Synonyms
Askin-Rosai tumor; Askin tumor; Peripheral neuroepithelioma of the thoracopulmonary region; Pleural malignant small cell tumor of the thoracopulmonary region; Primitive neuroectodermal tumor
Definition
Primitive neuroectodermal tumors (PNETs) of the thoracopulmonary region carry the specific name of Askin tumor. They are a group of highly aggressive malignant tumors involving the bones and soft tissue and are characterized by the presence of undifferentiated small round blue cells of neuroectodermal origin (Figs. 1 and 2).
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References and Further Reading
Biswas, B., et al. (2013). Evaluation of outcome and prognostic factors in thoracic primitive neuroectodermal tumor: A study of 84 cases. Annals of Thoracic Surgery, 96, 2006–2014.
Desai, S. S., & Jambhekar, N. A. (2010). Pathology of Ewing’s sarcoma/PNET: Current opinion and emerging concepts. Indian Journal Orthopaedic, 44(4), 363–368.
Folpe, A. L., Hill, C. E., Parham, D. M., O’Shea, P. A., & Weiss, S. W. (2000). Immunohistochemical detection of FLI-1 protein expression a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing’s sarcoma/primitive neuroectodermal tumor. American Journal of Surgical Pathology, 24(12), 1657–1662.
Frederic, B., Askin, M., Rosai, J., Sibley, R. K., Dehner, L. P., & Mcalister, W. H. (1979). Malignant small cell tumor of the thoracopulmonary region in childhood. A distinctive clinicopathologic entity of uncertain histogenesis. Cancer, 43, 2438–2451.
Sumegi, J., Nishio, J., Nelson, M., Frayer, R. W., Perry, D., & Bridge, J. A. (2011). A novel t(4;22)(q31;q12) produces an EWSR1–SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. Modern Pathology, 24, 333–342.
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Sirohi, D., Jagirdar, J. (2018). Malignant Small Cell Tumor of Thoracopulmonary Region/Primitive Neuroectodermal Tumor/Askin Tumor, Pleural. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_368
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DOI: https://doi.org/10.1007/978-3-319-66796-6_368
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