TY - JOUR
T1 - Reappraising hyalinizing clear cell carcinoma
T2 - A population-based study with molecular confirmation
AU - Hernandez–Prera, Juan C.
AU - Kwan, Ricky
AU - Tripodi, Joseph
AU - Chiosea, Simion
AU - Cordon–Cardo, Carlos
AU - Najfeld, Vesna
AU - Demicco, Elizabeth G.
N1 - Publisher Copyright:
© 2016 Wiley Periodicals, Inc.
PY - 2017/3/1
Y1 - 2017/3/1
N2 - Background: Hyalinizing clear cell carcinoma (HCCC) is a rare malignancy, characterized by EWSR1-ATF1 gene fusion, whose behavior is poorly understood, as it was for many years considered a diagnosis of exclusion. Methods: All available salivary gland carcinomas (n = 594) from our institution were reviewed. Diagnosis of HCCC was confirmed by fluorescence in situ hybridization (FISH) for EWSR1. Literature review was performed. Results: We found 15 patients with HCCCs (10 women, 5 men), 13 with EWSR1 rearrangement. Median age at diagnosis was 57 years (range, 31–87 years). Oral cavity (n = 9) and base of tongue (n = 4) were the most frequent primary sites. Combining our cases with those identified in literature review, the 10-year risk of local recurrence and locoregional nodal metastasis were 49% and 15%, respectively. Conclusion: Molecularly confirmed HCCC accounted for 2.5% of salivary gland malignancies at our institution. HCCCs are indolent tumors with a propensity for locoregional recurrence.
AB - Background: Hyalinizing clear cell carcinoma (HCCC) is a rare malignancy, characterized by EWSR1-ATF1 gene fusion, whose behavior is poorly understood, as it was for many years considered a diagnosis of exclusion. Methods: All available salivary gland carcinomas (n = 594) from our institution were reviewed. Diagnosis of HCCC was confirmed by fluorescence in situ hybridization (FISH) for EWSR1. Literature review was performed. Results: We found 15 patients with HCCCs (10 women, 5 men), 13 with EWSR1 rearrangement. Median age at diagnosis was 57 years (range, 31–87 years). Oral cavity (n = 9) and base of tongue (n = 4) were the most frequent primary sites. Combining our cases with those identified in literature review, the 10-year risk of local recurrence and locoregional nodal metastasis were 49% and 15%, respectively. Conclusion: Molecularly confirmed HCCC accounted for 2.5% of salivary gland malignancies at our institution. HCCCs are indolent tumors with a propensity for locoregional recurrence.
KW - EWSR1
KW - clear cell carcinoma
KW - hyalinizing clear cell carcinoma
KW - salivary gland
UR - http://www.scopus.com/inward/record.url?scp=85006241100&partnerID=8YFLogxK
U2 - 10.1002/hed.24637
DO - 10.1002/hed.24637
M3 - Article
C2 - 27898192
AN - SCOPUS:85006241100
SN - 1043-3074
VL - 39
SP - 503
EP - 511
JO - Head and Neck
JF - Head and Neck
IS - 3
ER -