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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References
Rao BN. Nonrhabdomyosarcoma in children: prognostic factors influencing survival.Semin Surg Oncol 1993;9:524–31.
Trojanowski JQ, Klienman GM, Proppe KH. Malignant tumors of nerve sheath origin.Cancer 1980;46:1202–12.
Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumors of the buttock and lower extremity: a study of 43 cases.Cancer 1990;66:1253–65.
Wanebo JE, Malik JM, VandenBerg SR, Wanebo HJ, Drieson N, Persing JA. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 28 cases.Cancer 1993;71:1247–53.
Storm FK, Eilber FR, Mirra J, Morton DL. Neurofibrosarcoma.Cancer 1980;45:126–9.
Ducatman BS, Scheitnauer BW, Peipgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases.Cancer 1986;57:2006–21.
Bruner JM. Peripheral nerve sheath tumors of the head and neck.Semin Diagn Pathol 1987;4:136–49.
Maurer HM, Moon T, Donaldson S, Fernandez C, Gehan EA, Hammond D, Hays DM, et al. The Intergroup Rhabdomyosarcoma Study: a preliminary report.Cancer 1977;40:2015–26.
Rao BN, Santana VM, Parham D, Pratt CB, Fleming ID, Hudson M, Fontanesi J, et al. Pediatric nonrhabdomyosarcomas of the extremities: influence of size, invasiveness, and grade on outcome.Arch Surg 1991;126:1490–5.
Ricci A, Parham DM, Woodruff JM, Callihan T, Green A, Erlandson RA. Malignant peripheral nerve sheath tumors arising from ganglioneuromas.Am J Surg Pathol 1984;8:19–29.
Coffin CM, Dehner LP. Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases.Pediatr Pathol 1989;9:387–407.
Ghosh BC, Ghosh L, Huvos AG, Fortner JG. Malignant schwannoma: a clinicopathologic study.Cancer 1973;31:184–90.
Sordillo PP, Helson L, Hadju SI, Magill GB, Kosloff C, Golbey RB, Beattie EJ. Malignant schwannoma—clinical characteristics, survival, and response to therapy.Cancer 1981;47:2503–9.
Raney B, Schnaufer L, Ziegler M, Chatten J, Littman P, Jarrett P. Treatment of children with neurogenic sarcoma: experience at the Children's Hospital of Philadelphia.Cancer 1987;59:1–5.
Hajdu SI. Peripheral nerve sheath tumors: histogenesis, classification, and prognosis.Cancer 1993;72:3549–52.
Newbould MJ, Wilkinson N, Mene A. Post-radiation malignant peripheral nerve sheath tumour: a report of two cases.Histopathology 1990;17:263–5.
Woodruff JM, Chernik NL, Smith MC, Millett WB, Foote FW. Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “triton” tumors).Cancer 1973;32:426–39.
Brooks JS, Freeman M, Enterline HT. Malignant “triton” tumors: natural history and immunohistochemistry of nine new cases with literature review.Cancer 1985;55:2543–9.
Dewit L, Albus-Lutter CE, DeJong A, Voute PA. Malignant schwannoma with a rhabdomyoblastic component, a socalled triton tumor: a clinicopathologic study.Cancer 1986;58:1350–6.
Woodruff JM, Christensen WN. Glandular peripheral nerve sheath tumors.Cancer 1993;72:3618–28.
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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