title: Survival from rare cancer in adults: a population-based study.
abstract: BACKGROUND
  Rare cancers are a challenge to clinical practice, and treatment experience, even in major cancer centres to which rare cancers are usually referred, is often limited. We aimed to study the epidemiology of rare cancers in a large population of several countries.
  
  
  METHODS
  We analysed survival by age, sex, subsite, and morphology in 57,144 adults with 14 selected rare cancers diagnosed 1983-94. Variations in survival over time and between European regions were also assessed for variations in quality of care. We also estimated the adjusted relative excess risk of death for every rare cancer.
  
  
  RESULTS
  Overall 5-year relative survival was good (ie, >65%) for placental choriocarcinoma (85.4% [95% CI 81.4-89.5]), thyroid medullary carcinoma (72.4% [69.2-75.5]), ovarian germ-cell cancer (73.0% [70.0-76.0]), lung carcinoid (70.1% [67.3-72.9]), and cervical adenocarcinoma (65.5% [64.3-66.6]); intermediate (ie, 35-65%) for testicular cancer at age 65 years or older (64.0% [59.3-68.7]), sarcoma of extremities (60.0% [58.9-61.2]), digestive-system endocrine cancers (55.6% [54.9-56.3]), anal squamous-cell carcinoma (53.1% [51.5-54.8]), and uterine sarcoma (43.5% [42.0-44.9]); low for carcinoma of adrenal-gland cortex (32.7% [28.3-37.2]) and bladder squamous-cell carcinoma (20.4% [18.8-22.0]); and poor for angiosarcoma of liver (6.4% [1.8-11.0]) and mesothelioma (4.7% [4.3-5.2]). Survival was usually better for women than men and poor in those aged 75 years or older. Survival significantly improved over time for ovarian germ-cell cancer, sarcomas of extremities, digestive-system endocrine tumours, anal squamous-cell carcinoma, and angiosarcoma of liver. Survival in northern Europe was higher than in the other geographic groupings for most cancers.
  
  
  CONCLUSIONS
  Because effective treatments are available for several of the rare cancers we assessed, further research is needed to ascertain why survival is lower in some European countries than in others, particularly in older patients. Audit of best practice for rare cancers with treatment protocols would be useful.
authors.affiliation: Epidemiology Unit, National Cancer Institute, Milan, Italy. gemma.gatta@istitutotumori.mi.it
authors.forename: Gemma
authors.initials: G
authors.lastname: Gatta
authors.forename: Laura
authors.initials: L
authors.lastname: Ciccolallo
authors.forename: Ian
authors.initials: I
authors.lastname: Kunkler
authors.forename: Riccardo
authors.initials: R
authors.lastname: Capocaccia
authors.forename: Franco
authors.initials: F
authors.lastname: Berrino
authors.forename: Michel P
authors.initials: MP
authors.lastname: Coleman
authors.forename: Roberta
authors.initials: R
authors.lastname: De Angelis
authors.forename: Jean
authors.initials: J
authors.lastname: Faivre
authors.forename: Jean Michel
authors.initials: JM
authors.lastname: Lutz
authors.forename: Carmen
authors.initials: C
authors.lastname: Martinez
authors.forename: Torgil
authors.initials: T
authors.lastname: Möller
authors.forename: Risto
authors.initials: R
authors.lastname: Sankila
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000401:mortality*; D011787:Quality of Health Care; D035583:Rare Diseases / Q000401:mortality*; D012306:Risk; D016019:Survival Analysis