title: Management of adenoid cystic carcinoma of the breast: a Rare Cancer Network study.
abstract: BACKGROUND
  Mammary adenoid cystic carcinoma (ACC) is a rare breast cancer. The aim of this retrospective study was to assess prognostic factors and patterns of failure, as well as the role of radiation therapy (RT), in ACC.
  
  
  METHODS
  Between January 1980 and December 2007, 61 women with breast ACC were treated at participating centers of the Rare Cancer Network. Surgery consisted of lumpectomy in 41 patients and mastectomy in 20 patients. There were 51(84%) stage pN0 and 10 stage cN0 (16%) patients. Postoperative RT was administered to 40 patients (35 after lumpectomy, 5 after mastectomy).
  
  
  RESULTS
  With a median follow-up of 79 months (range, 6-285), 5-year overall and disease-free survival rates were 94% (95% confidence interval [CI], 88%-100%) and 82% (95% CI, 71%-93%), respectively. The 5-year locoregional control (LRC) rate was 95% (95% CI, 89%-100%). Axillary lymph node dissection or sentinel node biopsy was performed in 84% of cases. All patients had stage pN0 disease. In univariate analysis, survival was not influenced by the type of surgery or the use of postoperative RT. The 5-year LRC rate was 100% in the mastectomy group versus 93% (95% CI, 83%-100%) in the breast-conserving surgery group, respectively (p = 0.16). For the breast-conserving surgery group, the use of RT significantly correlated with LRC (p = 0.03); the 5-year LRC rates were 95% (95% CI, 86%-100%) for the RT group versus 83% (95% CI, 54%-100%) for the group receiving no RT. No local failures occurred in patients with positive margins, all of whom received postoperative RT.
  
  
  CONCLUSIONS
  Breast-conserving surgery is the treatment of choice for patients with ACC breast cancer. Axillary lymph node dissection or sentinel node biopsy might not be recommended. Postoperative RT should be proposed in the case of breast-conserving surgery.
authors.affiliation: Hôpital de Sion, CHCVs, Sion, Switzerland. kaouthar.khanfir@rsv-gnw.ch
authors.forename: Kaouthar
authors.initials: K
authors.lastname: Khanfir
authors.forename: Adel
authors.initials: A
authors.lastname: Kallel
authors.forename: Sylviane
authors.initials: S
authors.lastname: Villette
authors.forename: Yazid
authors.initials: Y
authors.lastname: Belkacémi
authors.forename: Claire
authors.initials: C
authors.lastname: Vautravers
authors.forename: Tandat
authors.initials: T
authors.lastname: Nguyen
authors.forename: Robert
authors.initials: R
authors.lastname: Miller
authors.forename: Ye Xiong
authors.initials: YX
authors.lastname: Li
authors.forename: Alphonse G
authors.initials: AG
authors.lastname: Taghian
authors.forename: Liesbeth
authors.initials: L
authors.lastname: Boersma
authors.forename: Philip
authors.initials: P
authors.lastname: Poortmans
authors.forename: Hadassah
authors.initials: H
authors.lastname: Goldberg
authors.forename: Hansjorg
authors.initials: H
authors.lastname: Vees
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Senkus
authors.forename: Sefik
authors.initials: S
authors.lastname: Igdem
authors.forename: Mahmut
authors.initials: M
authors.lastname: Ozsahin
authors.forename: Wendy
authors.initials: W
authors.lastname: Jeanneret Sozzi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001943:Breast Neoplasms / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D003528:Carcinoma, Adenoid Cystic / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015412:Mastectomy, Segmental / Q000379:methods; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D035583:Rare Diseases / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate