This Joint Action on Rare Cancers (JARC) will be aimed at:
1. prioritising rare cancers (RCs) in the agenda of the Europe (EU) and Member States;
2. developing innovative and shared solutions for European Reference Networks (ERNs) on RCs, in the areas of quality of care, innovation, education and state of the art definition on prevention, diagnosis and treatment.
The objectives of JARC will be achieved by creating a platform for competent national authorities as well as institutions, scientific and professional societies and patient organisations, to produce consensus-based recommendations, with a special view to the new ERNs, seen as a great opportunity for improvement of RC patient outcomes in the EU.
Following the results of the RARECARE project, all the 12 families of RCs will be considered. Consensus-based recommendations about RCs will be provided to improve: 1) epidemiological surveillance of RCs; 2) quality of healthcare, primarily through the new ERNs; 3) the availability of clinical practice guidelines on RCs; 4) innovation, also by optimizing clinical research regulations as well as practices and semantics regarding patient data and tissues; 5) medical and patient education; 6) health policy measures on RCs at the EU and national level; 7) patient empowerment (which will be pursued across all items dealt with by JARC). All EU stakeholders in the field of RCs and rare diseases will be involved.
JARC will carry forward the aims of the Third Health Programme by improving chances of EU RC patients to have access to appropriate healthcare, primarily through optimal shaping of ERNs. Thus, all this should result in reduced healthcare inequalities, increased innovation in health, increased sustainability of health systems, decreased health migration and reduction of gaps in rare cancers survival across EU countries.
The Joint Action on Rare Cancers (JARC) is aimed to integrate and maximize efforts of the European Union (EU) Commission, EU Member States and all stakeholders to advance quality of care and research on rare cancers (RCs). According to the results of the RARECARE project, around 4 million people in the EU are affected by RCs. There about 200 different rare cancers which can be grouped in the following major families: head and neck cancers, thoracic rare cancers, male genital and urogenital rare cancers, female genital rare cancers, neuroendocrine tumours, tumours of the endocrine organs, central nervous system tumours, digestive rare cancers, rare skin cancers including uveal melanoma, haematological rare malignancies, all paediatric cancers.
The public health challenge posed by rare cancers combines both the typical problems of rare diseases (such as the limited professional expertise multidisciplinary in the community, or the difficulties in clinical research) and those of cancer, with the need of a timely and appropriate diagnosis and optimal treatment from the very beginning of the patient’s journey. An accurate clinical, pathologic and biological assessment of the disease of the individual patient is key to survival and cure, as well as an expert clinical decision provided by a multidisciplinary team. To this end, proper referral of patients and effective clinical networking are crucial in rare cancers. This is the main reason why JARC decided to shape its efforts around the new European Reference Networks (ERNs). European Reference Networks, three of which are specifically devoted to rare cancers, have been conceived by the EU Commission as a means to provide “highly specialised healthcare for rare or low-prevalence complex diseases”. The formal activation of ERNs is a cornerstone in the EU cooperation on rare cancers, and this Joint Action will be instrumental to make them grow up the best way possible.
In fact, JARC aims at optimizing the process of creation of the ERNs, by providing them with performance indicators/criteria operational solutions and professional guidance in the areas of quality of care, epidemiology, research and innovation, education and state of the art definition on prevention, diagnosis and treatment of rare cancers.
The Istituto Nazionale Tumori, with a long tradition on several rare cancers, has the privilege to serve as the coordinator of this EU Joint Action armed with the collaboration of 34 partners from different MS. The Member States involved in the present JA ensure the coverage of all European regions: Western Europe (France, Belgium), Central Europe (Czech Republic, Germany, Hungary), Eastern Europe (Poland, Romania), Northern Europe (Sweden, Finland, Norway, Ireland, United Kingdom, and Lithuania) and Southern Europe (Cyprus, Croatia, Greece, Italy, Spain).
The JARC is structured in 10 work packages (WPs): coordination, dissemination, evaluation (as any EU project), epidemiology, quality of care, clinical practice guidelines, innovation and access to innovation, medical education, childhood cancers and, rare cancers policy. Patients are represented across all work packages, driving the JARC efforts along the needs of the only end users of all what we can do, in care and research as well.
The WPs work include reviews of scientific and lay literature on RCs, innovative ways of ad hoc collection and analyses of data pertaining to RCs, discussion with experts. The most important requirement is to seek the widest consensus possible among all relevant stakeholders.
Improving epidemiological surveillance of rare cancers in the EU
• A consensus paper about the rationale of the rare cancer list was developed based on a consensus meeting.
• In collaboration with the European Network of Cancer Registries (ENCR), draft recommendations were defined for rare cancers with registration problems and without existing registration guidelines (sarcoma, including gastrointestinal stromal tumour (GIST) and neuroendocrine tumours). A pilot study was performed to assess the feasibility for cancer registries to collect stage for rare cancers taking the example of childhood cancers.
• Recommendations for the standardized estimation of rare cancers indicators at the European and country level were discussed and finalised.
Identifying standards of care for all families of rare cancers to ensure sharing of best practices and equality of care for rare cancers across Europe, particularly through clinical networking
• The existing networks of care for all 11 families of rare adult cancers, across all Member States (MS) were mapped to identify gaps in current scenarios.
• After a literature review on existing care networks, and also an in-depth comparative study of other cancer accreditation standards, a draft of the Quality Standards for Rare Cancer Networks has been achieved with inputs from Partners in the JARC, EURACAN, EURORDIS, ECPC. A first consensus meeting was to discuss the draft with a wider community of experts. The draft was also shared with the ERN Monitoring and Evaluation working group.
Clinical practice guidelines
• A review of clinical practice guidelines (CPG) available at the national and international level on all families of rare cancers and a quality assessment of oncology guidelines identified has been finalised. Out of the 537 guidelines produced 228 full text guidelines were selected for quality evaluation based on the Cluzeau instrument and the binary scored instrument, iCAHE (international Centre for Allied Health Evidence).
Rare cancers translational research landscape
• Recommendations on standardization of clinical data and patient-centred clinical outcomes for big data optimal exploitation and connection to virtual bio banks and quality assurance/quality control (QA/QC) in regard to biological samples, data collection and molecular screening were produced.
• Recommendations to solve regulatory and organisational challenges in conducting international investigator-driven collaborative clinical trials on rare cancers were identified.
• Education materials and training opportunities were identified for professionals (6449 review papers; 37 life events; 13 links and 41 books were collected) and for patients in collaboration with EURORDIS, ECPC and CCI Europe.
• A survey to collect information on pre and post-graduate courses dedicated to rare cancers was completed. The survey targeted European Union of Medical Specialists, European Board of Medical Genetics, European Society of Human Genetics and JARC partners.
• Availability of standard treatments across Europe for childhood cancers. A questionnaire on essential medicines was sent to national representatives of paediatric oncologists, oncology pharmacists, and patients, in up to 37 European countries. Another work stream and dedicated working group focused on radiotherapy resources. Data cleaning and analysis began in view of producing the recommendations based on the collected evidence.
• Access to innovative therapies for childhood cancers through referrals to clinical trials across Europe. The strategic partner of this task was Innovative Therapies or Children with Cancer (ITCC). A first set of recommendations to increase and facilitate children’s access to innovation through clinical trials was agreed during a face-to-face meeting including major stakeholders with focus on ITCC network countries.
• Optimal care and research for young people with extreme
1. List of rare cancers reviewed and discussed during the consensus conference for the benefit of clarifying which cancers are rare and which are the most relevant, from an organisational point of view, families of rare cancers.
2. Recommendations for the standardized estimation of rare cancers indicators at the European and country level available supporting the standardisation of the epidemiological analyses and the data comparison in Europe.
3. Survey on availability of clinical practice guidelines for all rare cancer families finalised together with the assessment of the quality of existing clinical practice guidelines (for each family of rare cancers). This will be of great benefit for ERN experts and for clinicians in general.
4. Proposals to improve collaborative international clinical trials in the EU, particularly inter-group clinical trials finalised for the benefit of all researchers involved in such challenging studies.
5. Map of available continuous medical education resources (for professionals and patients) on the 12 families of rare cancers available for the benefit of the ERNs members.
6. List of national networks on rare cancers in Europe available.
7. Orientations on policy measures on rare cancers for national plans and strategies on cancers and rare diseases finalised
The last two achievements will contribute to start discussion about the integration of the ERN at national level.
The JARC is in line with the planned activites. The outcomes envisioned for the second reporting period have been achieved and are listed above.
The website focused on the dissemination of the goals, results, papers, interviews and news arising from the project to make them available to JARC partners, general public, stakeholders, researchers and patients, scientific and professional societies, Member States, EU Experts on rare cancers.
JARC activities and outcomes are annually evaluated by an external advisory board.