title: MYB rearrangement and clinicopathologic characteristics in head and neck adenoid cystic carcinoma.
abstract: OBJECTIVE
  Salivary gland adenoid cystic carcinoma (ACC) is rare, aggressive, and challenging to treat. Many ACCs have a t(6;9) chromosomal translocation resulting in a MYB-NFIB fusion gene, but the clinical significance is unclear. The purposes of this study were to describe the clinicopathologic factors impacting survival and to determine the prevalence and clinical significance of MYB-NFIB fusion.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Medical records of patients treated for ACC of the head and neck from 1974 to 2011 were reviewed and clinicopathologic data recorded. Fluorescence in situ hybridization (FISH) was used to detect MYB rearrangement in archival tumor tissue as a marker of MYB-NFIB fusion.
  
  
  RESULTS
  One hundred fifty-eight patients were included, with median follow-up 75.1 months. Median overall survival was 171.5 months (95% confidence interval [CI] = 131.9-191.6), and median disease-free survival was 112.0 months (95% CI = 88.7-180.4). Advanced stage was associated with decreased overall survival (adjusted ptrend  < 0.001), and positive margins were associated with decreased disease-free survival (adjusted hazard ratio [aHR] = 8.80, 95% CI = 1.25-62.12, P = 0.029). Ninety-one tumors were evaluable using FISH, and 59 (65%) had evidence of a MYB-NFIB fusion. MYB-NFIB positive tumors were more likely than MYB-NFIB negative tumors to originate in minor salivary glands (adjusted prevalence ratios = 1.51, 95% CI = 1.07-2.12, P = 0.019). MYB-NFIB tumor status was not significantly associated with disease-free or overall survival (hazard ratio [HR] = 1.53, 95% CI = 0.77-3.02, P = 0.22 and HR = 0.91, 95% CI = 0.46-1.83, P = 0.80, respectively, for MYB-NFIB positive compared with MYB-NFIB negative tumors).
  
  
  CONCLUSIONS
  Stage and margin status were important prognostic factors for ACC. Tumors with evidence of MYB-NFIB fusion were more likely to originate in minor salivary glands, but MYB-NFIB tumor status was not significantly associated with prognosis.
  
  
  METHODS
  4.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery.
authors.forename: Eleni M
authors.initials: EM
authors.lastname: Rettig
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery.
authors.forename: Marietta
authors.initials: M
authors.lastname: Tan
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery.
authors.forename: Shizhang
authors.initials: S
authors.lastname: Ling
authors.affiliation: Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Raluca
authors.initials: R
authors.lastname: Yonescu
authors.affiliation: Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Justin A
authors.initials: JA
authors.lastname: Bishop
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery.|Milton J. Dance Jr. Head and Neck Center, Greater Baltimore Medical Center.|Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, U.S.A.
authors.forename: Carole
authors.initials: C
authors.lastname: Fakhry
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery.|Milton J. Dance Jr. Head and Neck Center, Greater Baltimore Medical Center.
authors.forename: Patrick K
authors.initials: PK
authors.lastname: Ha
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003528:Carcinoma, Adenoid Cystic / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D004273:DNA, Neoplasm / Q000235:genetics*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D015321:Gene Rearrangement / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D015996:Survival Rate / Q000639:trends; D055815:Young Adult
chemical_list: D004273:DNA, Neoplasm; C545202:MYB-NFIB fusion protein, human; D015514:Oncogene Proteins, Fusion
keywords: Adenoid cystic carcinoma; MYB; MYB-NFIB fusion gene; disease-free survival; minor salivary glands; salivary gland neoplasms; survival