Zusammenfassung
Hintergrund und Ziel
Ungefähr 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 Methoden
Seltene thorakale Tumorerkrankungen mit seinen Vertretern aus Lunge, Pleura und Thymus wurden aus Veröffentlichungen internationaler Tumorregister (z. B. RareCareNet, Rare Cancer Network) identifiziert und beschrieben.
Ergebnisse
Im 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.
Schlussfolgerung
Seltene 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.
Abstract
Background
Rare 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 methods
With 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.
Results
The 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.
Conclusion
Rare 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.
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Frille, A. Seltene thorakale Tumorentitäten. Pneumologe 13, 33–42 (2016). https://doi.org/10.1007/s10405-015-0012-3
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DOI: https://doi.org/10.1007/s10405-015-0012-3