Elsevier

European Journal of Cancer

Volume 48, Issue 17, November 2012, Pages 3257-3266
European Journal of Cancer

Incidence, survival and prevalence of myeloid malignancies in Europe

https://doi.org/10.1016/j.ejca.2012.05.024Get rights and content

Abstract

Background

The Surveillance of Rare Cancers in Europe (RARECARE) project aims at increasing knowledge of rare cancers in Europe. This manuscript describes the epidemiology of myeloid malignancies (MMs), taking into account the morphological characterisation of these tumours.

Methods

We used data gathered by RARECARE on cancer patients diagnosed from 1995 to 2002 and archived in 64 European population-based cancer registries, followed up to 31st December 2003 or later.

Results

The overall annual crude incidence of MMs was 8.6 per 100,000. Acute myeloid leukaemia (AML) and myeloproliferative neoplasms (MPN) were most common, with incidence rates of 3.7 and 3.1 per 100,000 year respectively, followed by 1.8 for myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MD/MPN) and 0.1 for histiocytic and dendritic cell neoplasms (HDCN). The 5-year relative survival rate ranged from 18% for chronic myelomonocytic leukaemia, 19% for AML, 29% for MDS and 44% for chronic myeloid leukaemia to relatively favourable rates for MPN (62%) and HDCN (83%). Total number of new cases of MMs in the EU27 is estimated at 43,000 annually, total number of prevalent cases (1st January 2008) at 189,000 cases.

Conclusion

MMs form a large variety of rare entities with specific characteristics. Collection of detailed information (immunophenotype, genetic abnormalities, molecular data and clinical data) and an up-to-date classification system is essential for their surveillance, especially now that more and more targeted therapies are being introduced.

Introduction

Haematological malignancies (HMs) are thought to derive from a pluripotential or multipotential stem cell that gives rise to very diverse proliferations, some of them being characterised by specific genetic abnormalities.1, 2 Changing definitions and classifications of HMs complicate epidemiological studies and comparative analyses of incidence, survival and prevalence of these diseases.

In the past, the generic term “leukaemia” was used to group those types of lymphoid and myeloid proliferations which mainly involved the peripheral blood, subdivided into chronic and acute leukaemia. In the third edition of the International Classification of Diseases for Oncology (ICD-O-3),3 HMs are divided into two main categories based on cell lineage − myeloid and lymphoid. Within each of these groups, HMs are further sub-divided according to cell of origin, extent of maturation, morphology, immunohistochemistry, genetic characteristics and clinical behaviour.

This disease grouping is more useful for epidemiologic and public health studies on incidence, survival and prevalence and for testing aetiological hypotheses, because these groups are likely to have a distinct physiopathology and prognosis, and are more compatible with clinical classifications than the broad categories used by cancer registries (CRs) in the past.

In 2008, 78,416 cases of leukaemia - including both lymphoid and myeloid leukaemia - were estimated in Europe by the GLOBOCAN project, accounting for approximately 2.4% of all cancers.4 The overall age standardised (world population) incidence rate of leukaemia was 6.8 per 100,000 (crude rate: 10.7).4 Figures by morphological subtype were recently provided for the 20 European countries represented in the cancer registry-based project on Haematological malignancies (HAEMACARE), in which the age-standardised incidence rate per 100,000 was 7.5 for myeloid malignancies (MMs) and 24.5 for lymphoid malignancies.5

The aim of the present study is to provide estimates of incidence, prevalence and survival for the MMs in Europe according to the grouping proposed by the project Surveillance of Rare Cancers in Europe (RARECARE). In Europe there was no internationally agreed definition of rare cancers thus RARECARE has provided a definition of rare cancers and a list of rare entities.6 An international group of experts agreed that rare cancers are those that present specific problems in clinical decision making, health care organisation and clinical research because of their low frequency. Thus, the definition of rare cancers was based on their frequency and rare cancers were defined as those with an annual incidence below six per 100,000. According to this definition all MMs are actually rare.

Section snippets

Material and methods

The present analyses are based on the list of cancers provided by RARECARE.6 The grouping of rare cancers was based on the ICD-O-3 classification system.3 However, the groups of tumours were identified not only by considering their morphology, but also by aggregating single tumours to clinically meaningful groups (i.e. perceived by clinicians as single diseases). Such grouping is more relevant for clinical decision making and research. The RARECARE list classifies cancer entities into a

Results

Table 1 shows the main data quality indicators for the MMs diagnosed in 1995–2002. The highest proportions of cases notified by death certificate only were found for AML and related precursor neoplasms (4.6%), CML (4.6%) and for MDS (3.7%). For all other diseases the proportions did not exceed 4.0%. Overall, 0.3% of these cases were diagnosed at autopsy. The proportion of cases with less than 5 years of follow-up (censored cases) was highest for the category of HDCN (4.5%) and did not exceed 1.4

Discussion

The availability of the RARECARE database allowed us to estimate incidence, survival and prevalence of MMs in Europe. Due to variations in registration rules between registries and continuous developments in the classification of MMs, some registration issues might have affected our estimates.

Incidence rates in Europe for AML, CML and CMML are not very different from the rates in the United States America (USA) as reported by Surveillance Epidemiology and End Results (SEER).12 Rates for MDS and

Conflict of interest statement

The authors declare no conflict of interest. The founding sources had no role in study design, data collection, data analysis, data interpretation, in writing this report, or in the decision to submit for publication.

Acknowledgements

Funding: This research was supported by the European Commission through the Executive Agency for Health and Consumers (grant No 2006113), and the Programma Italia-USA Malattie Rare (grant No. 526D/42).

The Childhood Cancer Research Group receives funding from the Department of Health, the National Cancer Intelligence Network, the Scottish Executive and Children with Cancer UK. The views expressed here are those of the authors and not necessarily those of the Department of Health, the National

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