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Malignant Small Cell Tumor of Thoracopulmonary Region/Primitive Neuroectodermal Tumor/Askin Tumor, Pleural

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Pathology of the Pleura and Mediastinum

Part of the book series: Encyclopedia of Pathology ((EP))

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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).

Malignant Small Cell Tumor of Thoracopulmonary Region/Primitive Neuroectodermal Tumor/Askin Tumor, Pleural, Fig. 1
figure 105 figure 105

Thoracic primitive neuroectodermal tumor (PNET). (a) Well-encapsulated hypercellular mass (H&E, 20×). (b) The lesion is comprised of monotonous and spindled small round blue cells with delicate intervening fibrous septae (H&E, 40×). (c) Focal areas of necrosis are noted (arrow; H&E, 40×). (d) Small round bluecells with perivascular rosetting...

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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.

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  • 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.

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  • 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.

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  • 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.

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  • 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.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. University of Texas Health Science Center at San Antonio, San Antonio, TX, 78229, USA

    Deepika Sirohi & Jaishree Jagirdar

Authors
  1. Deepika Sirohi
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  2. Jaishree Jagirdar
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Corresponding author

Correspondence to Deepika Sirohi .

Editor information

Editors and Affiliations

  1. Department of Pathology, The University of Mississippi Medical School, The University of Mississippi Medical Center, Jackson, MS, USA

    Timothy Craig Allen

  2. Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA

    Saul Suster

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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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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-66795-9

  • Online ISBN: 978-3-319-66796-6

  • eBook Packages: MedicineReference Module Medicine

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