Lancet Oncol. 2006 Feb;7(2):132-40.
Survival from rare cancer in adults: a population-based study.
Gatta G, Ciccolallo L, Kunkler I, Capocaccia R, Berrino F, Coleman MP,
De Angelis R, Faivre J, Lutz JM, Martinez C, Moller T, Sankila R;
EUROCARE Working Group.
Epidemiology Unit, National Cancer Institute, Milan, Italy.
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.
FINDINGS: 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. INTERPRETATION: 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.
PMID: 16455477 [PubMed – in process]