Foreword

I am extremely pleased to introduce the Italian Cancer Figures Report 2015, completely devoted to the assessment of the burden of rare cancers in Italy.

This report is the latest valuable contribution to knowledge and policy development in the country by the Italian Association of Cancer Registries (AIRTUM).

The Ministry of Health, through its Centre for Prevention and Disease Control, has systematically supported AIRTUM, making careful use of the wealth of information provided by AIRTUM to orient national policies and monitor their implementation.

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AIRTUM reports have been the main source of information and the benchmark for all health professionals and patient associations over the past ten years, as the share of Italian population screened by the cancer registries has increased from 23% to 51% so far, and is set to reach 70% in the next few years.

This year’s report is the first in Italy to analyse rare tumours from an epidemiological perspective. It shows that one out of four diagnosed cancer cases belongs to the category of rare cancers; this proportion is similar to other European estimates (24%).This means that the affected population is more than significant and calls for highly specific care, based on cancer patterns, as well as individual needs.

Cancers defined as “rare” challenge clinical decision making, health care organisation and clinical research due to their low frequency in the population and the resulting limited expertise available. In their daily life experience, rare cancer patients and their families must overcome a wide range of obstacles, such as: 

  • misdiagnosis and delay in diagnosis;
  • lack of scientific knowledge due to the small number of patients that may not allow for traditional clinical trial design, and limited availability of registries and tissue banks; 
  • difficulties in developing therapeutic tools and defining therapeutic strategies, and shortage of therapeutic products; 
  • low-quality healthcare due to poor, not experienced-based protocols, inappropriate referral procedures by general practitioners and misdiagnoses by inexperienced laboratories.

The most striking figure in this report relates to patient survival, which is shorter than for patients affected by more common cancers, at any evaluated period: at 1, 3, and 5 years. This is a particularly challenging figure for the National Health Service, health care professionals, and, of course and above all, patients and their families.

Italy is already committed to improving its health service delivery system in order to provide these patients with high-quality, more effective, and fairer care.

This effort will take on concrete form in a National Network for Rare Cancers which will include the most experienced cancer centres and professionals.This task is not trivial, given the current devolution and the federal structure of our national health system. This report underlines the need to overcome local interests, and provides sound, evidence-based, powerful tools to enhance scientific research and elicit new and non-traditional epidemiological, clinical, and public health analyses.

Thanks to this report we can expect an improvement in the understanding of rare tumours that will be useful not only for Italy, but also for a broader international scientific and medical audience, while supporting patient associations, strengthening their links with scientific associations, whose accountability will be key in forging a new alliance between providers and beneficiaries.

In conclusion, I would like to congratulate and thank all those who actively carried out this work and those who enthusiastically contributed to it.

To their commitment we will continue to add our own, making sure that our decisions and the related resource allocation process are based on a careful, comprehensive need assessment, so as to generate equitable care for those who might otherwise be neglected.

Ranieri Guerra
Head of Strategic direction for health prevention,
Ministry of Health

Introduction

This new monograph of the Italian Association of Cancer Registries (AIRTUM) deals with “rare” cancers, defined as those diagnosed in less than 6 every 100,000 citizens per year in the European population.

Rare cancers are a red-hot issue for all oncology stakeholders: patients, clinicians, the pharmaceutical industry, and policymakers. The reason is that their rarenessmakes diagnosismore difficult and causes a lack of specific drugs and widespread expertise in treatment.Moreover, the continuous developments in cancer research have provided new insights into cancer biology, resulting in better identification of specific oncological entities. For example, we are now able – using specific biomarkers – to identify two or several tumours, previously considered as a single type, which differ from each other in biochemical footprint, treatment, and prognosis, as well.

This is what prompted AIRTUMto address this issue, in collaboration with the ItalianNational Cancer Institute ofMilan, the “Istituto Superiore di Sanità”, and the involvement of the Italian Association ofMedical Oncology (AIOM), the Italian Federation of Volunteer-Based CancerOrganisations (FAVO), the Italian Society of Haematology (SIE), and the Italian Society of Surgical oncology (SICO).

AIRTUM’s huge database proved effective in providing highquality, reliable information even on rare cancers, with more than 330,000 cases used to compute incidence (years: 2000s) and 280,000 used to estimate survival. The study shows that about 89,000 (or 25%) of new annual cancer diagnoses in Italy are of rare cancers. The huge overall number does not solve the peculiarities of rarity: among the 198 analysed rare cancer types, more than two thirds have an incidence below 0.5 cases per 100,000 per year.

Some tumour types that are rare according to the European definition (diffuse large B-cell lymphoma, squamous cell carcinoma of larynx, multiple myeloma, hepatocellular carcinoma, and thyroid carcinoma) have an incidence in Italy that exceeds the European cut-off, introducing the need for harmonization in definition and classification to obtain reliable international comparisons.

The monograph provides an impressive amount of information on detailed types of rare tumours. To cite a few data, 5- year relative survival appears to be lower on average for rare tumours than for frequent types. The number of Italian citizens living with a rare tumour diagnosis has been estimated to be 900,000. Prevalence differs according to the different combination of incidence and survival in each of the rare tumours.

This monograph provides an invaluable amount of previously unpublished information on the epidemiology of rare tumours in Italy, both as an overall group and for each of the 198 types, which have been analysed in detail for the first time.

As is the case for all other AIRTUM reports, the data in this monograph are available to all interested stakeholders, and they focus on national peculiarities, quantifying individual burdens and outcomes, and supporting health care planners in the laying out of case-specific health care services. Rare cancers are not only relevant today, but their relevance is expected to grow in the future, pending further discoveries and major progress in ongoing cancer research.

The quality and reliability of AIRTUMdata, recently confirmed even by theMinistry of Health, support the dissemination of reliable information regarding the epidemiology of cancer in Italy, now including rare tumours.

Steering Committee
Italian Association of Cancer Registries (AIRTUM)

 

Errata corrige

English

Some errors have crept into this Monograph. As soon as possible the PDF files will be available on this website. The erratum will be published in the issue 2-2016.

Page 2. In the «Scientific contributors» section it was added the pancreas register: «Registro tumori del pancreas, SC gastroenterologia ed endoscopia digestiva, Dipartimento tecnologie avanzate, ASMN-IRCCS Reggio Emilia. Romano Sassatelli, Tiziana Cassetti, Paolo Giorgi Rossi, Massimo Vicentini».

Page 2. The affiliation referred to Emanuele Crocetti «European Commission, DG Joint Research Centre (JRC), Ispra, Varese» was changed to «Associazione Italiana Registri Tumori; Registro Tumori della Romagna, Meldola (FC)».

Page 4. In the «Acknowledgements» section it was added the statement «The present monograph has been carried out thanks to the contribution of the "Rare Cancers in Italy: surveillance and evaluation of the access to diagnosis and treatment” project, funded by the grant for research on rare diseases (Italian Ministry of Health, Directorate general for scientific and techonologic research)».

Pagina 17. The values showed in Table 3 under the «Microscopically confirmed cases (%)» column for rare tumours (in green) by macroareas were modified. See attached file.

Italiano

Sono stati riscontrati alcuni errori nella monografia. A breve verranno messi online i pdf corretti e verranno inseriti gli errata corrige nel numero 2-2016 della rivista.

Pagina 2. Nella sezione «Scientific contributors» è stato aggiunto il Registro tumori del pancreas con la seguente dicitura: «Registro tumori del pancreas, SC gastroenterologia ed endoscopia digestiva, Dipartimento tecnologie avanzate, ASMN-IRCCS Reggio Emilia. Romano Sassatelli, Tiziana Cassetti, Paolo Giorgi Rossi, Massimo Vicentini».

Pagina 2. L'affiliazione di Emanuele Crocetti «European Commission, DG Joint Research Centre (JRC), Ispra, Varese» è stata sostituita con «Associazione Italiana Registri Tumori; Registro Tumori della Romagna, Meldola (FC)».

Pagina 4. Nella sezione «Ringraziamenti» è stata aggiunta la frase: «La presente monografia è stata realizzata anche grazie al contributo del progetto “Rare Cancers in Italy: surveillance and evaluation of the access to diagnosis and treatment” finanziato dal bando di ricerca sulle malattie rare del Ministero della salute, Direzione generale della ricerca scientifica e tecnologica».

Pagina 17. I valori presenti nella tabella 3 alla colonna «Microscopically confirmed cases (%)» per i tumori rari (in verde) divisi per macroarea sono stati modificati. Vedi tabella allegata.


Indice

Introduction

p. 5
Foreword - National Centre for Disease Prevention and Control (CCM)
PREFAZIONE Centro nazionale per la prevenzione e il controllo delle malattie (CCM)
p. 7
Introduction - Italian Association of Cancer Registries (AIRTUM)
Introduzione - Associazione italiana dei registri tumori (AIRTUM)
p. 9
Abstract
Riassunto
p. 14
1 Materials and methods
Materiali e metodi
p. 22
2 The burden of rare cancers in italy
L’impatto dei tumori rari in Italia
p. 28
3 A guide to the cancer-specific data sheets
Guida alla lettura delle schede specifiche per tumore

Cancer-specific data sheets

p. 33
Epithelial tumours of head and neck
Tumori epiteliali di testa e collo
p. 39
Tumours of the eye
Tumori dell’occhio
p. 43
Rare epithelial tumours of the digestive system
Tumori rari epiteliali dell’apparato digestivo
p. 51
Rare epithelial tumours of the thoracic cavity
Tumori rari epiteliali della cavitÀ toracica
p. 56
Rare tumours of the female genital system
Tumori rari dell’apparato genitale femminile
p. 64
Rare epithelial tumours of the urinary system
Tumori rari epiteliali dell’apparato urinario
p. 69
Rare tumours of the male genital system
Tumori rari dell’apparato genitale maschile
p. 76
Rare skin tumours and malignant melanoma of mucosa
Tumori rari della pelle e melanoma maligno mucoso
p. 80
Embryonal tumours
Tumori embrionali
p. 84
Sarcomas
Sarcomi
p. 90
Neuroendocrine tumours
Tumori neuroendocrini
p. 94
Tumours of the central nervous system
Tumori del sistema nervoso centrale
p. 98
Tumours of the endocrine organs
Tumori degli organi endocrini
p. 101
Rare haematological diseases/malattie ematologiche rare comments from other scientific associations and patients’ organisations
Commenti da altre associazioni scientifiche e di pazienti

Comments

p. 110
The role of cancer patient organisations as advocates for people with rare types of cancer favo
Il ruolo delle organizzazioni di pazienti oncologici in difesa di chi è affetto da tumore raro
p. 111
Necessary steps to cope with rare cancers in italy aiom
Passi necessari per affrontare i tumori rari in italia
p. 113
Registering rare haematological tumours: it is time for a breakthrough! sie
La registrazione dei tumori ematologici rari: È tempo di una svolta!
p. 115
Defining criteria to build a national excellence network sico
Definire i criteri per costruire una rete nazionale di eccellenza
p. 116
The italian rare cancer network
La rete italiana dei tumori rari

The network of the airtum cancer registries

p. 117
Staff, contacts, acknowledgements
 10/02/2016      Visite