title: Adenoid cystic carcinoma of the breast in the United States (1977 to 2006): a population-based cohort study.
abstract: BACKGROUND
  Adenoid cystic carcinoma of the breast (breast-ACC) is a rare and special type of basal-like tumor for which scant population-based descriptive data exist. We sought to provide new population-based information on breast-ACC incidence, relative survival, and associated cancer risk in the United States.
  
  
  METHODS
  Using data from the Surveillance, Epidemiology and End Results Program, we calculated age-adjusted incidence rates (IRs), IR ratios (IRRs), and relative survival for breast-ACC, and standardized incidence ratios (SIRs) for other cancers.
  
  
  RESULTS
  Overall 338 women (IR = 0.92/1 million person-years) were diagnosed with breast-ACC during 1977 to 2006. Blacks had 39% lower IRs than Whites (IRR = 0.61, 95% confidence interval = 0.37 to 0.96), and IRs remained constant over the 30-year period. Ninety-five percent of cases presented with localized stage (n = 320; IR = 0.87), and the highest IRs were observed for estrogen receptor (ER)-negative/progesterone receptor (PR)-negative tumors (IR = 0.56). Like other typically ER-negative tumors, age-specific IRs increased until midlife and then plateaued. Five-year, 10-year, and 15-year relative survival was 98.1%, 94.9%, and 91.4%, respectively. The risk of female breast cancer was not increased following (SIR = 0.89, 95% confidence interval = 0.43 to 1.64) or preceding (SIR = 0.71, 95% confidence interval = 0.28 to 1.46) breast-ACC. Similarly, no association was observed for breast-ACC and risk of all other cancers combined, solid tumors, or lymphohematopoietic malignancies.
  
  
  CONCLUSIONS
  Breast-ACC among women is characterized by ER-negative/PR-negative expression, rare regional lymph node involvement, a favorable prognosis with excellent survival, and absence of associated cancers. These findings reinforce the importance of tailored treatments for breast-ACC and lend credence to the apparent heterogeneity of basal-like breast cancers.
authors.affiliation: Department of Veterans Affairs Medical Center, Medical Service (111), 921 NE 13th Street, Oklahoma City, OK 73104, USA. bassam-ghabach@ouhsc.edu
authors.forename: Bassam
authors.initials: B
authors.lastname: Ghabach
authors.forename: William F
authors.initials: WF
authors.lastname: Anderson
authors.forename: Rochelle E
authors.initials: RE
authors.lastname: Curtis
authors.forename: Mark M
authors.initials: MM
authors.lastname: Huycke
authors.forename: Jackie A
authors.initials: JA
authors.lastname: Lavigne
authors.forename: Graça M
authors.initials: GM
authors.lastname: Dores
mesh_terms: D000293:Adolescent; D000328:Adult; D001741:Black or African American / Q000706:statistics & numerical data; D000368:Aged; D001943:Breast Neoplasms / Q000453:epidemiology*; D003528:Carcinoma, Adenoid Cystic / Q000453:epidemiology*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D018426:SEER Program / Q000706:statistics & numerical data*; D016019:Survival Analysis; D014481:United States / Q000453:epidemiology; D044465:White People / Q000706:statistics & numerical data; D055815:Young Adult