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Seltene Thorakale Tumorentitäten

Armin Frille
Published 2015 · Medicine

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ZusammenfassungHintergrund und ZielUngefähr 22 % aller Krebsdiagnosen und 21 % aller thorakalen Tumoren werden in der Europäischen Union (EU) von seltenen Entitäten eingenommen. Die Häufigkeit seltener Tumoren wird genauer durch die Inzidenz (< 6/100.000/Jahr) als durch die Prävalenz abgebildet. Ihre Seltenheit bedingt, dass evidenzbasierte Strategien zur Diagnostik, Therapie und Nachsorge noch unterrepräsentiert sind. Ziel war es, einen Überblick über seltene thorakale Tumorentitäten zu geben und ausgewählte Seltenheiten epidemiologisch und klinisch einzuordnen.Material und MethodenSeltene thorakale Tumorerkrankungen mit seinen Vertretern aus Lunge, Pleura und Thymus wurden aus Veröffentlichungen internationaler Tumorregister (z. B. RareCareNet, Rare Cancer Network) identifiziert und beschrieben.ErgebnisseIm Fokus der Betrachtung hinsichtlich Diagnostik, Therapie und Prognose standen die diffuse idiopathische pulmonale neuroendokrine Zellhyperplasie, das adenoid-zystische Karzinom der Speicheldrüsen, der kongenitale peribronchiale Myofibroblastentumor, das primär pulmonale Melanom, das lokalisierte maligne Mesotheliom und das Thymuskarzinom. Ungefähr drei Viertel aller seltenen Tumoren hatten eine Inzidenz von < 0,5/100.000/Jahr. Das relative 5-Jahres-Überleben war für seltene Tumoren geringer, was der zeitlichen Verzögerung in der Diagnosestellung, der Abwesenheit evidenzbasierter Leitlinien und dem mangelnden Therapieerfolg dieser seltenen Tumorentitäten geschuldet sein mag.SchlussfolgerungSeltene thorakale Tumoren sollten in pneumoonkologischen Zentren behandelt und an international kooperierende Krebsregister angebunden werden. In der EU steht hierfür z. B. das RareCareNet zur Verfügung, das durch den Zusammenschluss von 94 Krebsregistern aus 24 EU-Ländern seltene Entitäten epidemiologisch erfasst.AbstractBackgroundRare forms of cancer account for 22 % of all cancers and 21 % of thoracic cancers in the European Union (EU). The incidence, defined as < 6 new cases per 100,000 per year appears to be more useful to represent the frequency of rare cancers than the prevalence. Due to this rarity, evidence-based concepts for the diagnostic work-up, therapy and follow-up are still lacking. The aim of this study was to identify and to review the epidemiology and clinical manifestation of selected rare thoracic tumor entities.Material and methodsWith the help of international tumor registries (e. g. RareCareNet and Rare Cancer Network) rare tumor entities originating from the lungs, pleura and thymus were identified and reviewed.ResultsThe focus was on the following entities: idiopathic pulmonary neuroendocrine cell hyperplasia, adenoid cystic carcinoma, congenital peribronchial myofibroblastic tumor, primary pulmonary melanoma, localized malignant mesothelioma and thymic carcinoma. Approximately 75 % of all rare cancers had an incidence of < 0.5 cases per 100,000 per year. The relative 5-year survival for rare tumors was reduced due to a significant latency period in diagnosis, to a lack of evidence-based guidelines and to high mortality.ConclusionRare thoracic cancers should be treated in multidisciplinary centers for thoracic oncology and surveillance by internationally cooperating cancer registries. The European RareCareNet collects and analyzes patient data from 94 cancer registries in 24 EU countries.
This paper references
10.1177/003335491012500106
The Occurrence of Rare Cancers in U.S. Adults, 1995–2004
R. Greenlee (2010)
10.1007/978-3-642-79214-4
Kompendium Internistische Onkologie
Hans-Joachim Schmoll (1987)
10.1056/NEJM199210293271806
Brief report: idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease.
S. Aguayo (1992)
results of the IFCT-GFPC0701MAPS randomized phase 3 trial
G Zalcman
Whole exome sequencing of adenoid cysticcarcinoma
StephensPJ (2013)
10.1097/00000478-199705000-00016
Congenital pulmonary myofibroblastic tumor: a case report with cytogenetic analysis and review of the literature.
B. Alobeid (1997)
10.1016/j.ejca.2011.08.008
Rare cancers are not so rare: the rare cancer burden in Europe.
G. Gatta (2011)
Thymic carcinomawith overexpressionofmutatedKITandthe response to imatinib.NEngl
P Strobel (2004)
10.1056/NEJM200406173502523
Thymic carcinoma with overexpression of mutated KIT and the response to imatinib.
P. Stroebel (2004)
Cytogenetics of Bone and Soft Tissue Tumors
A. Sandberg (1995)
10.1002/1097-0142(19910215)67:4<1025::AID-CNCR2820670427>3.0.CO;2-F
Thymic carcinoma. A clinicopathologic study of 60 cases
S. Suster (1991)
10.1097/00008390-200512000-00008
Isolated melanoma in the lung where there is no known primary site: metastatic disease or primary lung tumour?
J. D. de Wilt (2005)
10.3322/caac.20107
Global cancer statistics
A. Jemal (2011)
10.1093/AJCP/94.5.649
Primary malignant melanoma of the lower respiratory tract. Report of a case and literature review.
T. Jennings (1990)
10.1097/00000478-198201000-00009
Melanin-containing peripheral carcinoid of the lung.
R. Grazer (1982)
10.1007/978-1-349-95810-8_652
International Agency for Research on Cancer (IARC)
V. Cogliano (2006)
10.1002/9781119196235
Textbook of Uncommon Cancer
D. Raghavan (2012)
10.1007/s10875-011-9644-1
Immune-Mediated Diseases and Immunodeficiencies Associated with Thymic Epithelial Neoplasms
M. Khawaja (2011)
Hrsg) (2010) Management of rare adult tumours
Y Belkace ́mi (2010)
10.1200/JCO.2007.13.7273
Unresectable adenoid cystic carcinoma of the trachea treated with chemoradiation.
A. Allen (2007)
10.1007/s00292-015-0030-2
Pulmonale neuroendokrine Tumoren in der neuen WHO-Klassifikation 2015
P. A. Schnabel (2015)
10.1002/9781118464557
Textbook of Uncommon Cancer: Fourth Edition
Derek Raghavan (2012)
10.1097/JTO.0000000000000393
The Impact of Thymoma Histotype on Prognosis in a Worldwide Database
C. Weis (2015)
10.4081/rt.2012.e35
The Rare Cancer Network: achievements from 1993 to 2012.
Ajaykumar B. Patel (2012)
10.1056/NEJMRA050152
Advances in malignant mesothelioma.
B. Robinson (2005)
2013)Whena thymic carcinoma“becomes
V Rossi (2016)
10.1172/JCI67201
Whole exome sequencing of adenoid cystic carcinoma.
P. Stephens (2013)
10.1097/JTO.0b013e31826915f1
Initial Analysis of the International Association For the Study of Lung Cancer Mesothelioma Database
V. Rusch (2012)
Congenital pulmonarymyofibroblastic tumor: a case report with cytogenetic analysis and review of the literature
B Alobeid (1997)
10.1200/JCO.2015.33.15_SUPPL.7500
Bevacizumab 15mg/kg plus cisplatin-pemetrexed (CP) triplet versus CP doublet in Malignant Pleural Mesothelioma (MPM): Results of the IFCT-GFPC-0701 MAPS randomized phase 3 trial.
G. Zalcman (2015)
10.1200/JCO.2003.11.136
Phase III study of pemetrexed in combination with cisplatin versus cisplatin alone in patients with malignant pleural mesothelioma.
N. Vogelzang (2003)
10.1007/978-2-287-92246-6
Management of Rare Adult Tumours
Yazid Belkacaemi (2010)
HavenerLA,HoweHL(2010)Theoccurrenceof rare cancers in U.S. adults, 1995–2004
RT Greenlee (2004)
10.1007/s00292-015-0030-2
[Pulmonary neuroendocrine tumors in the new WHO 2015 classification: Start of breaking new grounds?].
P. Schnabel (2015)
10.1016/S0022-5223(99)70469-1
Resection margins, extrapleural nodal status, and cell type determine postoperative long-term survival in trimodality therapy of malignant pleural mesothelioma: results in 183 patients.
D. Sugarbaker (1999)
10.1002/1097-0142(19811201)48:11<2485::AID-CNCR2820481123>3.0.CO;2-R
Follow‐up study of thymomas with special reference to their clinical stages
A. Masaoka (1981)
Contributors to the IRD (2015) The impact ofthymomahistotypeonprognosis inaworldwide database
CA Weis (2015)
ThompsonJF(2005)Isolatedmelanomainthelung where there is no known primary site: metastatic disease or primary lung tumour? Melanoma
JH deWilt (2005)
The rare cancernetwork: achievements
A Patel (2012)
10.1016/j.prp.2013.06.019
Association of small foci of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with adenocarcinoma of the lung.
M. Mireskandari (2013)
10.3322/caac.21262
Global cancer statistics, 2012
L. Torre (2015)
10.1097/01.pas.0000165529.78945.dc
Localized Malignant Mesothelioma
T. Allen (2005)
10.1016/j.ejca.2012.02.047
Rare thoracic cancers, including peritoneum mesothelioma.
S. Siesling (2012)
10.1007/978-3-642-60157-6
Histological Typing of Tumours of the Thymus
J. Rosai (1999)
10.1016/j.lungcan.2013.01.003
When a thymic carcinoma "becomes" a GIST.
V. Rossi (2013)



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