Survival from rare cancer in adults

A population-based study

Gemma Gatta, Laura Ciccolallo, Ian Kunkler, Riccardo Capocaccia, Franco Berrino, Michel P. Coleman, Roberta De Angelis, Jean Faivre, Jean Michel Lutz, Carmen Martinez, Torgil Möller, Risto Sankila, W. Oberaigner, H. H. Storm, T. Aareleid, M. Jechova, M. Rousarova, T. Hakulinen, G. Hédelin, I. Tron & 97 others E. Le Gall, G. Launoy, J. Macé-Lesec'h, G. Chaplain, P. M. Carli, A. Danzon, B. Tretarre, M. Colonna, B. Lacour, N. Raverdy, C. Berger, B. Freycon, P. Grosclaude, J. Estève, P. Kaatsch, H. Ziegler, D. Hölzel, G. Schubert Fritschle, L. Tryggvadottir, C. Allemani, P. Baili, P. Crosignani, A. Micheli, M. Sant, E. Taussig, S. Sowe, S. Ferretti, E. Conti, M. Vercelli, A. Quaglia, F. Pannelli, M. Federico, M. E. Artioli, M. Ponz De Leon, P. Benatti, V. De Lisi, L. Servente, R. Zanetti, S. Patriarca, C. Magnani, G. Pastore, L. Gafa, R. Tumino, F. Falcini, M. Budroni, E. Paci, E. Crocetti, P. Zambon, S. Guzzinati, E. Carrani, P. Roazzi, M. Santaquilani, A. Tavilla, F. Valente, A. Verdecchia, M. Dalmas, F. Langmark, A. Andersen, Paulo Pinheiro, J. Rachtan, M. Bielska-Lasota, Z. Wronkowski, M. Zwierko, I. Pleško, A. Obsitníkováa, V. Pompe-Kirn, M. Primic-Zakelj, I. Izarzugaza, C. Martinez-Garcia, I. Garau, C. Navarro, M. D. Chirlaque, E. Ardanaz, C. Moreno, J. Galceran, A. Torrella, R. Peris-Bonet, L. Barlow, G. Jundt, C. Bouchardy, J. W.W. Coebergh, A. van der Does-van den Berg, O. Visser, S. Godward, E. M.I. Williams, D. Forman, M. J. Quinn, M. Roche, S. Edwards, C. Stiller, J. Verne, H. Møller, J. Bell, H. Botha, G. Lawrence, R. Black, J. A. Steward

Research output: Contribution to journalArticle

82 Citations (Scopus)

Abstract

Backround: 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.

Original languageEnglish (US)
Pages (from-to)132-140
Number of pages9
JournalLancet Oncology
Volume7
Issue number2
DOIs
StatePublished - Feb 1 2006
Externally publishedYes

Fingerprint

Population
Survival
Neoplasms
Sarcoma
Squamous Cell Carcinoma
Hemangiosarcoma
Antineoplastic Protocols
Digestive System Neoplasms
Endocrine Gland Neoplasms
Extremities
Digestive System
Choriocarcinoma
Quality of Health Care
Liver
Adrenal Cortex
Mesothelioma
Testicular Neoplasms
Carcinoid Tumor
Adrenal Glands
Practice Guidelines

ASJC Scopus subject areas

  • Oncology

Cite this

Gatta, G., Ciccolallo, L., Kunkler, I., Capocaccia, R., Berrino, F., Coleman, M. P., ... Steward, J. A. (2006). Survival from rare cancer in adults: A population-based study. Lancet Oncology, 7(2), 132-140. https://doi.org/10.1016/S1470-2045(05)70471-X

Survival from rare cancer in adults : A population-based study. / Gatta, Gemma; Ciccolallo, Laura; Kunkler, Ian; Capocaccia, Riccardo; Berrino, Franco; Coleman, Michel P.; De Angelis, Roberta; Faivre, Jean; Lutz, Jean Michel; Martinez, Carmen; Möller, Torgil; Sankila, Risto; Oberaigner, W.; Storm, H. H.; Aareleid, T.; Jechova, M.; Rousarova, M.; Hakulinen, T.; Hédelin, G.; Tron, I.; Le Gall, E.; Launoy, G.; Macé-Lesec'h, J.; Chaplain, G.; Carli, P. M.; Danzon, A.; Tretarre, B.; Colonna, M.; Lacour, B.; Raverdy, N.; Berger, C.; Freycon, B.; Grosclaude, P.; Estève, J.; Kaatsch, P.; Ziegler, H.; Hölzel, D.; Schubert Fritschle, G.; Tryggvadottir, L.; Allemani, C.; Baili, P.; Crosignani, P.; Micheli, A.; Sant, M.; Taussig, E.; Sowe, S.; Ferretti, S.; Conti, E.; Vercelli, M.; Quaglia, A.; Pannelli, F.; Federico, M.; Artioli, M. E.; Ponz De Leon, M.; Benatti, P.; De Lisi, V.; Servente, L.; Zanetti, R.; Patriarca, S.; Magnani, C.; Pastore, G.; Gafa, L.; Tumino, R.; Falcini, F.; Budroni, M.; Paci, E.; Crocetti, E.; Zambon, P.; Guzzinati, S.; Carrani, E.; Roazzi, P.; Santaquilani, M.; Tavilla, A.; Valente, F.; Verdecchia, A.; Dalmas, M.; Langmark, F.; Andersen, A.; Pinheiro, Paulo; Rachtan, J.; Bielska-Lasota, M.; Wronkowski, Z.; Zwierko, M.; Pleško, I.; Obsitníkováa, A.; Pompe-Kirn, V.; Primic-Zakelj, M.; Izarzugaza, I.; Martinez-Garcia, C.; Garau, I.; Navarro, C.; Chirlaque, M. D.; Ardanaz, E.; Moreno, C.; Galceran, J.; Torrella, A.; Peris-Bonet, R.; Barlow, L.; Jundt, G.; Bouchardy, C.; Coebergh, J. W.W.; van der Does-van den Berg, A.; Visser, O.; Godward, S.; Williams, E. M.I.; Forman, D.; Quinn, M. J.; Roche, M.; Edwards, S.; Stiller, C.; Verne, J.; Møller, H.; Bell, J.; Botha, H.; Lawrence, G.; Black, R.; Steward, J. A.

In: Lancet Oncology, Vol. 7, No. 2, 01.02.2006, p. 132-140.

Research output: Contribution to journalArticle

Gatta, G, Ciccolallo, L, Kunkler, I, Capocaccia, R, Berrino, F, Coleman, MP, De Angelis, R, Faivre, J, Lutz, JM, Martinez, C, Möller, T, Sankila, R, Oberaigner, W, Storm, HH, Aareleid, T, Jechova, M, Rousarova, M, Hakulinen, T, Hédelin, G, Tron, I, Le Gall, E, Launoy, G, Macé-Lesec'h, J, Chaplain, G, Carli, PM, Danzon, A, Tretarre, B, Colonna, M, Lacour, B, Raverdy, N, Berger, C, Freycon, B, Grosclaude, P, Estève, J, Kaatsch, P, Ziegler, H, Hölzel, D, Schubert Fritschle, G, Tryggvadottir, L, Allemani, C, Baili, P, Crosignani, P, Micheli, A, Sant, M, Taussig, E, Sowe, S, Ferretti, S, Conti, E, Vercelli, M, Quaglia, A, Pannelli, F, Federico, M, Artioli, ME, Ponz De Leon, M, Benatti, P, De Lisi, V, Servente, L, Zanetti, R, Patriarca, S, Magnani, C, Pastore, G, Gafa, L, Tumino, R, Falcini, F, Budroni, M, Paci, E, Crocetti, E, Zambon, P, Guzzinati, S, Carrani, E, Roazzi, P, Santaquilani, M, Tavilla, A, Valente, F, Verdecchia, A, Dalmas, M, Langmark, F, Andersen, A, Pinheiro, P, Rachtan, J, Bielska-Lasota, M, Wronkowski, Z, Zwierko, M, Pleško, I, Obsitníkováa, A, Pompe-Kirn, V, Primic-Zakelj, M, Izarzugaza, I, Martinez-Garcia, C, Garau, I, Navarro, C, Chirlaque, MD, Ardanaz, E, Moreno, C, Galceran, J, Torrella, A, Peris-Bonet, R, Barlow, L, Jundt, G, Bouchardy, C, Coebergh, JWW, van der Does-van den Berg, A, Visser, O, Godward, S, Williams, EMI, Forman, D, Quinn, MJ, Roche, M, Edwards, S, Stiller, C, Verne, J, Møller, H, Bell, J, Botha, H, Lawrence, G, Black, R & Steward, JA 2006, 'Survival from rare cancer in adults: A population-based study', Lancet Oncology, vol. 7, no. 2, pp. 132-140. https://doi.org/10.1016/S1470-2045(05)70471-X
Gatta G, Ciccolallo L, Kunkler I, Capocaccia R, Berrino F, Coleman MP et al. Survival from rare cancer in adults: A population-based study. Lancet Oncology. 2006 Feb 1;7(2):132-140. https://doi.org/10.1016/S1470-2045(05)70471-X
Gatta, Gemma ; Ciccolallo, Laura ; Kunkler, Ian ; Capocaccia, Riccardo ; Berrino, Franco ; Coleman, Michel P. ; De Angelis, Roberta ; Faivre, Jean ; Lutz, Jean Michel ; Martinez, Carmen ; Möller, Torgil ; Sankila, Risto ; Oberaigner, W. ; Storm, H. H. ; Aareleid, T. ; Jechova, M. ; Rousarova, M. ; Hakulinen, T. ; Hédelin, G. ; Tron, I. ; Le Gall, E. ; Launoy, G. ; Macé-Lesec'h, J. ; Chaplain, G. ; Carli, P. M. ; Danzon, A. ; Tretarre, B. ; Colonna, M. ; Lacour, B. ; Raverdy, N. ; Berger, C. ; Freycon, B. ; Grosclaude, P. ; Estève, J. ; Kaatsch, P. ; Ziegler, H. ; Hölzel, D. ; Schubert Fritschle, G. ; Tryggvadottir, L. ; Allemani, C. ; Baili, P. ; Crosignani, P. ; Micheli, A. ; Sant, M. ; Taussig, E. ; Sowe, S. ; Ferretti, S. ; Conti, E. ; Vercelli, M. ; Quaglia, A. ; Pannelli, F. ; Federico, M. ; Artioli, M. E. ; Ponz De Leon, M. ; Benatti, P. ; De Lisi, V. ; Servente, L. ; Zanetti, R. ; Patriarca, S. ; Magnani, C. ; Pastore, G. ; Gafa, L. ; Tumino, R. ; Falcini, F. ; Budroni, M. ; Paci, E. ; Crocetti, E. ; Zambon, P. ; Guzzinati, S. ; Carrani, E. ; Roazzi, P. ; Santaquilani, M. ; Tavilla, A. ; Valente, F. ; Verdecchia, A. ; Dalmas, M. ; Langmark, F. ; Andersen, A. ; Pinheiro, Paulo ; Rachtan, J. ; Bielska-Lasota, M. ; Wronkowski, Z. ; Zwierko, M. ; Pleško, I. ; Obsitníkováa, A. ; Pompe-Kirn, V. ; Primic-Zakelj, M. ; Izarzugaza, I. ; Martinez-Garcia, C. ; Garau, I. ; Navarro, C. ; Chirlaque, M. D. ; Ardanaz, E. ; Moreno, C. ; Galceran, J. ; Torrella, A. ; Peris-Bonet, R. ; Barlow, L. ; Jundt, G. ; Bouchardy, C. ; Coebergh, J. W.W. ; van der Does-van den Berg, A. ; Visser, O. ; Godward, S. ; Williams, E. M.I. ; Forman, D. ; Quinn, M. J. ; Roche, M. ; Edwards, S. ; Stiller, C. ; Verne, J. ; Møller, H. ; Bell, J. ; Botha, H. ; Lawrence, G. ; Black, R. ; Steward, J. A. / Survival from rare cancer in adults : A population-based study. In: Lancet Oncology. 2006 ; Vol. 7, No. 2. pp. 132-140.
@article{30638c3694e6434aa4f198c3121542f0,
title = "Survival from rare cancer in adults: A population-based study",
abstract = "Backround: 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 = "Gemma Gatta and Laura Ciccolallo and Ian Kunkler and Riccardo Capocaccia and Franco Berrino and Coleman, {Michel P.} and {De Angelis}, Roberta and Jean Faivre and Lutz, {Jean Michel} and Carmen Martinez and Torgil M{\"o}ller and Risto Sankila and W. Oberaigner and Storm, {H. H.} and T. Aareleid and M. Jechova and M. Rousarova and T. Hakulinen and G. H{\'e}delin and I. Tron and {Le Gall}, E. and G. Launoy and J. Mac{\'e}-Lesec'h and G. Chaplain and Carli, {P. M.} and A. Danzon and B. Tretarre and M. Colonna and B. Lacour and N. Raverdy and C. Berger and B. Freycon and P. Grosclaude and J. Est{\`e}ve and P. Kaatsch and H. Ziegler and D. H{\"o}lzel and {Schubert Fritschle}, G. and L. Tryggvadottir and C. Allemani and P. Baili and P. Crosignani and A. Micheli and M. Sant and E. Taussig and S. Sowe and S. Ferretti and E. Conti and M. Vercelli and A. Quaglia and F. Pannelli and M. Federico and Artioli, {M. E.} and {Ponz De Leon}, M. and P. Benatti and {De Lisi}, V. and L. Servente and R. Zanetti and S. Patriarca and C. Magnani and G. Pastore and L. Gafa and R. Tumino and F. Falcini and M. Budroni and E. Paci and E. Crocetti and P. Zambon and S. Guzzinati and E. Carrani and P. Roazzi and M. Santaquilani and A. Tavilla and F. Valente and A. Verdecchia and M. Dalmas and F. Langmark and A. Andersen and Paulo Pinheiro and J. Rachtan and M. Bielska-Lasota and Z. Wronkowski and M. Zwierko and I. Pleško and A. Obsitn{\'i}kov{\'a}a and V. Pompe-Kirn and M. Primic-Zakelj and I. Izarzugaza and C. Martinez-Garcia and I. Garau and C. Navarro and Chirlaque, {M. D.} and E. Ardanaz and C. Moreno and J. Galceran and A. Torrella and R. Peris-Bonet and L. Barlow and G. Jundt and C. Bouchardy and Coebergh, {J. W.W.} and {van der Does-van den Berg}, A. and O. Visser and S. Godward and Williams, {E. M.I.} and D. Forman and Quinn, {M. J.} and M. Roche and S. Edwards and C. Stiller and J. Verne and H. M{\o}ller and J. Bell and H. Botha and G. Lawrence and R. Black and Steward, {J. A.}",
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month = "2",
day = "1",
doi = "10.1016/S1470-2045(05)70471-X",
language = "English (US)",
volume = "7",
pages = "132--140",
journal = "The Lancet Oncology",
issn = "1470-2045",
publisher = "Lancet Publishing Group",
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}

TY - JOUR

T1 - Survival from rare cancer in adults

T2 - A population-based study

AU - Gatta, Gemma

AU - Ciccolallo, Laura

AU - Kunkler, Ian

AU - Capocaccia, Riccardo

AU - Berrino, Franco

AU - Coleman, Michel P.

AU - De Angelis, Roberta

AU - Faivre, Jean

AU - Lutz, Jean Michel

AU - Martinez, Carmen

AU - Möller, Torgil

AU - Sankila, Risto

AU - Oberaigner, W.

AU - Storm, H. H.

AU - Aareleid, T.

AU - Jechova, M.

AU - Rousarova, M.

AU - Hakulinen, T.

AU - Hédelin, G.

AU - Tron, I.

AU - Le Gall, E.

AU - Launoy, G.

AU - Macé-Lesec'h, J.

AU - Chaplain, G.

AU - Carli, P. M.

AU - Danzon, A.

AU - Tretarre, B.

AU - Colonna, M.

AU - Lacour, B.

AU - Raverdy, N.

AU - Berger, C.

AU - Freycon, B.

AU - Grosclaude, P.

AU - Estève, J.

AU - Kaatsch, P.

AU - Ziegler, H.

AU - Hölzel, D.

AU - Schubert Fritschle, G.

AU - Tryggvadottir, L.

AU - Allemani, C.

AU - Baili, P.

AU - Crosignani, P.

AU - Micheli, A.

AU - Sant, M.

AU - Taussig, E.

AU - Sowe, S.

AU - Ferretti, S.

AU - Conti, E.

AU - Vercelli, M.

AU - Quaglia, A.

AU - Pannelli, F.

AU - Federico, M.

AU - Artioli, M. E.

AU - Ponz De Leon, M.

AU - Benatti, P.

AU - De Lisi, V.

AU - Servente, L.

AU - Zanetti, R.

AU - Patriarca, S.

AU - Magnani, C.

AU - Pastore, G.

AU - Gafa, L.

AU - Tumino, R.

AU - Falcini, F.

AU - Budroni, M.

AU - Paci, E.

AU - Crocetti, E.

AU - Zambon, P.

AU - Guzzinati, S.

AU - Carrani, E.

AU - Roazzi, P.

AU - Santaquilani, M.

AU - Tavilla, A.

AU - Valente, F.

AU - Verdecchia, A.

AU - Dalmas, M.

AU - Langmark, F.

AU - Andersen, A.

AU - Pinheiro, Paulo

AU - Rachtan, J.

AU - Bielska-Lasota, M.

AU - Wronkowski, Z.

AU - Zwierko, M.

AU - Pleško, I.

AU - Obsitníkováa, A.

AU - Pompe-Kirn, V.

AU - Primic-Zakelj, M.

AU - Izarzugaza, I.

AU - Martinez-Garcia, C.

AU - Garau, I.

AU - Navarro, C.

AU - Chirlaque, M. D.

AU - Ardanaz, E.

AU - Moreno, C.

AU - Galceran, J.

AU - Torrella, A.

AU - Peris-Bonet, R.

AU - Barlow, L.

AU - Jundt, G.

AU - Bouchardy, C.

AU - Coebergh, J. W.W.

AU - van der Does-van den Berg, A.

AU - Visser, O.

AU - Godward, S.

AU - Williams, E. M.I.

AU - Forman, D.

AU - Quinn, M. J.

AU - Roche, M.

AU - Edwards, S.

AU - Stiller, C.

AU - Verne, J.

AU - Møller, H.

AU - Bell, J.

AU - Botha, H.

AU - Lawrence, G.

AU - Black, R.

AU - Steward, J. A.

PY - 2006/2/1

Y1 - 2006/2/1

N2 - Backround: 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.

AB - Backround: 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.

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U2 - 10.1016/S1470-2045(05)70471-X

DO - 10.1016/S1470-2045(05)70471-X

M3 - Article

VL - 7

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EP - 140

JO - The Lancet Oncology

JF - The Lancet Oncology

SN - 1470-2045

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