Adenoid cystic carcinoma of the breast in the United States (1977 to 2006): a population-based cohort study

• Published in: Breast cancer research : BCR 2010-01, Vol.12 (4), p.R54-R54, Article R54
• Main Authors: Ghabach, Bassam, Anderson, William F, Curtis, Rochelle E, Huycke, Mark M, Lavigne, Jackie A, Dores, Graça M
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; African Americans - statistics & numerical data; Aged; Breast cancer; Breast Neoplasms - epidemiology; Carcinoma, Adenoid Cystic - epidemiology; Care and treatment; Cohort Studies; Demographic aspects; European Continental Ancestry Group - statistics & numerical data; Female; Humans; Incidence; Middle Aged; Risk factors; SEER Program - statistics & numerical data; Survival Analysis; United States - epidemiology; Young Adult
• ISSN: 1465-542X; 1465-5411; 1465-542X
• DOI: 10.1186/bcr2613

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. 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. 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. 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.