title: Inflammatory breast cancer management in the national comprehensive cancer network: the disease, recurrence pattern, and outcome.
abstract: BACKGROUND
  Inflammatory breast cancer (IBC) is an uncommon clinicopathologic entity characterized by rapid progression and aggressive behavior. We used the National Comprehensive Cancer Network (NCCN) Outcomes Database to characterize recurrence patterns and outcomes.
  
  
  METHODS
  Patients with newly diagnosed IBC treated between 1999 and 2009 at 12 NCCN institutions were identified, and baseline characteristics were obtained. Patients had multimodality therapy if they received 2 of 3 treatments: surgery, perioperative (neoadjuvant or adjuvant) chemotherapy, or perioperative radiation. The first site of recurrence/metastatic diagnosis was identified. Overall survival was calculated on the basis of stage at diagnosis and receipt of multimodality therapy.
  
  
  RESULTS
  We identified 673 patients, of whom 195 (29%) had metastatic disease at presentation. Median follow-up was 29 months. Of patients in stage III, 82% received > 1 treatment modality. Among 203 patients in stage III with recurrence, the most frequent sites of first recurrence were bone (28%), central nervous system (CNS), lung, and liver (all 21%). Human epidermal growth factor receptor 2 positive and triple negative subtypes had higher rates of CNS recurrence (P = .001). Median survival was 66 months (95% confidence interval [CI], 54-107) for stage III and 26 months (95% CI, 22-33) for stage IV. Among 82% of patients in stage III receiving multimodality therapy, the median survival was 107 months (95% CI, 71 to not reached).
  
  
  CONCLUSIONS
  This large, retrospective, multi-institutional study confirms the aggressive clinical features, unique recurrence patterns, and adverse prognosis of IBC. The high rate of CNS recurrence among high-risk subtypes, despite the inflammatory nature of the breast cancer, suggests that new strategies are needed for earlier detection or prevention of brain metastases to improve long-term prognosis.
authors.affiliation: Fox Chase Cancer Center, Philadelphia, PA. Electronic address: Jennifer.matro@uphs.upenn.edu.
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Matro
authors.affiliation: Fox Chase Cancer Center, Philadelphia, PA.
authors.forename: Tianyu
authors.initials: T
authors.lastname: Li
authors.affiliation: Thomas Jefferson University Hospital, Philadelphia, PA.
authors.forename: Massimo
authors.initials: M
authors.lastname: Cristofanilli
authors.affiliation: Dana Farber Cancer Institute, Boston, MA.
authors.forename: Melissa E
authors.initials: ME
authors.lastname: Hughes
authors.affiliation: City of Hope Cancer Center, Duarte, CA.
authors.forename: Rebecca A
authors.initials: RA
authors.lastname: Ottesen
authors.affiliation: Dana Farber Cancer Institute, Boston, MA.
authors.forename: Jane C
authors.initials: JC
authors.lastname: Weeks
authors.affiliation: Fox Chase Cancer Center, Philadelphia, PA.
authors.forename: Yu-Ning
authors.initials: YN
authors.lastname: Wong
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019058:Community Networks / Q000458:organization & administration; D003191:Comprehensive Health Care / Q000379:methods / Q000458:organization & administration; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D058922:Inflammatory Breast Neoplasms / Q000453:epidemiology / Q000628:therapy*; D008875:Middle Aged; D012008:Recurrence; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: Brain metastases; Inflammatory breast cancer; Metastatic breast cancer; Multimodality therapy; Triple negative breast cancer