Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

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

  • Deepika SirohiEmail author
  • Jaishree Jagirdar
Reference work entry


Askin-Rosai tumor; Askin tumor; Peripheral neuroepithelioma of the thoracopulmonary region; Pleural malignant small cell tumor of the thoracopulmonary region; Primitive neuroectodermal tumor


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

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. 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.PubMedCrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.University of Texas Health Science Center at San AntonioSan AntonioUSA