Primary low-grade fibromyxoid sarcoma of the kidney in a child with the alternative EWSR1-CREB3L1 gene fusion Journal Article
| Authors: | Rubinstein, J. C.; Visa, A.; Zhang, L.; Antonescu, C. R.; Christison-Lagay, E. R.; Morotti, R. |
| Article Title: | Primary low-grade fibromyxoid sarcoma of the kidney in a child with the alternative EWSR1-CREB3L1 gene fusion |
| Abstract: | We present the case of a 6-year-old boy with a deceptively bland spindle cell renal neoplasm found to harbor the EWSR1-CREB3L1 gene fusion. This fusion has recently been described as a variant translocation in low-grade fibromyxoid sarcoma (LGFMS), a tumor more typically characterized by a recurrent t(7;16) chromosomal translocation, resulting in the fusion of FUS and CREB3L2 genes. LGFMS is an indolent tumor with late metastatic potential and a propensity for long-term disease recurrence. The tumor is rare in children, with only 33 published cases. In the pediatric population, it has not previously been reported arising in the kidney. |
| Keywords: | pediatric; molecular analysis; tumor; renal; clinicopathological analysis; low-grade fibromyxoid sarcoma; sclerosing epithelioid fibrosarcoma; ewsr1-creb3l1 fusion |
| Journal Title: | Pediatric and Developmental Pathology |
| Volume: | 17 |
| Issue: | 4 |
| ISSN: | 1093-5266 |
| Publisher: | Sage Publications, Inc. |
| Date Published: | 2014-07-01 |
| Start Page: | 321 |
| End Page: | 326 |
| Language: | English |
| ACCESSION: | WOS:000342383300012 |
| DOI: | 10.2350/14-05-1487-cr.1 |
| PROVIDER: | wos |
| PUBMED: | 24896634 |
| Notes: | Article -- Source: Wos |
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