Summary
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon sarcoma in the pediatric population; however, its presence should be considered in a child with an enlarging or painful soft-tissue mass. Diagnosis of this neoplasm depends on either the demonstration of its origin within a peripheral nerve or the association with a contiguous neurofibroma. We have identified 16 cases of MPNST involving children 16 years of age or less, which represent 12.8% of the total cases seen at the Mayo Clinic. Most of the lesions arose in children with von Recklinghausen's disease and were associated with a contiguous neurofibromatous component. The mean survival of patients who were known to have died of tumor was only 1.8 years. This sarcoma requires prompt aggressive therapy utilizing wide surgical excision. Because of the association of MPNST with von Recklinghausen's neurofibromatosis, a careful workup and family history should be obtained for the potential prognostic value and for the purpose of genetic counseling.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Enzinger FM, Weiss SW: Soft tissue tumors. CV Mosby Company, St. Louis, 1983, pp 625–654.
Riccardi VM: Von Recklinghausen neurofibromatosis. N Engl J Med 305:1617–1627, 1981.
Crowe FW, Schull WJ, Neel JV: A clinical, pathological, and genetic study of multiple neurofibromatosis. Charles C Thomas, Publisher, Springfield, Illinois, 1956.
Preston FW, Walsh WS, Clarke TH: Cutaneous neurofibromatosis (von Recklinghausen's disease): clinical manifestations and incidence of sarcoma in sixty-one male patients. Arch Surg 64:813–827, 1952.
Brasfield RD, Das Gupta TK: Von Recklinghausen's disease: a clinicopathological study. Ann Surg 175:86–104, 1972.
Guccion JG, Enzinger FM: Malignant schwannoma associated with von Recklinghausen's neurofibromatosis. Virchows Arch [Pathol Anat] 383:43–57, 1979.
D'Agostino AN, Soule EH, Miller RH: Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (von Recklinghausen's disease). Cancer 16:1015–1027, 1963.
Ducatman BS, Scheithauer BW: Postirradiation neurofibrosarcoma. Cancer 51:1028–1033, 1983.
Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB, Beattie EJ: Malignant schwannoma: clinical characteristics, survival, and response to therapy. Cancer 47: 2503–2509, 1981.
Ghosh BC, Ghosh L, Huvos AG, Fortner JG: Malignant schwannoma: a clinicopathologic study. Cancer 31: 184–190, 1973.
Das Gupta TK, Brasfield RD: Solitary malignant schwannoma. Ann Surg 171:419–428, 1970.
Taxy JB, Battifora H, Trujillo Y, Dorfman HD: Electron microscopy in the diagnosis of malignant schwannoma. Cancer 48:1381–1391, 1981.
Stefansson K, Wollmann R, Jerkovic M: S-100 protein in soft-tissue tumors derived from Schwann cells and melanocytes. Am J Pathol 106:261–268, 1982.
Woodruff JM, Chernik NL, Smith MC, Millett WB, Foote FW Jr: Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant ‘Triton’ tumors). Cancer 32:426–439, 1973.
Woodruff JM: Peripheral nerve tumors showing glandular differentiation (glandular schwannomas). Cancer 37:2399–2413, 1976.
Krumerman MS, Stingle W: Synchronous malignant glandular schwannomas in congenital neurofibromatosis. Cancer 41:2444–2451, 1978.
Vieta JO, Pack GT: Malignant neurilemonas of peripheral nerves. Am J Surg 82:416–431, 1951.
White HR Jr: Survival in malignant schwannoma: an 18-year study. Cancer 27:720–729, 1971.
Foley KM, Woodruff JM, Ellis FT, Posner JB: Radiation-induced malignant and atypical peripheral nerve sheath tumors. Ann Neurol 7:311–318, 1980.
Bergström R: Changes in peripheral nerve tissue after irradiation with high energy protons. Acta Radiol (Stockh) 58:301–312, 1962.
Cavanagh JB: Effects of X-irradiation on the proliferation of cells in peripheral nerve during Wallerian degeneration in the rat. Br J Radiol 41:275–281, 1968.
Hope DG, Mulvihill JJ: Malignancy in neurofibromatosis. Adv Neurol 29:33–55, 1981.
D'Agostino AN, Soule EH, Miller RH: Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease). Cancer 16:1003–1014, 1963.
Goldman RL, Jones SE, Heusinkveld RS: Combination chemotherapy of metastatic malignant schwannoma with vincristine, Adriamycin, cyclophosphamide, and imidazole carboxamide: a case report. Cancer 39:1955–1958, 1977.
Ducatman BS, Scheithauer BW: Malignant peripheral nerve sheath tumors with divergent differentiation. Cancer (in press).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ducatman, B.S., Scheithauer, B.W., Piepgras, D.G. et al. Malignant peripheral nerve sheath tumors in childhood. J Neuro-Oncol 2, 241–248 (1984). https://doi.org/10.1007/BF00253276
Issue Date:
DOI: https://doi.org/10.1007/BF00253276