Review 331
Improving Outcomes for Teenagers and Young Adults (TYA) with Cancer Verbesserung der Behandlungsergebnisse für Adoleszente und junge Erwachsene mit Krebs
Affiliations
Key words
▶ cancer ● ▶ teenager ● ▶ young adult ● ▶ specialist services ●
Schlüsselwörter ▶ Krebs ● ▶ Teenager ● ▶ junge Erwachsene ●
Bibliography DOI http://dx.doi.org/ 10.1055/s-0033-1358392 Published online: October 28, 2013 Klin Padiatr 2013; 225: 331–334 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Dan Stark Oncology Leeds Teaching Hospital NHS Trust Level 4 Bexley Wing Leeds Ls97TF United Kingdom Tel.: + 49/113/2068 266 Fax: + 49/113/2067 871 [email protected]
D. Stark1, I. Lewis2 1 2
Oncology, Leeds Teaching Hospital NHS Trust, Leeds, United Kingdom Alderhey Childrens Hospital, Childrens Services, Liverpool, United Kingdom
Abstract
Zusammenfassung
The management of TYA with cancer is characterized by biological features in comparison to children. Therefore specialized treatment units have been established within professional structures of care for this group, and a European multidisciplinary framework for the treatment of TYA with cancer was founded. Objectives are to promote interdisciplinary collaboration and provide strategic concepts to improve patient care centered to the special needs of this age group. Access to clinical trials for all TYA in the EU will be improved and research initiated, examining biology, epidemiology and health services. Special goals of the interprofessional cooperation are: i) Improvement in survival and the quality of survival, where TYA are disadvantaged by existing structures of care. ii) Provision of all the required expertise in dedicated and effective multiprofessional teams, sometimes across traditional healthcare boundaries. iii) Provision of an environment for care that meets the specific needs of TYA. Different measurements are discussed improving outcomes for TYA is proceeding at different speeds in different parts of the world. In some there are established teams, bringing together paediatric and adult specialists from many healthcare professions, reviewing and contributing to the optimal care of all TYA with cancer as part of national health policy.
Krebserkrankungen bei TYA zeichnen sich durch biologische Besonderheiten aus. Deshalb sind interdisziplinäre Behandlungsgruppen speziell für die Patienten dieser Altersgruppe geschaffen worden, die sich international zu einer Arbeitsgruppe zusammengeschlossen haben. Allgemeine Ziele der TYA sind, die Besonderheiten der Krebserkrankungen in dieser Altersgruppe bei der Therapie zu berücksichtigen, den Herausforderungen der interprofessionellen Zusammenarbeit für eine angemessene Versorgung dieser Patienten gerecht zu werden und dem Wunsch vieler Patienten und den an der Behandlung beteiligten Professionen nach einer besseren Versorgung zu entsprechen. Die speziellen Ziele beinhalten: i) Bestehende Strukturen verbessern, wenn diese das Überleben und die Lebensqualität nachteilig beeinflussen. ii) Die erforderliche Expertise in einem geeigneten multiprofessionellen Team verfügbar zu machen, auch wenn dies über die traditionellen Gesundheitsstrukturen hinausgehen sollte. iii) Bereitstellung einer angemessenen Umgebung, die den spezifischen Bedürfnissen dieser Patientengruppe gerecht wird. Hierfür wurden in den verschiedenen Ländern unterschiedliche Maßnahmen ergriffen. Verschiedene Wege zu Verwirklichung der integrierten Zusammenarbeit von pädiatrischen und internistischen Mitarbeitern werden vorgestellt.
Teenagers and Young Adults (TYA) are best considered as the younger patients treated in adults services and the older patients treated in children’s services. The precise definition varies depending where that interface lies in differ-
ent healthcare systems. TYA with cancer aged 15–24 have unmet needs both clinically and for supportive care in many current healthcare systems [22, 32]. Clinically, their cancer survival remains disappointing when compared to younger
▼
▼
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
Authors
children, and is poor in sarcomas whether compared to young children or slightly older adults. In other cancer types, notably brain tumours, survival of TYA is better than either younger or older age groups [2, 13, 28]. In addition, TYA cancer outcomes have not had the same improvement as children or adults over recent times. This has been attributed to low levels of trial recruitment in this patient population [3]. In terms of their care, when asked, TYA patients wish to have a specialist multidisciplinary team that is trained to meet their specific needs, where the team members realise they are neither small children nor older adults, and can support the TYA to navigate cancer treatment at such a disruptive time in their personal and social development [15]. Where this is provided, such care is associated with better concordance with therapy [5]. This specialist team approach can be enhanced by developing a specific TYA ward, where TYA can spend time with others ‘in the same boat’ rather than being a minority on children’s or adult wards [18]. Only collaboration between a range of professionals can truly provide the required expertise and leadership for specialist TYA cancer services. However TYA may be disadvantaged by traditional healthcare systems. Neither children’s nor adult cancer services have TYA as their focus. Due to the case-mix, one service concentrates upon providing services to younger children and the other upon providing services for much older adults. TYA can fall down a ‘gap’ [20]. In some settings the problem is even more serious than that. Without active collaboration TYA can become a source of conflict between children’s and adult cancer services. Both may feel that the treatment paradigms that will be most effective in TYA cancer are those paradigms which they are most familiar with. Yet these approaches may be surprisingly different across an arbitrary age-based interface. At first glance the epidemiology of TYA cancer appears to sit between a children’s and an adult pattern, with most prevalent cancers being lymphomas, brain tumours, and carcinomas/epithelial cancers [22]. However it is important to appreciate that, even where TYA tumours may appear to be related to children’s or adult cancers, where the biology has been examined it is often distinct from either comparator age-group [8, 21, 23, 24, 33]. Neither adult nor paediatric oncologists (nor haematologists) currently have all the expertise to manage these cancers alone based upon their existing practices. The argument for bringing paediatric and adult specialists together for the benefit of TYA was first advanced nearly two decades ago Lewis [19]. Open multi-professional discussion of the many uncertainties, and a resultant culture of joint working, has proven helpful in many centres, providing TYA with the advantages of all the expertise required to treat them. Interestingly there is growing recent evidence that where specialist TYA services and/or collaboration between adult and paediatric care are used, survival is better. Historical data indicated much poorer survival for TYA with ALL than younger children [4]. There was much discussion whether this was due to choice of protocol, biology, or treatment approach [27]. Protocols based upon the approach used in paediatric haematology are now implemented in TYA, and survival is greatly improved where trial recruitment is high [16]. In Ewing sarcoma historical data suggested poorer outcomes for TYA treated in institutions where the practice was mainly adult oncology than in institutions where the practice was predominantly paediatric [25]. To improve this, a single trial protocol, Euro-Ewings 99, was developed and recruited to collaboratively for patients aged up to 40 years, and recent outcomes indicate where patients are treated
on that protocol this difference in survival is no longer apparent [26]. Other jointly developed trials are urgently required, to inform treatment delivery and include biological endpoints to identify the source of variation. Across the UK, survival from TYA cancer in the years since 2000 have been examined from NHS data sources and specialist TYA cancer registration. Survival has been examined in relation to the complexity of treatment administered (defined as surgery, chemotherapy or radiotherapy alone, vs. 2 modalities vs. 3 modalities). Patients requiring three modalities of treatment may have better survival when managed predominantly in TYA specialist centres than when managed outside those services [1]. However the problem is not overcome, with evidence emerging that adolescents are still less likely to be treated on schedule according to those improved protocols than children or adults in acute leukaemia, implying that challenges may still remain in supportive care, to deliver protocol treatment to young people [9]. When a culture of open multidisciplinary discussion is in place involving clinicians from paediatric and adult services, improved clinical trial recruitment and TYA-specific research programmes may emerge as questions are asked at the interface between children’s and adults’ protocols. More such developments are required to improve clinical outcomes. All clinical groups who have a stake in the care of some TYA can help their patients further, by developing a culture of joint discussion of TYA cancer cases, and sharing a role in decision-making and patient support. Specialist teams and environments for TYA cancer care are developing at different speeds in different places, and with this in mind the EU-FP7 collaborative European Network for Cancer in Children and Adolescents (‘ENCCA’) includes a specific work programme (‘ENTYAC’) that is focussing upon TYA cancer. This has 6 key areas: 1. developing a European Multidisciplinary Framework for TYA Cancer, to promote collaboration and provide strategic direction, 2. promoting and developing TYA multi-professional education for the workforce, 3. improving access to clinical trials for all TYA in the EU, 4. developing a European TYA research initiative, examining biology, epidemiology and health services, 5. promoting healthy lifestyles in the TYA population and cancer survivors and 6. placing patient and support organisations at the heart of what we do. This growing group has > 150 individual professional affiliated members, links with 20 other collaborative multi-professional groups with TYA as all or a large part of their focus, and developing a structure where young people are able to express their views about the work, amounting to over 10 000 members. This is being led by a steering group within a structured framework ▶ Fig. 1). (● There remains a lot for this group to do, and for future work after this collaborative completes its currently funded work in 2014. 116 professionals from 24 European countries completed a survey to benchmark the current development and variation of TYA services. Analysis of the survey identified variations within and between countries. These included: ▶ The recognition of TYA as a special group, ▶ the availability of policy guidance on specialist care for TYA, to promote equitable access, ▶ professional awareness of existing units with a TYA special interest,
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
332 Review
Review 333
Collaboration
Work packages; Bone sarcomas (7), education(15), ethics (18), biological research (5), Virtual Portal (3), registration (11), survivorship (13) ENCCA STEERING GROUP
Oversight and responsibility for delivery
Tasks
Projects
UK
France
2- Education
Programme of educational events Training and competencie s
Germany
Spain
3- Clinical trials
4- Research
Existing trial opportunities recruitment, and eligibility
Epidemiology and registration
Italy
5- Healthy lifestyles
Fertility preservation
Health services research Biology
Late effects and survivorship screening and care Cancer secondary prevention
Workforce skillmix Communication
Scandanavia
other ENCCA members
6- Patients
Integrate patients effectively into all tasks Link international resources Link charities and advocacy bodies
Patient and professional information
Other EU nonENCCA members
Service guidance
Shared translated set of information sheets for patients and professionals Linked charities and advocacy bodies and processes
Linked protocols and policy documents
Web ENCCA TYA Information platform, becoming an ENCCA TYA Virtual Research institute
Fig. 1 Framework to promote collaboration in Teenage and Young Adult Cancer Care 2011.
▶ availability of specific health service funding to care for this patient group, ▶ professional membership of specialist TYA teams and ▶ patterns of working across interfaces, including adultpaediatric or haematology- oncology. The ENTYAC group is still developing education for professionals treating TYA cancer. The first specific ENTYAC conference was held in Institut Curie in Paris in 2013, and included input from patients, a range of professionals and ethicists, and discussion of the paediatric-adult interface in synovial sarcoma management. These offer the opportunity to provide a European perspective, building upon earlier global initiatives such as the TCT international conference [31]. Initiatives are also developing in professional training to examine and co-ordinate curricula for certification and define competencies required in order to provide care to TYA with cancer [10]. There are on-going projects describing the recruitment to clinical trials across Europe in TYA, and focussed collaborations in key disease areas such as management of bone sarcomas. There is some early work attempting to bring together tumour registries with an interest in TYA cancer epidemiology and there is a study developing that is exploring variations of time to cancer diagnosis in TYAs between different countries. There are studies completed on the ethics of consent and information exchange when TYA are considering fertility-damaging cancer treatment, which indicate inequity in legal and ethical codes across Europe. Other on-going studies are examining the extent existing tumour banks
hold clinically annotated samples from TYA patients, including TYA in early phase trials and sequential European surveys of patient, carer and professional opinion of existing services.
Conclusion
▼
TYA cancer care development is in a very exciting phase. In more and more countries there are established TYA MDTs, bringing together paediatric and adult specialists. Where felt to be necessary there are specialised wards and out-patient services tailored to TYA. In several there are charities and patient groups working alongside professionals to achieve these aims. Collaboration is the key, based upon the disease profile and patient needs, with the patient at the centre rather than professional boundaries. Such collaboration is being formalised in ongoing discussions between international professional organisations such as ECCO [11], ESMO [12] and SIOP [17], beginning with improving multi-professional education. Meanwhile national professional membership organisations for TYA cancer care professionals [14, 30], and specific charities are developing professional awareness and training, service design and further collaborative research [6, 7, 29]. Many healthcare professionals are becoming aware that specialties must work together, for a group of patients we cannot serve alone. All those with a stake in improving a part of TYA cancer care can learn from each other to improve patient experience and outcomes.
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
National groups
Steering group- multi-national, multi-professional- see members in Appendix 2
Acknowledgements
▼
ENTYAC is funded as work-package 17 of ENCCA, the European Network for Cancer in Children and Adolescents. Information described in this article has received funding from the European Union’s Seventh Framework Programme (FP7/2007-2013) under the project ENCCA, grant agreement HEALTH-F2-2011-261474.
Conflict of interest: The authors have no conflict of interest to disclose. References 1 Birch R. Teenagers and Young Adults with Cancer. Leeds: Leeds, 2013 2 Bleyer A, Choi M, Fuller CD et al. Relative lack of conditional survival improvement in young adults with cancer. Semin Oncol 2009; 36: 460–467 3 Bleyer A, Montello M, Budd T et al. National survival trends of young adults with sarcoma: lack of progress is associated with lack of clinical trial participation. Cancer 2005; 103: 1891–1897 4 Boissel N, Auclerc MF, Lheritier V et al. Should adolescents with acute lymphoblastic leukemia be treated as old children or young adults? Comparison of the French FRALLE-93 and LALA-94 trials. J Clin Oncol 2003; 21: 774–780 5 Butow P, Palmer S, Pai A et al. Review of adherence-related issues in adolescents and young adults with cancer. J Clin Oncol 2010; 28: 4800–4809 6 CanTeen Australia. 2013; (Accessed 04-07-2013, 2013, at https:// www.canteen.org.au/) 7 CanTeen Ireland. 2009; (Accessed 04-07-2013, 2013, at http://www. canteen.ie/) 8 Chan JK, Urban R, Cheung MK et al. Ovarian cancer in younger vs older women: a population-based analysis. Br J Cancer 2006; 95: 1314–1320 9 Cluzeau TDN, Huguet F, Raffoux E et al. A Pediatric Treatment of PhNegative Acute Lymphoblastic Leukemia (ALL) Is Effective and Safe In Adolescents and Young Adults (AYAs) until 29 Years of Age. American Society of Haematology (ASH) 2010 10 Curriculum for Specialty Training In Medical Oncology. 2010;at http://www.gmc-uk.org/Medical_Oncology_curriculum_2010. pdf_32731045.pdf ) 11 European Cancer Organisation. 2013; (Accessed 04-07-2013, 2013, at http://www.ecco-org.eu/) 12 European Society for Medical Oncology. 2013; (Accessed 04-07-2013, 2013, at http://www.esmo.org/) 13 Gatta G, Zigon G, Capocaccia R et al. Survival of European children and young adults with cancer diagnosed 1995-2002. Eur J Cancer 2009; 45: 992–1005 14 Groupe Onco-hematologie Adolescents et Jeunes Adultes. 2013; (Accessed 04-07-2013, 2013, at http://go-aja.fr/index.php?code = o8K V0p3C39rX2dPmxA = = &PHPSESSID = o4i7qt0c3jomggud vcrvuvnqi7) 15 Hollis R, Morgan S. The adolescent with cancer – at the edge of noman’s land. Lancet Oncol 2001; 2: 43–48
16 Hunger SP, Lu X, Devidas M et al. Improved survival for children and adolescents with acute lymphoblastic leukemia between 1990 and 2005: a report from the children’s oncology group. J Clin Oncol 2012; 30: 1663–1669 17 International Society of Paediatric Oncology. 2013; (Accessed 04-072013, 2013, at http://siop-online.org/) 18 Kelly D, Pearce S, Mulhall A. ‘Being in the same boat’: ethnographic insights into an adolescent cancer unit. Int J Nurs Stud 2004; 41: 847–857 19 Lewis IJ. Cancer in adolescence. Br Med Bull 1996; 52: 887–897 20 Michelagnoli MP, Pritchard J, Phillips MB. Adolescent oncology – a homeland for the “lost tribe”. Eur J Cancer 2003; 39: 2571–2572 21 Moorman AV, Harrison CJ, Buck GA et al. Karyotype is an independent prognostic factor in adult acute lymphoblastic leukemia (ALL): analysis of cytogenetic data from patients treated on the Medical Research Council (MRC) UKALLXII/Eastern Cooperative Oncology Group (ECOG) 2993 trial. Blood 2007; 109: 3189–3197 22 NICE (UK). Improving outcomes in children and young people with cancer. Cancer service guidance. London: 2005 23 Nicholson BD, Picton SV, Chumas P et al. Changes in the patterns of care of central nervous system tumours among 16-24 year olds and the effect on survival in Yorkshire between 1990 and 2009. Clin Oncol (R Coll Radiol) 2013; 25: 205–214 24 Palmer RD, Murray MJ, Saini HK et al. Malignant germ cell tumors display common microRNA profiles resulting in global changes in expression of messenger RNA targets. Cancer Res 2010; 70: 2911–2923 25 Paulussen SAS, Juergens HF. Cure rates in Ewing tumor patients aged over 15 years are better in pediatric oncology units. Results of GPOH CESS/EICESS studies. Proc Am Soc Clin Oncol 2003; 816 26 Paulussen MDU, Jürgens H, Ranft A. Should Adolescents with Ewing Sarcoma be treated in pediatric or non-pediatric oncology institutions? An analysis of GPOH Ewing trial (CESS/EICESS/EURO-E.W.I.N.G.) data. Pediatr Blood Cancer 2012; 1046 27 Stock W, La M, Sanford B et al. What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Children’s Cancer Group and Cancer and Leukemia Group B studies. Blood 2008; 112: 1646–1654 28 Survival in Teenagers and Young Adults with Cancer in the UK 2012;(Accessed 02-07-2013, 2013, at http://www.ncin.org.uk/view. aspx?rid = 1607 .) 29 Teenage Cancer Trust, Research. 2013; (Accessed 05-07-2013, 2013, at http://www.teenagecancertrust.org/what-we-do/research/) 30 Teenagers and Young Adults with Cancer. 2013; (Accessed 04-07-2013, 2013, at http://www.tyac.org.uk/) 31 The Teenage Cancer Trust International Conference. 2013; (Accessed 04-07-2013, 2013, at http://www.teenagecancertrust.org/whatwe-do/international-conference/registration/) 32 Thomas DM, Albritton KH, Ferrari A. Adolescent and young adult oncology: an emerging field. J Clin Oncol 2010; 28: 4781–4782 33 Weir HK, Marrett LD, Cokkinides V et al. Melanoma in adolescents and young adults (ages 15–39 years): United States, 1999–2006. J Am Acad Dermatol 2011; 65: S38–S49
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
334 Review
Improving Outcomes for Teenagers and Young Adults (TYA) with Cancer Verbesserung der Behandlungsergebnisse für Adoleszente und junge Erwachsene mit Krebs
Affiliations
Key words
▶ cancer ● ▶ teenager ● ▶ young adult ● ▶ specialist services ●
Schlüsselwörter ▶ Krebs ● ▶ Teenager ● ▶ junge Erwachsene ●
Bibliography DOI http://dx.doi.org/ 10.1055/s-0033-1358392 Published online: October 28, 2013 Klin Padiatr 2013; 225: 331–334 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Dan Stark Oncology Leeds Teaching Hospital NHS Trust Level 4 Bexley Wing Leeds Ls97TF United Kingdom Tel.: + 49/113/2068 266 Fax: + 49/113/2067 871 [email protected]
D. Stark1, I. Lewis2 1 2
Oncology, Leeds Teaching Hospital NHS Trust, Leeds, United Kingdom Alderhey Childrens Hospital, Childrens Services, Liverpool, United Kingdom
Abstract
Zusammenfassung
The management of TYA with cancer is characterized by biological features in comparison to children. Therefore specialized treatment units have been established within professional structures of care for this group, and a European multidisciplinary framework for the treatment of TYA with cancer was founded. Objectives are to promote interdisciplinary collaboration and provide strategic concepts to improve patient care centered to the special needs of this age group. Access to clinical trials for all TYA in the EU will be improved and research initiated, examining biology, epidemiology and health services. Special goals of the interprofessional cooperation are: i) Improvement in survival and the quality of survival, where TYA are disadvantaged by existing structures of care. ii) Provision of all the required expertise in dedicated and effective multiprofessional teams, sometimes across traditional healthcare boundaries. iii) Provision of an environment for care that meets the specific needs of TYA. Different measurements are discussed improving outcomes for TYA is proceeding at different speeds in different parts of the world. In some there are established teams, bringing together paediatric and adult specialists from many healthcare professions, reviewing and contributing to the optimal care of all TYA with cancer as part of national health policy.
Krebserkrankungen bei TYA zeichnen sich durch biologische Besonderheiten aus. Deshalb sind interdisziplinäre Behandlungsgruppen speziell für die Patienten dieser Altersgruppe geschaffen worden, die sich international zu einer Arbeitsgruppe zusammengeschlossen haben. Allgemeine Ziele der TYA sind, die Besonderheiten der Krebserkrankungen in dieser Altersgruppe bei der Therapie zu berücksichtigen, den Herausforderungen der interprofessionellen Zusammenarbeit für eine angemessene Versorgung dieser Patienten gerecht zu werden und dem Wunsch vieler Patienten und den an der Behandlung beteiligten Professionen nach einer besseren Versorgung zu entsprechen. Die speziellen Ziele beinhalten: i) Bestehende Strukturen verbessern, wenn diese das Überleben und die Lebensqualität nachteilig beeinflussen. ii) Die erforderliche Expertise in einem geeigneten multiprofessionellen Team verfügbar zu machen, auch wenn dies über die traditionellen Gesundheitsstrukturen hinausgehen sollte. iii) Bereitstellung einer angemessenen Umgebung, die den spezifischen Bedürfnissen dieser Patientengruppe gerecht wird. Hierfür wurden in den verschiedenen Ländern unterschiedliche Maßnahmen ergriffen. Verschiedene Wege zu Verwirklichung der integrierten Zusammenarbeit von pädiatrischen und internistischen Mitarbeitern werden vorgestellt.
Teenagers and Young Adults (TYA) are best considered as the younger patients treated in adults services and the older patients treated in children’s services. The precise definition varies depending where that interface lies in differ-
ent healthcare systems. TYA with cancer aged 15–24 have unmet needs both clinically and for supportive care in many current healthcare systems [22, 32]. Clinically, their cancer survival remains disappointing when compared to younger
▼
▼
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
Authors
children, and is poor in sarcomas whether compared to young children or slightly older adults. In other cancer types, notably brain tumours, survival of TYA is better than either younger or older age groups [2, 13, 28]. In addition, TYA cancer outcomes have not had the same improvement as children or adults over recent times. This has been attributed to low levels of trial recruitment in this patient population [3]. In terms of their care, when asked, TYA patients wish to have a specialist multidisciplinary team that is trained to meet their specific needs, where the team members realise they are neither small children nor older adults, and can support the TYA to navigate cancer treatment at such a disruptive time in their personal and social development [15]. Where this is provided, such care is associated with better concordance with therapy [5]. This specialist team approach can be enhanced by developing a specific TYA ward, where TYA can spend time with others ‘in the same boat’ rather than being a minority on children’s or adult wards [18]. Only collaboration between a range of professionals can truly provide the required expertise and leadership for specialist TYA cancer services. However TYA may be disadvantaged by traditional healthcare systems. Neither children’s nor adult cancer services have TYA as their focus. Due to the case-mix, one service concentrates upon providing services to younger children and the other upon providing services for much older adults. TYA can fall down a ‘gap’ [20]. In some settings the problem is even more serious than that. Without active collaboration TYA can become a source of conflict between children’s and adult cancer services. Both may feel that the treatment paradigms that will be most effective in TYA cancer are those paradigms which they are most familiar with. Yet these approaches may be surprisingly different across an arbitrary age-based interface. At first glance the epidemiology of TYA cancer appears to sit between a children’s and an adult pattern, with most prevalent cancers being lymphomas, brain tumours, and carcinomas/epithelial cancers [22]. However it is important to appreciate that, even where TYA tumours may appear to be related to children’s or adult cancers, where the biology has been examined it is often distinct from either comparator age-group [8, 21, 23, 24, 33]. Neither adult nor paediatric oncologists (nor haematologists) currently have all the expertise to manage these cancers alone based upon their existing practices. The argument for bringing paediatric and adult specialists together for the benefit of TYA was first advanced nearly two decades ago Lewis [19]. Open multi-professional discussion of the many uncertainties, and a resultant culture of joint working, has proven helpful in many centres, providing TYA with the advantages of all the expertise required to treat them. Interestingly there is growing recent evidence that where specialist TYA services and/or collaboration between adult and paediatric care are used, survival is better. Historical data indicated much poorer survival for TYA with ALL than younger children [4]. There was much discussion whether this was due to choice of protocol, biology, or treatment approach [27]. Protocols based upon the approach used in paediatric haematology are now implemented in TYA, and survival is greatly improved where trial recruitment is high [16]. In Ewing sarcoma historical data suggested poorer outcomes for TYA treated in institutions where the practice was mainly adult oncology than in institutions where the practice was predominantly paediatric [25]. To improve this, a single trial protocol, Euro-Ewings 99, was developed and recruited to collaboratively for patients aged up to 40 years, and recent outcomes indicate where patients are treated
on that protocol this difference in survival is no longer apparent [26]. Other jointly developed trials are urgently required, to inform treatment delivery and include biological endpoints to identify the source of variation. Across the UK, survival from TYA cancer in the years since 2000 have been examined from NHS data sources and specialist TYA cancer registration. Survival has been examined in relation to the complexity of treatment administered (defined as surgery, chemotherapy or radiotherapy alone, vs. 2 modalities vs. 3 modalities). Patients requiring three modalities of treatment may have better survival when managed predominantly in TYA specialist centres than when managed outside those services [1]. However the problem is not overcome, with evidence emerging that adolescents are still less likely to be treated on schedule according to those improved protocols than children or adults in acute leukaemia, implying that challenges may still remain in supportive care, to deliver protocol treatment to young people [9]. When a culture of open multidisciplinary discussion is in place involving clinicians from paediatric and adult services, improved clinical trial recruitment and TYA-specific research programmes may emerge as questions are asked at the interface between children’s and adults’ protocols. More such developments are required to improve clinical outcomes. All clinical groups who have a stake in the care of some TYA can help their patients further, by developing a culture of joint discussion of TYA cancer cases, and sharing a role in decision-making and patient support. Specialist teams and environments for TYA cancer care are developing at different speeds in different places, and with this in mind the EU-FP7 collaborative European Network for Cancer in Children and Adolescents (‘ENCCA’) includes a specific work programme (‘ENTYAC’) that is focussing upon TYA cancer. This has 6 key areas: 1. developing a European Multidisciplinary Framework for TYA Cancer, to promote collaboration and provide strategic direction, 2. promoting and developing TYA multi-professional education for the workforce, 3. improving access to clinical trials for all TYA in the EU, 4. developing a European TYA research initiative, examining biology, epidemiology and health services, 5. promoting healthy lifestyles in the TYA population and cancer survivors and 6. placing patient and support organisations at the heart of what we do. This growing group has > 150 individual professional affiliated members, links with 20 other collaborative multi-professional groups with TYA as all or a large part of their focus, and developing a structure where young people are able to express their views about the work, amounting to over 10 000 members. This is being led by a steering group within a structured framework ▶ Fig. 1). (● There remains a lot for this group to do, and for future work after this collaborative completes its currently funded work in 2014. 116 professionals from 24 European countries completed a survey to benchmark the current development and variation of TYA services. Analysis of the survey identified variations within and between countries. These included: ▶ The recognition of TYA as a special group, ▶ the availability of policy guidance on specialist care for TYA, to promote equitable access, ▶ professional awareness of existing units with a TYA special interest,
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
332 Review
Review 333
Collaboration
Work packages; Bone sarcomas (7), education(15), ethics (18), biological research (5), Virtual Portal (3), registration (11), survivorship (13) ENCCA STEERING GROUP
Oversight and responsibility for delivery
Tasks
Projects
UK
France
2- Education
Programme of educational events Training and competencie s
Germany
Spain
3- Clinical trials
4- Research
Existing trial opportunities recruitment, and eligibility
Epidemiology and registration
Italy
5- Healthy lifestyles
Fertility preservation
Health services research Biology
Late effects and survivorship screening and care Cancer secondary prevention
Workforce skillmix Communication
Scandanavia
other ENCCA members
6- Patients
Integrate patients effectively into all tasks Link international resources Link charities and advocacy bodies
Patient and professional information
Other EU nonENCCA members
Service guidance
Shared translated set of information sheets for patients and professionals Linked charities and advocacy bodies and processes
Linked protocols and policy documents
Web ENCCA TYA Information platform, becoming an ENCCA TYA Virtual Research institute
Fig. 1 Framework to promote collaboration in Teenage and Young Adult Cancer Care 2011.
▶ availability of specific health service funding to care for this patient group, ▶ professional membership of specialist TYA teams and ▶ patterns of working across interfaces, including adultpaediatric or haematology- oncology. The ENTYAC group is still developing education for professionals treating TYA cancer. The first specific ENTYAC conference was held in Institut Curie in Paris in 2013, and included input from patients, a range of professionals and ethicists, and discussion of the paediatric-adult interface in synovial sarcoma management. These offer the opportunity to provide a European perspective, building upon earlier global initiatives such as the TCT international conference [31]. Initiatives are also developing in professional training to examine and co-ordinate curricula for certification and define competencies required in order to provide care to TYA with cancer [10]. There are on-going projects describing the recruitment to clinical trials across Europe in TYA, and focussed collaborations in key disease areas such as management of bone sarcomas. There is some early work attempting to bring together tumour registries with an interest in TYA cancer epidemiology and there is a study developing that is exploring variations of time to cancer diagnosis in TYAs between different countries. There are studies completed on the ethics of consent and information exchange when TYA are considering fertility-damaging cancer treatment, which indicate inequity in legal and ethical codes across Europe. Other on-going studies are examining the extent existing tumour banks
hold clinically annotated samples from TYA patients, including TYA in early phase trials and sequential European surveys of patient, carer and professional opinion of existing services.
Conclusion
▼
TYA cancer care development is in a very exciting phase. In more and more countries there are established TYA MDTs, bringing together paediatric and adult specialists. Where felt to be necessary there are specialised wards and out-patient services tailored to TYA. In several there are charities and patient groups working alongside professionals to achieve these aims. Collaboration is the key, based upon the disease profile and patient needs, with the patient at the centre rather than professional boundaries. Such collaboration is being formalised in ongoing discussions between international professional organisations such as ECCO [11], ESMO [12] and SIOP [17], beginning with improving multi-professional education. Meanwhile national professional membership organisations for TYA cancer care professionals [14, 30], and specific charities are developing professional awareness and training, service design and further collaborative research [6, 7, 29]. Many healthcare professionals are becoming aware that specialties must work together, for a group of patients we cannot serve alone. All those with a stake in improving a part of TYA cancer care can learn from each other to improve patient experience and outcomes.
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
National groups
Steering group- multi-national, multi-professional- see members in Appendix 2
Acknowledgements
▼
ENTYAC is funded as work-package 17 of ENCCA, the European Network for Cancer in Children and Adolescents. Information described in this article has received funding from the European Union’s Seventh Framework Programme (FP7/2007-2013) under the project ENCCA, grant agreement HEALTH-F2-2011-261474.
Conflict of interest: The authors have no conflict of interest to disclose. References 1 Birch R. Teenagers and Young Adults with Cancer. Leeds: Leeds, 2013 2 Bleyer A, Choi M, Fuller CD et al. Relative lack of conditional survival improvement in young adults with cancer. Semin Oncol 2009; 36: 460–467 3 Bleyer A, Montello M, Budd T et al. National survival trends of young adults with sarcoma: lack of progress is associated with lack of clinical trial participation. Cancer 2005; 103: 1891–1897 4 Boissel N, Auclerc MF, Lheritier V et al. Should adolescents with acute lymphoblastic leukemia be treated as old children or young adults? Comparison of the French FRALLE-93 and LALA-94 trials. J Clin Oncol 2003; 21: 774–780 5 Butow P, Palmer S, Pai A et al. Review of adherence-related issues in adolescents and young adults with cancer. J Clin Oncol 2010; 28: 4800–4809 6 CanTeen Australia. 2013; (Accessed 04-07-2013, 2013, at https:// www.canteen.org.au/) 7 CanTeen Ireland. 2009; (Accessed 04-07-2013, 2013, at http://www. canteen.ie/) 8 Chan JK, Urban R, Cheung MK et al. Ovarian cancer in younger vs older women: a population-based analysis. Br J Cancer 2006; 95: 1314–1320 9 Cluzeau TDN, Huguet F, Raffoux E et al. A Pediatric Treatment of PhNegative Acute Lymphoblastic Leukemia (ALL) Is Effective and Safe In Adolescents and Young Adults (AYAs) until 29 Years of Age. American Society of Haematology (ASH) 2010 10 Curriculum for Specialty Training In Medical Oncology. 2010;at http://www.gmc-uk.org/Medical_Oncology_curriculum_2010. pdf_32731045.pdf ) 11 European Cancer Organisation. 2013; (Accessed 04-07-2013, 2013, at http://www.ecco-org.eu/) 12 European Society for Medical Oncology. 2013; (Accessed 04-07-2013, 2013, at http://www.esmo.org/) 13 Gatta G, Zigon G, Capocaccia R et al. Survival of European children and young adults with cancer diagnosed 1995-2002. Eur J Cancer 2009; 45: 992–1005 14 Groupe Onco-hematologie Adolescents et Jeunes Adultes. 2013; (Accessed 04-07-2013, 2013, at http://go-aja.fr/index.php?code = o8K V0p3C39rX2dPmxA = = &PHPSESSID = o4i7qt0c3jomggud vcrvuvnqi7) 15 Hollis R, Morgan S. The adolescent with cancer – at the edge of noman’s land. Lancet Oncol 2001; 2: 43–48
16 Hunger SP, Lu X, Devidas M et al. Improved survival for children and adolescents with acute lymphoblastic leukemia between 1990 and 2005: a report from the children’s oncology group. J Clin Oncol 2012; 30: 1663–1669 17 International Society of Paediatric Oncology. 2013; (Accessed 04-072013, 2013, at http://siop-online.org/) 18 Kelly D, Pearce S, Mulhall A. ‘Being in the same boat’: ethnographic insights into an adolescent cancer unit. Int J Nurs Stud 2004; 41: 847–857 19 Lewis IJ. Cancer in adolescence. Br Med Bull 1996; 52: 887–897 20 Michelagnoli MP, Pritchard J, Phillips MB. Adolescent oncology – a homeland for the “lost tribe”. Eur J Cancer 2003; 39: 2571–2572 21 Moorman AV, Harrison CJ, Buck GA et al. Karyotype is an independent prognostic factor in adult acute lymphoblastic leukemia (ALL): analysis of cytogenetic data from patients treated on the Medical Research Council (MRC) UKALLXII/Eastern Cooperative Oncology Group (ECOG) 2993 trial. Blood 2007; 109: 3189–3197 22 NICE (UK). Improving outcomes in children and young people with cancer. Cancer service guidance. London: 2005 23 Nicholson BD, Picton SV, Chumas P et al. Changes in the patterns of care of central nervous system tumours among 16-24 year olds and the effect on survival in Yorkshire between 1990 and 2009. Clin Oncol (R Coll Radiol) 2013; 25: 205–214 24 Palmer RD, Murray MJ, Saini HK et al. Malignant germ cell tumors display common microRNA profiles resulting in global changes in expression of messenger RNA targets. Cancer Res 2010; 70: 2911–2923 25 Paulussen SAS, Juergens HF. Cure rates in Ewing tumor patients aged over 15 years are better in pediatric oncology units. Results of GPOH CESS/EICESS studies. Proc Am Soc Clin Oncol 2003; 816 26 Paulussen MDU, Jürgens H, Ranft A. Should Adolescents with Ewing Sarcoma be treated in pediatric or non-pediatric oncology institutions? An analysis of GPOH Ewing trial (CESS/EICESS/EURO-E.W.I.N.G.) data. Pediatr Blood Cancer 2012; 1046 27 Stock W, La M, Sanford B et al. What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Children’s Cancer Group and Cancer and Leukemia Group B studies. Blood 2008; 112: 1646–1654 28 Survival in Teenagers and Young Adults with Cancer in the UK 2012;(Accessed 02-07-2013, 2013, at http://www.ncin.org.uk/view. aspx?rid = 1607 .) 29 Teenage Cancer Trust, Research. 2013; (Accessed 05-07-2013, 2013, at http://www.teenagecancertrust.org/what-we-do/research/) 30 Teenagers and Young Adults with Cancer. 2013; (Accessed 04-07-2013, 2013, at http://www.tyac.org.uk/) 31 The Teenage Cancer Trust International Conference. 2013; (Accessed 04-07-2013, 2013, at http://www.teenagecancertrust.org/whatwe-do/international-conference/registration/) 32 Thomas DM, Albritton KH, Ferrari A. Adolescent and young adult oncology: an emerging field. J Clin Oncol 2010; 28: 4781–4782 33 Weir HK, Marrett LD, Cokkinides V et al. Melanoma in adolescents and young adults (ages 15–39 years): United States, 1999–2006. J Am Acad Dermatol 2011; 65: S38–S49
Stark D, Lewis I. Improving Outcomes for Teenagers … Klin Padiatr 2013; 225: 331–334
Downloaded by: University of Queensland. Copyrighted material.
334 Review
