title: Reappraising hyalinizing clear cell carcinoma: A population-based study with molecular confirmation.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  Molecularly confirmed HCCC accounted for 2.5% of salivary gland malignancies at our institution. HCCCs are indolent tumors with a propensity for locoregional recurrence. © 2016 Wiley Periodicals, Inc. Head Neck 39: 503-511, 2017.
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Juan C
authors.initials: JC
authors.lastname: Hernandez-Prera
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Ricky
authors.initials: R
authors.lastname: Kwan
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Joseph
authors.initials: J
authors.lastname: Tripodi
authors.affiliation: Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
authors.forename: Simion
authors.initials: S
authors.lastname: Chiosea
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Carlos
authors.initials: C
authors.lastname: Cordon-Cardo
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Vesna
authors.initials: V
authors.lastname: Najfeld
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Elizabeth G
authors.initials: EG
authors.lastname: Demicco
mesh_terms: D051697:Activating Transcription Factor 1 / Q000235:genetics*; D018262:Adenocarcinoma, Clear Cell / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D002294:Carcinoma, Squamous Cell / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D034802:RNA-Binding Protein EWS / Q000235:genetics*; D016601:RNA-Binding Proteins / Q000235:genetics; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D015996:Survival Rate
chemical_list: C531110:ATF1 protein, human; D051697:Activating Transcription Factor 1; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins
keywords: EWSR1; clear cell carcinoma; hyalinizing clear cell carcinoma; salivary gland