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).
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.
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.
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.
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.
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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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