Abstract
The purpose of this study was to validate the hypothesis of neural histogenesis of Ewing's sarcoma of bone and related tumors by light microscopic, electron microscopic, and immunohistochemical analysis. We studied 32 round-cell sarcomas (19 cases of Ewing's sarcoma of bone, 3 extraskeletal Ewing's sarcomas, 5 peripheral primitive neuroectodermal tumors (PNET) and 5 cases of unclassified small round-cell type of neurogenic sarcoma (NS). Immunoreactivity for MIC2 was observed in all cases of Ewing's sarcoma and PNET, and in 1 cases of NS. Positive immunoreactivity for neural markers (NSE, synaptophysin, S-100) was found frequently in some tumors. Ultrastructurally, some specific features of neural differentiation, such as a fragmented basal lamina and neurosecretory granule-like particles, were found even in typical cases of Ewing's sarcoma of bone, which presented without a rosette arrangement and were almost negative for neural immuno-markers, but positive for MIC2. These ultrastructural neural features were observed less frequently in Ewing's sarcoma of bone than in PNET and NS. However, no significant correlation was demonstrated between the immunoreactivity for neural markers and the ultrastructural and histological neural features. These results support the hypothesis of a neural origin of Ewing's sarcoma and related neoplasms, and suggest that some overlapping entity could persist in PNET and Ewing's sarcoma and that this entity could be seen in histological and immunohistochemical studies of both tumors.
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References
Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975;36:240–51.
Ambros IM, Ambros PF, Strehl S, et al. MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer 1991; 67:1886–93.
Aurias A, Rimbaut C, Buffe D, et al. Chromosomal translocation in Ewing's sarcoma. N Engl J Med 1983;309:496–7.
Brinkhuis M, Wijnaendts LCD, van der Linden JC, et al. Peripheral primitive neuroectodermal tumour and extra-osseous Ewing's sarcoma; a histological, immunohistochemical and DNA flow cytometric study. Virchows Arch B Cell Pathol 1995; 425:611–6.
Colville HC, Willis RA. Neuroblastoma metastases in bones, with a criticism of Ewing's endothelioma. Am J Pathol 1933;9:421–9.
Dardick I, Rippstein P, Perkins G. Reliability of criteria for ultrastructural identification of neuroendocrine granules. Ultrastruct Pathol 1993;17:37–47.
Dehner LP. Primitive neuroectodermal tumor and Ewing's sarcoma. Am J Surg Pathol 1993;17:1–13.
Dehner LP. Neuroepithelioma (Primitive neuroectodermal tumor) and Ewing's sarcoma (At least a partial consensus). Arch Pathol Lab Med 1994;118:606–7.
Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors —a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994;331:294–9.
Dierick AM, Roels H, Langlois M. The immunophenotype of Ewing's sarcoma: An immunohistochemical analysis. Pathol Res Pract 1993;189:26–32.
Dockhorn-Dworniczak B, Schäfer KL, Dantcheva R, et al. Diagnostic value of the molecular genetic detection of the t(11;22) translocation in Ewing's tumours. Virchows Arch A Pathol Anat Histopathol 1994;425:107–12.
Ewing J. Diffuse endothelioma of bone. Proc NY Pathol Soc 1921;21:17–24.
Fellinger EJ, Grain-Chesa P, Glasser DB, et al. Comparison of cell surface antigen HBA71 (p30/32MIC2), neuron-specific enolase, and vimentin in the immunohistochemical analysis of Ewing's sarcoma of bone. Am J Surg Pathol 1992;16:746–55.
Jaffe R, Santamaria M, Yunis EJ, et al. The neuroectodermal tumor of bone. Am J Surg Pathol 1984;8:885–98.
Kawaguchi K, Koike M. Neuron-specific enolase and Leu-7 immunoreactive small round-cell neoplasm: The relationship to Ewing's sarcoma in bone and soft tissue. Am J Clin Pathol 1986;86:79–83.
Kitagawa Y. Clinicopathological study of Ewing's sarcoma and primitive neuroectodermal tumor. J Jpn Orthop Assoc 1993;67:1140–50 (in Japanese).
Ladanyi M, Lewis R, Grain-Chesa P, et al. EWS rearrangement in Ewing's sarcoma and peripheral neuroectodermal tumor. Molecular detection and correlation with cytogenetic analysis and MIC2 expression. Diagn Mol Pathol 1993;2:141–6.
Llombart-Bosch A, Lacombe MJ, Contesso G, et al. Small round blue cell sarcoma of bone mimicking atypical Ewing's sarcoma with neuroectodermal features. Cancer 1987;60:1570–82.
Miki H, Unno K, Park P, et al. Morphogenesis and origin of fibrous long-spacing collagen fibers in collagenase-treated mouse skin tissues. Tissue Cell 1993;25:669–80.
Navarro S, Cavazzana AO, Llombart-Bosch A, et al. Comparison of Ewing's sarcoma of bone and peripheral neuroepithelioma (an immunocytochemical and ultrastructural analysis of two primitive neuroectodermal neoplasms). Arch Pathol Lab Med 1994; 118:608–15.
Navas-Palacios JJ, Aparicio-Duque R, Valdés MD. On the histogenesis of Ewing's sarcoma. An ultrastructural, immunohistochemical, and cytochemical study. Cancer 1984;53:1882–901.
Ramani P, Rampling D, Link M. Immunocytochemical study of 12E7 in small round-cell tumours of childhood: An assessment of its sensitivity and specificity. Histopathology 1993;23:557–61.
Rettig WJ, Garin-Chesa P, Huvos AG. Ewing's sarcoma: New approaches to histogenesis and molecular plasticity. Lab Invest 1992;2:133–7.
Schajowicz F. Histological typing of bone tumours. 2nd ed. Berlin Heidelberg: Springer New York Tokyo, 1993;22–4.
Schmidt D, Mackay B, Ayala AG. Ewing's sarcoma with neuroblastoma-like features. Ultrastruct Pathol 1982;3:143–51.
Schmidt D, Herrmann C, Jürgens H, et al. Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing's sarcoma. A report from the Kiel pediatric tumor registry. Cancer 1991;68:2251–9.
Tsuneyoshi M, Yokoyama R, Hashimoto H, et al. Comparative study of neuroectodermal tumor and Ewing's sarcoma of the bone. Histopathologic, immunohistochemical and ultrastructural features. Acta Pathol Jpn 1989;39:573–81.
Ushigome S, Shimoda T, Takai K, et al. Immunocytochemical and ultrastructural studies of the histogenesis of Ewing's sarcoma and putatively related tumors. Cancer 1989;64:52–62.
Weiss SW. Histological typing of soft tissue tumours. 2nd ed. Berlin Heidelberg: Springer New York Tokyo, 1994:43–9.
Yunis EJ. Ewing's sarcoma and related small round cell neoplasms in children. Am J Surg Pathol 1986;10(Suppl 1):54–62.
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Abe, S. Ultrastructural and immunohistochemical study of Ewing's sarcoma and related tumors: Morphological neural markers suggesting neural histogenesis in 32 small round-cell sarcomas. J Orthop Sci 2, 75–83 (1997). https://doi.org/10.1007/BF02489516
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DOI: https://doi.org/10.1007/BF02489516