Summary
Thirteen soft tissue neurogenic sarcomas from twelve patients with neurofibromatosis (Von Recklinghausen's disease) were ultrastructurally examined. Electron microscopic studies revealed a wide spectrum of morphological manifestations varying from schwannian to fibroblastic, histiocytic, fibrohistiocytic and relatively undifferentiated cellular proliferations. A similar variation on light microscopic appearances has been previously reported in these neurogenic sarcomas. Neurogenic sarcomas occurring in patients with neurofibromatosis (Von Recklinghausen's disease), represent a heterogenous group of neoplasms with various patterns of differentiation identified ultrastructurally. The morphologic expressions of these neurogenic neoplasms can be conceptualized as a disorderly growth of the various peripheral nerve cellular components, or, as has been previously suggested, as a result of the multipotential nature and metaplastic ability of Schwann cells. S-100 protein immunohistochemistry was only positive in those neoplasms ultrastructurally proven to represent schwannian cellular proliferations.
This study serves to document the range of fine structure that may be found in neurogenic sarcomas, to correlate the ultrastructural findings with the light microscopic appearance of these tumors, to determine the specificity of the electron microscopic findings, and immunohistochemistry for S-100 protein and assess their possible value in differential diagnosis.
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References
Bertrand I, Bernard R (1930) Degenerescence maligne d'une tumeur Schwannique du nerf radial dans un cas de maladie de Recklinghausen. Rev Neurol 2:66–70
Brandes WW (1933) A malignant neurinoma (Schwannoma) with epithelial elements. Arch Pathol 16:649–656
Carstens PH, Schrodt GR (1969) Malignant transformation of a benign encapsulated neurilemmoma. Am J Clin Pathol 51:144–149
D'Agostino AN, Soule EH, Miller RH (1963) Primary malignant neoplasms of nerves (malignant neurilemmomas) in patients without manifestations of multiple neurofibromatosis (Von Recklinghausen's disease). Cancer 16:1003–1014
D'Agostino AN, Soule EH, Miller RH (1963) Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (Von Recklinghausen's disease). Cancer 16:1015–1027
Denecke K (1932) Über zwei Fälle von metastasierenden Neurinomen des Magendarmkanals. Beitr Pathol Anat 89:242–248
Erlandson RA, Woodruff JM (1982) Peripheral nerve sheath tumors: An electron microscope study of 43 cases. Cancer 49:273–278
Fittipaldi C (1932) Contributo allo studio dei neurinomi. Riv Pat Nerv 39:521–558
Ghosh BC, Ghosh L, Huvos AG, Fortner JR (1973) Malignant schwannomas. A clinicopathologic study. Cancer 31:184–190
Gore L (1952) Primary malignant tumors of nerve: Report of 8 cases. Cancer 5:278
Guccion JG, Enzinger FM (1979) Malignant schwannoma associated with Recklinghausen's neurofibromatosis. Virch Arch [Pathol Anat] 383:43–57
Guleke N (1926) Zur Klinik des Neurinomas. Arch Klin Chir 142:478–498
Hajdu SI (1979) Tumors of peripheral nerves. In: Pathology of soft tissue tumors. Lea & Febiger, Philadelphia, p. 455
Harkin JC, Reed RJ (1969) Tumors of the peripheral nervous system. In: Firminger HI (eds). Atlas of Tumor Pathology, second series, FASC 3, AFIP, Washington, D.C.
Herrera GA, Reimann BEF, Salinas JA, Turbat EA (1982) Malignant schwannomas presenting as malignant fibrous histiocytomas. Ultr Pathol 3:253–261
Hiranandani LH, Hiranandani NL, Hiranandani GK (1957) Malignant schwannoma (neurogenic sarcoma). J Larying 81:1409–1413
Mackay B, Valenzuela J, Butler JJ (1975) Fine structural features of neurosarcomas. Proc 33rd Annual Meeting EMSA. GW Baily, Claitors Publication, Baton Rouge, pp 374–375
Markel SF, Enzinger FM (1982) Neuromuscular hamartoma - a benign “Triton tumor” composed of mature neural and striated muscle elements. Cancer 49:140–144
Quick D, Cutler M (1927) Neurogenic sarcoma: Clinical and pathologic study. Arch Surg 86:810
Robbins SL, Cotran RS (1979) In: Pathologic Basis of Disease. Chapter 7. W.B. Saunders, Philadelphia, London, Toronto, p. 238
Stewart FW, Copeland MM (1931) Neurogenic sarcoma. Am J Cancer 15:1235
Stout AP (1935) The malignant tumors of the peripheral nerves. Am J Cancer 25:1–36
Taxy JB, Battifora H, Trujillo Y, Dorfman HD (1981) Electron microscopy in the diagnosis of malignant schwannomas. Cancer 48:1381–1391
Trojanowski JQ, Kleinman GM, Proppe KH (1980) Malignant tumors of nerve sheath origin. Cancer 46:1202–1212
Stout AP (1946) Neurofibroma and neurilemmoma. Clin Proc 5:1–12
Vieta JO, Pack GT (1951) Malignant neurilemmomas of peripheral nerves. Am J Surg 82:416–431
Weiss SW, Langloss JM, Enzinger FM (1983) Values of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Inv 49:299–308
White HR, Jr (1971) Survival in malignant schwannoma. An 18 year study. Cancer 27:720–729
Woodruff JM, Chernk NL, Smith MC, Millett WB, Foote FW (1973) Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “Triton tumors”). Cancer 32:426–439
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Herrera, G.A., Pinto de Moraes, H. Neurogenic sarcomas in patients with neurofibromatosis (von Recklinghausen's disease). Vichows Archiv A Pathol Anat 403, 361–376 (1984). https://doi.org/10.1007/BF00737286
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DOI: https://doi.org/10.1007/BF00737286