Head and Neck Round Cell Sarcomas: A Comparative Clinicopathologic Analysis of 2 Molecular Subsets: Ewing and CIC-Rearranged Sarcomas.
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.
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Adepitan A
AA
Owosho
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Cherry L
CL
Estilo
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Joseph M
JM
Huryn
Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, USA.
Lei
L
Zhang
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
Christopher D M
CDM
Fletcher
Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, USA. antonesc@mskcc.org.
Cristina R
CR
Antonescu
D000293:Adolescent; D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D015321:Gene Rearrangement; D006258:Head and Neck Neoplasms / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012097:Repressor Proteins / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000473:pathology; D018228:Sarcoma, Small Cell / Q000235:genetics / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology; D055815:Young Adult
C498610:CIC protein, human; D012097:Repressor Proteins
CIC; CIC-DUX4; DUX4; EWSR1; SBRCT; Sarcoma