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

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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.

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Authors and Affiliations

  1. the Department of Pathology, St. Jude Children's Research Hospital, USA

    David M. Parham MD

  2. the Department of Hematology-Oncology, St. Jude Children's Research Hospital, USA

    Laura Bowman MD & Alberto S. Pappo MD

  3. the Department of Radiation Oncology, St. Jude Children's Research Hospital, USA

    James Fontanesi MD

  4. the Department of Surgery, the University of Tennessee, Memphis, Tennessee, U.S.A.

    James M. DeCou MD,  Bhaskar N. Rao MD & Thom E Lobe MD

  5. the Department of Pathology, the University of Tennessee, Memphis, Tennessee, U.S.A.

    David M. Parham MD

  6. the Department of Pediatrics, the University of Tennessee, Memphis, Tennessee, U.S.A.

    Laura Bowman MD & Alberto S. Pappo MD

  7. the Department of Radiation Oncology, the University of Tennessee, Memphis, Tennessee, U.S.A.

    James Fontanesi MD

  8. Rao, Department of Surgery, St. Jude Children's Research Hospital, 332 N. Lauderdale, Memphis, 38101, TN, U.S.A.

    James M. DeCou MD,  Bhaskar N. Rao MD & Thom E Lobe MD

Authors
  1. James M. DeCou MD
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  2. Bhaskar N. Rao MD
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  3. David M. Parham MD
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  4. Thom E Lobe MD
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  5. Laura Bowman MD
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  6. Alberto S. Pappo MD
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  7. James Fontanesi MD
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DeCou, J.M., Rao, B.N., Parham, D.M. et al. Malignant peripheral nerve sheath tumors: The St. Jude Children's Research Hospital experience. Annals of Surgical Oncology 2, 524–529 (1995). https://doi.org/10.1007/BF02307086

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  • DOI: https://doi.org/10.1007/BF02307086

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