Head and neck round cell sarcomas: A comparative clinicopathologic analysis of 2 molecular subsets: Ewing and CIC-rearranged sarcomas Journal Article
| Authors: | Owosho, A. A.; Estilo, C. L.; Huryn, J. M.; Zhang, L.; Fletcher, C. D. M.; Antonescu, C. R. |
| Article Title: | Head and neck round cell sarcomas: A comparative clinicopathologic analysis of 2 molecular subsets: Ewing and CIC-rearranged sarcomas |
| Abstract: | CIC-rearranged sarcoma (CRS) is a relatively new entity defined by its pathognomonic genetic signature and undifferentiated round cell phenotype, initially grouped together with the ‘Ewing sarcoma-like tumors’. However, increasing data suggest that these tumors should be regarded as a stand-alone pathologic entity. We conducted a clinicopathologic analysis on molecularly conformed Ewing sarcoma (ES) and CRS arising in the head and neck (HN) and compared to a well characterized cohort of ES and CRS from other locations. A total of 41 HN round cell sarcoma patients were selected from our institutional and consultation files, including 25 ES (median 20 years) and 16 CRS (median 29 years). Clinical follow-up information was available for all ES patients, ranging from 4 to 436 months (median 70 months), while for CRS, follow-up information was available in 11 patients (69%), ranging from 1 to 269 months (median 27 months). The most common location for ES was the facial and jaw bones (56%), while CRS occurred exclusively in the soft tissue, commonly in the neck. CRS showed variable CD99 staining in 75% of cases and diffuse WT1 (6/6) reactivity, while all ES expressed diffuse membranous staining for CD99 but none for WT1 (0/6). The 2-year overall survival (OS) rate for HN-CRS patients was 78%, while for HN-ES it was 100%. The OS of ES and CRS showed a trend toward a favorable outcome for HN-round cell sarcomas compared to other sites. Our findings suggest that HN-CRS have different clinical presentation and pathologic features compared to ES and should be classified as a stand-alone pathologic entity. © 2017, Springer Science+Business Media New York. |
| Keywords: | sarcoma; ewsr1; cic-dux4; cic; dux4; sbrct |
| Journal Title: | Head and Neck Pathology |
| Volume: | 11 |
| Issue: | 4 |
| ISSN: | 1936-055X |
| Publisher: | Humana Press Inc |
| Date Published: | 2017-12-01 |
| Start Page: | 450 |
| End Page: | 459 |
| Language: | English |
| DOI: | 10.1007/s12105-017-0808-z |
| PROVIDER: | scopus |
| PUBMED: | 28337592 |
| PMCID: | PMC5796609 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 1 December 2017 -- Source: Scopus |
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