Research HospitalUSA
  3. 3.the Department of Radiation OncologySt. Jude Children's Research HospitalUSA
  4. 4.the Department of Surgerythe University of TennesseeMemphisU.S.A.
  5. 5.the Department of Pathologythe University of TennesseeMemphisU.S.A.
  6. 6.the Department of Pediatricsthe University of TennesseeMemphisU.S.A.
  7. 7.the Department of Radiation Oncologythe University of Tennessee, Memphis, TennesseeU.S.A.
  8. 8.Rao, Department of SurgerySt. Jude Children's Research Hospital, 332 N. Lauderdale, MemphisU.S.A.

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