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http://purl.uniprot.org/citations/27079694http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
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Aims

Round-cell sarcomas lacking specific translocations represent a diagnostic challenge. The aim of this study was to describe seven cases of CIC-DUX4 fusion-positive sarcomas, including the first reported example arising primarily in bone.

Methods and results

Patients ranged in age from 15 years to 44 years (median: 33 years). Six cases arose from the soft tissues, and one from the iliac bone. Morphologically, all cases showed an undifferentiated round-cell population with greater atypia and pleomorphism than Ewing sarcoma. Immunohistochemically, all tumours showed focal and weak positivity for CD99, and five of seven showed nuclear and/or cytoplasmic positivity for Wilms tumour 1. Five patients had lung metastases at presentation. All patients received chemotherapy according to Ewing sarcoma protocols. All but one patient (the one with a bone tumour) died of disease after a mean of 14.5 months from the diagnosis (range: 8-20 months).

Conclusions

Our series confirms that CIC-DUX4 fusion-positive sarcomas are aggressive tumours with an adverse prognosis, and with clinical, histological and genetic differences from Ewing sarcoma. The best therapeutic approach needs to be investigated."xsd:string
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http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/author"Gamberi G."xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/author"Donati D."xsd:string
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http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/author"Palmerini E."xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/author"Sbaraglia M."xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/author"Vanel D."xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/date"2016"xsd:gYear
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/name"Histopathology"xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/pages"624-634"xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/title"CIC-DUX4 fusion-positive round-cell sarcomas of soft tissue and bone: a single-institution morphological and molecular analysis of seven cases."xsd:string
http://purl.uniprot.org/citations/27079694http://purl.uniprot.org/core/volume"69"xsd:string
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