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Over 14 000 patients aged 15-24 are estimated to be diagnosed with cancer in the European Union (EU) each year. Teenagers and young adults ( TYA) often fall down gaps between children's and adults cancer services. The specific challenges of providing optimal care to them are described, but we present a summary of recent progress. Progress to overcome these challenges is happening at different rates across Europe. We summarise the European national projects in this field but more recently we have seen the beginnings of European coordination. Within the EU 7th Funding Programme (FP7) European Network for Cancer Research in Children and Adolescents programme ( ENCCA), a specific European Network for Teenagers and Young Adults with Cancer has held a series of scientific meetings, including professionals, patients and caregivers. This group has proposed unanswered research questions and agreed key features of a high-quality service that can improve outcomes for TYA with cancer, including the primacy of collaboration between adult and paediatric services to eliminate the gap in the management of TYA with cancer. [ABSTRACT FROM AUTHOR]

The article presents clinical recommendations for diagnosis, treatment and follow-up of Ewing's sarcoma of the bone from the European Society for Medical Oncology (ESMO). The incidence rate of the disease in white Caucasians is 0.3/100,000 per year. Diagnosis includes biopsy, which provides sufficient material for conventional histology. Information is also presented on staging and risk assessment and treatment plan.

The article presents clinical recommendations for diagnosis, treatment and follow-up of osteosarcoma from the European Society for Medical Oncology (ESMO). Disease incidence is higher in adolescents and it usually arises in the metaphysis of a long extremity bone. Diagnosis involves an analysis of signs and symptoms, such as history of pain, but definitive diagnosis requires histological examination. Information is also presented on staging and risk assessment and treatment plan.

Background: Lesbian, gay, bisexual, transgender, and queer (LGBTQ) individuals with cancer have specific and unique health issues and needs. Reports persist of inequalities in the care provided for these patients, making it important to assess the attitudes and knowledge of LGBTQ needs among those who provide care., Materials and Methods: The European Society for Medical Oncology (ESMO) and the European Society for Paediatric Oncology (SIOP Europe) Adolescents and Young Adults Working Group designed this survey comprising 67 questions covering demographics, knowledge, and education of LGBTQ health needs, and attitudes regarding LGBTQ patients with cancer., Results: Among the 672 respondents, a majority do not ask about sexual orientation and gender identity during first visit (64% and 58%, respectively). Only a minority of the respondents considered themselves well informed regarding gay/lesbian and transgender patients' health (44% and 25%, respectively) and psychosocial needs (34%). There was high interest in receiving education regarding the unique health needs of LGBTQ patients (73%)., Conclusions: Survey respondents indicated a willingness to provide care to LGBTQ patients, but a lack of confidence in the knowledge of the health issues and needs of LGBTQ individuals. Lack of training provided in medical schools and postgraduate training programmes and strong interest for additional education on these issues were reported., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Cancer epidemiology is unique in adolescents and young adults (AYAs; aged 15-39 years). The European Society for Medical Oncology/European Society for Paediatric Oncology (ESMO/SIOPE) AYA Working Group aims to describe the burden of cancers in AYAs in Europe and across European Union (EU) countries., Patients and Methods: We used data available on the Global Cancer Observatory. We retrieved crude and age-standardised (World Standard Population) incidence and mortality rates. We reported about AYA cancer burden in Europe and between 28 EU member states. We described incidence and mortality for all cancers and for the 13 cancers most relevant to the AYA population., Results: Incidence and mortality varied widely between countries with the highest mortality observed in Eastern EU countries. Cancers of the female breast, thyroid and male testis were the most common cancers across countries followed by melanoma of skin and cancers of the cervix. Variations in cancer incidence rates across different populations may reflect different distribution of risk factors, variations in the implementation or uptake of screening as well as overdiagnosis. AYA cancer mortality disparities may be due to variation in early-stage diagnoses, different public education and awareness of cancer symptoms, different degrees of access or availability of treatment., Conclusions: Our results highlight the future health care needs and requirements for AYA-specialised services to ensure a homogeneous treatment across different countries as well as the urgency for preventive initiatives that can mitigate the increasing burden., Competing Interests: Disclosure DS reports receipt of honoraria for providing academic peer review for research programme from French INCa, non-financial interest for advisory role in the clinical reference group advising NHS England about cancer policy in children and young people and non-remunerated membership of the Association of Cancer Physicians of the UK, SIOP Europe Adolescent Cancer Committee; IBS reports receipt of honoraria for participation in Advisory Board from Roche, receipt of honoraria as invited speaker from AstraZeneca, Novartis, Pfizer, Roche, receipt of personal and institutional financial interest as local PI from Novartis, Roche, non-financial interest for serving as local PI in EORTC Breast Group, OncoDistinct studies and non-remunerated advisory role in the Ethical Committee Serbia, National Agency for Drug Registration Serbia, the Working Group for Oncology of the Ministry of Health Serbia; NG reports receipt of honoraria to institution for participation in Advisory Board from Y-mAbs Therapeutics, receipt of financial support to institution from Eisai for covering travel expenses and registration fees as invited speaker at international meeting for the presentation of the results from the studies as international PI, receipt of consultancy fee to institution from Ipsen, non-remunerated Co-chair of the Fostering Age inclusive Research (FAIR) trial group of ACCELERATE; non-remunerated member of the Executive Committee of EEC (Euro-Wing Consortium), non-financial interest for leadership role as Chair of the FOSTER Consortium (Fight Osteosarcoma Through European Research) and non-remunerated member of GO-AJA, SFCE, SIOP Europe; FP reports receipt of honoraria for participation in Advisory Board from Roche Diagnostics, receipt of honoraria for providing expert testimony from Ipsen, Merck and non-financial interest for leadership role as Scientific Director of the European School of Oncology; ATo reports receipt of honoraria for participation in Advisory Board from MSD, AstraZeneca, Pfizer, Eli Lilly, receipt of honoraria as invited speaker from Eli Lilly, Novartis and non-remunerated member of AIOM; KS reports receipt of honoraria for participation in Advisory Board from Bayer, Novartis, Novo Nordisk, Roche, non-financial interest for leadership role in PanCare, SPOG, SSPHO and non-remunerated member of Board of Directors of SIOP Europe; GM reports receipt of honoraria for participation in Advisory Board from BMS, Janssen, Roche, Takeda, and receipt of honoraria as invited speaker from Amgen, AstraZeneca, MSD, Novartis, Pfizer; SB reports receipt of honoraria for participation in Advisory Board from Bayer Healthcare, Boehringer Ingelheim, Eli Lilly, Hofmann La Roche, MAP-Biopharma; non-financial interest as principal investigator of the Bayer’s larotrectinib study; and non-remunerated member of the European Musculoskeletal Oncology Society (EMSOS), German Pediatric Oncology Society (GPOH); ES reports receipt of honoraria for participation in Advisory Board from AstraZeneca A.E., AstraZeneca UK Ltd., Gilead Sciences Hellas, Merck, Sharp & Dohme, Pfizer Hellas, receipt of honoraria as invited speaker from Amgen Hellas, Pfizer Hellas, receipt of honoraria for providing expert testimony from Ipsen and non-remunerated member of Board of Directors of Hellenic Oncology Research Group, Hellenic Society of Medical Oncology. All other authors have declared no conflicts of interest., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: The role of chemotherapy for patients with dedifferentiated chondrosarcoma (DDCS) is still under discussion. Here, we present the outcome in patients with DDCS treated with intensive chemotherapy from the EUROpean Bone Over 40 Sarcoma Study., Materials and Methods: The chemotherapy regimen included doxorubicin, ifosfamide and cisplatin. Postoperative methotrexate was added in case of poor histological response. Toxicity was graded based on the National Cancer Institute expanded common toxicity criteria, version 2.0, and survival was analysed using Kaplan-Meier curves, log-rank tests and univariate Cox regression models., Results: Fifty-seven patients with DDCS (localised, 34 [60%]; metastatic, 23 [40%]) aged 42-65 years were included. Surgical complete remission (SCR) was achieved in 36 (63%) patients. The median overall survival (OS) was 24 months (95% confidence interval, 22-25), and the 5-year OS was 39%. Patients with extremity localisation had a 5-year OS of 49% compared with 29% in patients with a central tumour (P = 0.08). Patients with localised disease had a 5-year OS of 46%, whereas patients with metastatic disease had a 5-year OS of 29% (P = 0.12). Patients in SCR had a 5-year OS of 49%, whereas patients not in SCR had a 5-year OS of 23% (P = 0.004). Chemotherapy toxicity was considerable but manageable. There was no treatment-related death, and 39 (70%) patients received ≥6 cycles of the planned nine chemotherapy cycles., Conclusions: Adding intensive chemotherapy to surgery for treatment of DDCS is feasible and shows favourable survival data compared with previous reports. With the limitations of data from a non-controlled trial, we conclude that chemotherapy could be considered in the management of patients aged >40 years., Competing Interests: Conflict of interest statement The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: S.B. has reported grants from Deutsche Krebshilfe, Deutsche Forschungsgemeinschaft and the European Science Foundation and personal fees from Lilly, Bayer, Pfizer, Novartis, Isofol and Clinigen; E.P. has reported advisory roles for Amgen, Daiichi Sankyo, Eli Lilly, EUSA Pharma and Deciphera, received research support from Bristol Myers Squibb, Pfizer and PharmaMar and received travel support from Lilly, PharmaMar and Takeda; L.K. has reported honoraria from Bayer; P.R. has reported advisory roles for Exactech and Stryker; A.F. has reported honoraria from Ipsen Pharma, PharmaMar and Lilly; P.C. has reported honoraria for the speaker, consultancy or advisory role from Bayer, Deciphera, Eisai, Eli Lilly, Nektar Therapeutics and Pfizer and received research funds from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc., Glaxo, Karyopharm Pharmaceuticals, Novartis, Pfizer and PharmaMar; P.R. has reported honoraria from Bayer, Clinigen, BMS, Roche, MSD, Deciphera, Novartis, Pfizer, PharmaMar, Lilly and Amgen; I.H., S.F., K.S.H., P.P., S.H.-N., D.M.D., C.B., B.B., E.St., M.G., T.K., M.E., V.F., M.K., R.B., D.B., O.B., A.C., R.S., R.B., T.K., L.H., G.A., B.-N.H., E.Se., A.F., F.K., G.H.-W. and S.S. have declared no conflicts of interest., (Copyright © 2021 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

It is well recognised that adolescents and young adults (AYA) with cancer have inequitable access to oncology services that provide expert cancer care and consider their unique needs. Subsequently, survival gains in this patient population have improved only modestly compared with older adults and children with cancer. In 2015, the European Society for Medical Oncology (ESMO) and the European Society for Paediatric Oncology (SIOPE) established the joint Cancer in AYA Working Group in order to increase awareness among adult and paediatric oncology communities, enhance knowledge on specific issues in AYA and ultimately improve the standard of care for AYA with cancer across Europe. This manuscript reflects the position of this working group regarding current AYA cancer care, the challenges to be addressed and possible solutions. Key challenges include the lack of specific biological understanding of AYA cancers, the lack of access to specialised centres with age-appropriate multidisciplinary care and the lack of available clinical trials with novel therapeutics. Key recommendations include diversifying interprofessional cooperation in AYA care and specific measures to improve trial accrual, including centralising care where that is the best means to achieve trial accrual. This defines a common vision that can lead to improved outcomes for AYA with cancer in Europe., Competing Interests: Funding The European Society for Medical Oncology (ESMO) (no grant number) and the European Society for Paediatric Oncology (SIOPE) (no grant number) were the legal sponsors of this position paper. No research funding for the meetings or manuscript preparation was received from any third parties. Disclosure DS reports receipt of research grants from Teenage Cancer Trust. FAP reports personal financial interest as a Scientific Director at the European School of Oncology; receipt of lecture/presentation fees from Prime Oncology and Takeda; receipt of honoraria for advisory board participation/advisory services from Roche, AstraZeneca, Clovis and Ipsen. LF reports receipt of funding from Teenage Cancer Trust. IB-S reports receipt of speaker fees from Roche, Novartis and Pfizer; direct research funding as Principal Investigator from Roche; financial support to institution for clinical trials from Roche. OS reports receipt of honoraria for advisory board participation from Genuity Science. LH reports receipt of speaker fees from Roche. WTAvdG reports receipt of research funding from Novartis, honoraria for advisory board from Bayer and consultancy fees from SpringWorks, all to her institutes. SB reports receipt of honoraria for advisory board participation from Pfizer, Bayer, Lilly, Novartis and Isofol. GM reports receipt of speakers fees from AstraZeneca, Roche, MSD, BMS, Pfizer, Takeda, Janssen, Novartis and Sanofi; receipt of consultancy fees from AstraZeneca, Roche, MSD, BMS, Novartis and Sanofi; direct research funding as Principal Investigator from AstraZeneca, Novartis and MSD; financial support to institution for clinical trials from AstraZeneca, Novartis and MSD. ES reports receipt of honoraria for the provision of advisory services from Roche Hellas, BMS, Pfizer Hellas, AstraZeneca, Amgen Hellas and Dimiourgiki Farmakeutikon Ypiresion AE; receipt of research funding from Astellas Pharma; travel and education support from Roche Hellas, Pfizer Hellas, Astellas Pharma, Novartis (Hellas), MSD Greece and Enorasis. All other authors have declared no conflicts of interest., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Irreversible sensorineural hearing loss is a common side effect of platinum treatment with the potential to significantly impair the neurocognitive, social and educational development of childhood cancer survivors. Genetic association studies suggest a genetic predisposition for cisplatin-induced ototoxicity. Among other candidate genes, thiopurine methyltransferase (TPMT) is considered a critical gene for susceptibility to cisplatin-induced hearing loss in a pharmacogenetic guideline. The aim of this cross-sectional cohort study was to confirm the genetic associations in a large pan-European population and to evaluate the diagnostic accuracy of the genetic markers., Methods: Eligibility criteria required patients to be aged less than 19 years at the start of chemotherapy, which had to include cisplatin and/or carboplatin. Patients were assigned to three phenotype categories: no, minor and clinically relevant hearing loss. Fourteen variants in eleven candidate genes (ABCC3, OTOS, TPMT, SLC22A2, NFE2L2, SLC16A5, LRP2, GSTP1, SOD2, WFS1 and ACYP2) were investigated. Multinomial logistic regression was performed to model the relationship between genetic predictors and platinum ototoxicity, adjusting for clinical risk factors. Additionally, measures of the diagnostic accuracy of the genetic markers were determined., Results: 900 patients were included in this study. In the multinomial logistic regression, significant unique contributions were found from SLC22A2 rs316019, the age at the start of platinum treatment, cranial radiation and the interaction term [platinum compound]∗[cumulative dose of cisplatin]. The predictive performance of the genetic markers was poor compared with the clinical risk factors., Conclusions: PanCareLIFE is the largest study of cisplatin-induced ototoxicity to date and confirmed a role for the polyspecific organic cation transporter SLC22A2. However, the predictive value of the current genetic candidate markers for clinical use is negligible, which puts the value of clinical factors for risk assessment of cisplatin-induced ototoxicity back into the foreground., Competing Interests: Conflict of interest statement The authors declare no conflict of interest., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Purpose: The objective of this study was to investigate potential correlations between pathologic fractures (PFs) and prognosis of patients with primary central high-grade osteosarcoma of the extremities., Methods: We retrospectively analyzed 2,847 patients registered in the Consecutive Cooperative Osteosarcoma Study Group database with primary central high-grade osteosarcoma of the extremities, treated between 1980 and 2010. Intended treatment included pre- and postoperative chemotherapy and surgery. Univariable and multivariable survival analyses were performed for all patients and then differentiated for adult and pediatric (≤ 18 years at time of diagnosis) patients., Results: A total of 2,193 patients were ≤ 18 years of age; 11.3% of all patients had PFs. In the overall cohort, presence of PF correlated significantly with tumor site, histologic subtype, relative tumor size, and primary metastases, but not with body mass index or local surgical remission. In univariable analysis, 5-year overall survival (OAS) of patients with and without PF was 63% versus 71%, respectively ( P = .007), and 5-year event-free survival (EFS) was 51% versus 58% ( P = .026). In pediatric patients, OAS and EFS did not differ significantly between patients with and without PF. In adults, 5-year OAS in patients with and without PF was 46% versus 69% ( P < .001), and 5-year EFS was 36% versus 56% ( P < .001). In multivariable analysis, PF was not a statistically significant factor for OAS or EFS in the total cohort or in pediatric patients. In adult patients, PF remained an independent prognostic factor for OAS ( P = .013; hazard ratio [HR], 1.893). It was not a significant prognostic factor for EFS ( P = .263; HR, 1.312)., Conclusion: In this largest study to date with extremity osteosarcomas, we observed the occurrence of PF to correlate with inferior OAS expectancies in adult but not in pediatric patients.

Aims: Survival after cancer diagnosed during childhood or adolescence continues to improve with new treatments and supportive therapies. Optimal long-term care requires that risks to vulnerable organs are clearly defined and translated into guidelines that are implemented into practice. PanCareLIFE is a pan-European consortium that addresses survivorship issues comprising fertility, hearing impairment and quality of life. This article describes the scientific basis of PanCareLIFE's studies., Methods: PanCareLIFE involves 17 partner institutions from eight European countries, with additional 11 data providers from five other countries. Study designs and methods include molecular genetic, cohort and case-control studies, a longitudinal study and an intervention study. Ethics and data protection issues have been taken into account from the beginning., Results: PanCareLIFE will investigate the way that treatment impairs female fertility, by evaluating anti-Müllerian hormone levels and the underlying genetic susceptibility to loss of fertility. For our fertility studies, more than 6000 survivors have completed questionnaires, more than 1500 provided serum samples and more than 400 case-control triads have been identified. Fertility preservation guidelines for boys and girls will be developed. More than 2000 survivors have contributed audiograms for the ototoxicity study. Almost 1000 samples were sent for genetic analysis related to ototoxicity and gonadal reserve. The SF-36 questionnaire will measure quality of life in more than 10,000 survivors., Conclusions: The large number of subjects enrolled in PanCareLIFE and the detailed information accumulated will allow in-depth evaluation of important outcomes. Fertility preservation guidelines will help patients and their families make informed decisions and contribute to their long-term well-being., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations., Patients and Methods: Specialist centres collaborated to report prognostic factors and outcome for 113 patients., Results: Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25)., Conclusions: Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Data on 5572 children and adolescents diagnosed with malignant bone tumours (International Classification of Childhood Cancer, Group VIII) before the age of 20 years during 1978-1997 in Europe were extracted from the Automated Childhood Cancer Information System (ACCIS) database. Age-standardised incidence among children during the period 1988-1997 was similar for boys and girls aged 0-14 years (5.5-5.6 per million). Among adolescents aged 15-19 years, males had higher incidence (19.3 per million) than females (10.7 per million). Among children, osteosarcoma accounted for 51% of registrations and Ewing's sarcoma for 41%. Among adolescents, 55% of registrations were osteosarcoma and 28% Ewing's sarcoma. Both tumours had their highest incidence in late childhood or early adolescence. There were no significant time trends in incidence during 1978-1997. Five-year survival estimates for patients diagnosed during 1988-1997 were, respectively, 59% and 51% among children and adolescents with osteosarcoma and 62% and 30% among children and adolescents with Ewing's sarcoma. Between 1978-1982 and 1993-1997, survival increased for both children and adolescents with osteosarcoma, and for children with Ewing's sarcoma.

Periosteal osteosarcoma is a rare primary malignant bone tumour. Treatment is by surgical excision, but controversy remains about the value of chemotherapy. The members of the European Musculo Skeletal Oncology Society (EMSOS) collaborated to produce a dataset of 119 patients. The predominant site for the tumour was the femur, followed by the tibia. All but 2 patients underwent surgery, with 9 requiring amputation and the others having limb salvage. A total of 81 patients had chemotherapy, of whom 50 had neoadjuvant chemotherapy. There was no standard chemotherapy regime, but all patients receiving chemotherapy were given doxorubicin combined with at least one other agent. The overall survival was 89% at 5 years and 83% at 10 years. Eight patients developed local recurrence, of whom 5 died. Survival was related to appearance of local recurrence (P < 0.0001) but no other single factor. The use of chemotherapy was not shown to be a prognostic factor, but was used in two-thirds of the patients in this study.