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Primitive Neuroectodermal Tumors (PNET), Cytological Findings

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Cytopathology

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

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Synonyms

Askin tumor; Peripheral neuroblastoma; Peripheral neuroepithelioma

Definition

Primitive tumors with neuroectodermal differentiation.

Clinical Features

  • Incidence

    2/1 000 000

  • Age

    A majority of patients are below 20 years.

  • Sex

    Males are more often affected.

  • Site

    Diaphysis of long bones, pelvis, and ribs. Soft tissue.

  • Treatment

    Chemotherapy, radiation, and surgery.

  • Outcome

    Approximately 50% of the patients survive. Patients with small, peripherally located tumors have best survival.

Microscopy

Double cell population: small cells with scanty cytoplasm and dark nuclei and larger cells with round nucleus and rich cytoplasm often with vacuoles. Rosette structures often present.

Immunophenotype

Vimentin, CD99 positive. Synaptophysin positivity in some cases (Fig. 1).

Primitive Neuroectodermal Tumors (PNET), Cytological Findings, Fig. 1
figure 15111 figure 15111

Ewing/PNET: tumor cells of two types; one with small hyperchromatic nuclei and with sparse or no cytoplasm. The second type is larger with round to oval...

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References and Further Reading

  • Fröstad, B., Tani, E., Brosjö, O., Skoog, L., & Kogner, P. (2002). Fine needle aspiration cytology in the diagnosis and management of children and adolescents with Ewing sarcoma and peripheral primitive neuroectodermal tumor. Medical and Pediatric Oncology, 38, 33–40.

    Article  PubMed  Google Scholar 

  • Klijanienko, J., Couturier, J., Bourdeaut, F., Fréneaux, P., Ballet, S., Brisse, H., Lagacé, R., Delattre, O., Pierron, G., Vielh, P., Sastre-Garau, X., & Michon, J. (2012). Fine-needle aspiration as a diagnostic technique in 50cases of primary Ewing sarcoma/peripheral neuroectodermal tumor. Institut Curie’s experience. Diagnostic Cytopathology, 40, 19–25.

    Article  PubMed  Google Scholar 

  • Sahu, K., Pai, R. R., & Khadilkar, U. N. (2000). Fine needle aspiration cytology of the Ewing’s sarcoma family of tumors. Acta Cytologica, 44, 332–336.

    Article  CAS  PubMed  Google Scholar 

  • Udayakumar, A. M., Sundareshan, T. S., Goud, T. M., Devi, M. G., Biswas, S., Appaji, L., Arunakumari, B. S., Rajan, K. R., & Prabhakaran, P. S. (2001). Cytogenetic characterization of Ewing tumors using fine needle aspiration samples, a 10-year experience and review of the literature. Cancer Genetics and Cytogenetics, 127, 42–48.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Lambert Skoog .

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Skoog, L., Tani, E.M. (2017). Primitive Neuroectodermal Tumors (PNET), Cytological Findings. In: Schmitt, F. (eds) Cytopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-33286-4_964

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  • DOI: https://doi.org/10.1007/978-3-319-33286-4_964

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-33285-7

  • Online ISBN: 978-3-319-33286-4

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