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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 and Martinez, C, et al. (2006) In The Lancet Oncology 7(2). p.132-140
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. Findings Overall 5-year relative survival was good (ie, > 65%) for placental choriocarcinoma (85-4% [95% CI 81.4-89.5]), thyroid medullary... (More)
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. (Less)
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Contribution to journal
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published
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The Lancet Oncology
volume
7
issue
2
pages
132 - 140
publisher
Elsevier
external identifiers
  • pmid:16455477
  • wos:000235315900030
  • scopus:31544438044
ISSN
1474-5488
DOI
10.1016/S1470-2045(05)70471-X
language
English
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yes
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fda4081e-5b18-4d86-8cbf-fdec1ae9b208 (old id 417707)
date added to LUP
2007-10-03 08:31:35
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2019-09-01 03:48:16
@article{fda4081e-5b18-4d86-8cbf-fdec1ae9b208,
  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. 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.},
  author       = {Gatta, G and Ciccolallo, L and Kunkler, I and Capocaccia, R and Berrino, F and Coleman, MP and De Angelis, R and Faivre, J and Lutz, JM and Martinez, C and Möller, Torgil and Sankila, R},
  issn         = {1474-5488},
  language     = {eng},
  number       = {2},
  pages        = {132--140},
  publisher    = {Elsevier},
  series       = {The Lancet Oncology},
  title        = {Survival from rare cancer in adults: a population-based study},
  url          = {http://dx.doi.org/10.1016/S1470-2045(05)70471-X},
  volume       = {7},
  year         = {2006},
}