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EnglishAims: 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.
| Titolo: | CIC-DUX4 fusion-positive round-cell sarcomas of soft tissue and bone: A single-institution morphological and molecular analysis of seven cases | |
| Autore/i: | GAMBAROTTI, MARCO; Benini, Stefania; GAMBERI, GABRIELLA; COCCHI, STEFANIA; PALMERINI, EMANUELA; Sbaraglia, Marta; DONATI, DAVIDE MARIA; PICCI, PIERO; VANEL, DANIEL; FERRARI, STEFANO; RIGHI, ALBERTO; Dei Tos, Angelo P. | |
| Autore/i Unibo: | ||
| Anno: | 2016 | |
| Rivista: | ||
| Digital Object Identifier (DOI): | http://dx.doi.org/10.1111/his.12985 | |
| Abstract: | 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. | |
| Data stato definitivo: | 2017-06-06T12:34:53Z | |
| Appare nelle tipologie: | 1.01 Articolo in rivista |
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