Rare cancers represent 22% of all tumors in Europe; however, the quality of the data of rare cancers may not be as good as the quality of data for common cancer. The project surveillance of rare cancers in Europe (RARECARE) had, among others, the objective of assessing rare cancer data quality in population-based cancer registries (CRs). Eight rare cancers were considered: mesothelioma, liver angiosarcoma, sarcomas, tumors of oral cavity, CNS tumors, germ cell tumors, leukemia, and malignant digestive endocrine tumors.
We selected data on 18,000 diagnoses and revised, on the basis of the pathologic and clinical reports (but not on pathologic specimens), unspecified morphology and topography codes originally attributed by CR officers and checked the quality of follow-up of long-term survivors of poor prognosis cancers.
A total of 38 CRs contributed from 13 European countries. The majority of unspecified morphology and topography cases were confirmed as unspecified. The few unspecified cases that, after the review, changed to a more specific diagnosis increased the incidence of the common cancer histotypes. For example, 11% of the oral cavity epithelial cancers were reclassified from unspecified to more specific diagnoses: 8% were reclassified as squamous cell carcinoma (commoner) and only 1% as adenocarcinoma (rarer). The revision confirmed the majority of long-term survivors revealing a relative high proportion of mesothelioma long-term survivors. The majority of appendix carcinoids changed behavior from malignant to borderline lesions.
Our study suggests that the problem of poorly specified morphology and topography cases is mainly one of difficulty in reaching a precise diagnosis. The awareness of the importance of data quality for rare cancers should increase among registrars, pathologists, and clinicians.
Tumori 2017; 103(1): 22 - 32
Article Type: ORIGINAL RESEARCH ARTICLE
AuthorsAnnalisa Trama, Rafael Marcos-Gragera, Maria Josè Sánchez Pérez, Jan Maarten van der Zwan, Eva Ardanaz, Christine Bouchardy, Juan Manuel Melchor, Carmen Martinez, Riccardo Capocaccia, Massimo Vicentini, Sabine Siesling, Gemma Gatta, and the RARECARE working group contributing to the data quality study
- • Accepted on 10/08/2016
- • Available online on 03/10/2016
- • Published in print on 21/01/2017
This article is available as full text PDF.
- Trama, Annalisa [PubMed] [Google Scholar] 1, * Corresponding Author (email@example.com)
- Marcos-Gragera, Rafael [PubMed] [Google Scholar] 2
- Sánchez Pérez, Maria Josè [PubMed] [Google Scholar] 3, 4
- van der Zwan, Jan Maarten [PubMed] [Google Scholar] 5
- Ardanaz, Eva [PubMed] [Google Scholar] 4, 6
- Bouchardy, Christine [PubMed] [Google Scholar] 7
- Melchor, Juan Manuel [PubMed] [Google Scholar] 3
- Martinez, Carmen [PubMed] [Google Scholar] 3
- Capocaccia, Riccardo [PubMed] [Google Scholar] 8
- Vicentini, Massimo [PubMed] [Google Scholar] 9
- Siesling, Sabine [PubMed] [Google Scholar] 5, 10
- Gatta, Gemma [PubMed] [Google Scholar] 1
- and the RARECARE working group contributing to the data quality study
Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan - Italy
Epidemiology Unit and Girona Cancer Registry, Oncology Coordination Plan, Department of Health, Girona Biomedical Research Institute, Girona - Spain
Escuela Andaluza de Salud Pública. Instituto de Investigación Biosanitaria ibs.GRANADA. Hospitales Universitarios de Granada/Universidad de Granada, Granada - Spain
CIBER de Epidemiología y Salud Pública (CIBERESP) - Spain
Department of Research, Comprehensive Cancer Centre The Netherlands, Utrecht - The Netherlands
Instituto de Salud Pública de Navarra, Pamplona - Spain
Geneva Cancer Registry, Institute of Global Health, Faculty of Medicine, University of Geneva, Geneva - Switzerland
Centro Nazionale di Epidemiologia, Sorveglianza e Promozione della Salute (CNESPS), Istituto Superiore di Sanità, Rome - Italy
Servizio Interaziendale di Epidemiologia, Azienda Unità Sanitaria Locale and IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia - Italy
Department of Health Technology and services research, MIRA Institute for Biomedical Technology and Technical Medicine, University of Twente, Enschede - The Netherlands