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01.11.2009 | Original Article

Histological heterogeneity of Ewing’s sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support

verfasst von: Antonio Llombart-Bosch, Isidro Machado, Samuel Navarro, Franco Bertoni, Patrizia Bacchini, Marco Alberghini, Apollon Karzeladze, Nikita Savelov, Semyon Petrov, Isabel Alvarado-Cabrero, Doina Mihaila, Philippe Terrier, Jose Antonio Lopez-Guerrero, Piero Picci

Erschienen in: Virchows Archiv | Ausgabe 5/2009

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Abstract

Ewing’s sarcoma (ES)/peripheral neuroectodermal tumor (PNET) are malignant neoplasms affecting children and young adults. We performed a study to typify the histological diversity and evaluate antibodies that may offer diagnostic/prognostic support. In total, 415 cases of genetically confirmed paraffin-embedded ES/PNET were analyzed on whole sections and in tissue microarrays. This study confirms the structural heterogeneity of ES/PNET, distinguishing three major subtypes: conventional ES (280 cases); PNET (53 cases); and atypical ES/PNET (80), including large cells, vascular-like patterns, spindle pattern, and adamantinoma-like configuration. All cases presented positivity for at least three of the four tested antibodies (CD99, FLI1, HNK1, and CAV1). CAV1 appeared as a diagnostic immunomarker of ES/PNET being positive in CD99-negative cases. Hence, the immunohistochemical analysis confirmed the diagnostic value of all four antibodies, which together cover more than 99% of the tumors, independently of the histological variety. The univariate analysis for survival revealed atypical ES as the only histological parameter apparently associated with less favorable clinical outcome, particularly in the subgroup of patients treated with surgery. In conclusion, the diagnosis of atypical ES is a challenge for the pathologist and needs support from molecular techniques to perform an optimal differential diagnosis with other small round cell tumors.

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Titel: Histological heterogeneity of Ewing’s sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support
verfasst von: Antonio Llombart-Bosch
Isidro Machado
Samuel Navarro
Franco Bertoni
Patrizia Bacchini
Marco Alberghini
Apollon Karzeladze
Nikita Savelov
Semyon Petrov
Isabel Alvarado-Cabrero
Doina Mihaila
Philippe Terrier
Jose Antonio Lopez-Guerrero
Piero Picci
Publikationsdatum: 01.11.2009
Verlag: Springer-Verlag
Erschienen in: Virchows Archiv / Ausgabe 5/2009
Print ISSN: 0945-6317
Elektronische ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-009-0842-7

Neu im Fachgebiet Pathologie

01.04.2024 | Forensische Traumatologie | CME

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Histological heterogeneity of Ewing’s sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support

Abstract

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Springer Medizin

Abstract

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