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Background: WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare contiguous gene deletion syndrome with a 45% to 60% risk of developing Wilms tumor (WT). Currently, surveillance and treatment recommendations are based on limited evidence. Methods: Clinical characteristics, treatments, and outcomes were analyzed for patients with WAGR and WT/nephroblastomatosis who were identified through International Society of Pediatric Oncology Renal Tumor Study Group (SIOP‐RTSG) registries and the SIOP‐RTSG network (1989‐2019). Events were defined as relapse, metachronous tumors, or death. Results: Forty‐three patients were identified. The median age at WT/nephroblastomatosis diagnosis was 22 months (range, 6‐44 months). The overall stage was available for 40 patients, including 15 (37.5%) with bilateral disease and none with metastatic disease. Histology was available for 42 patients; 6 nephroblastomatosis without further WT and 36 WT, including 19 stromal WT (52.8%), 12 mixed WT (33.3%), 1 regressive WT (2.8%) and 2 other/indeterminable WT (5.6%). Blastemal type WT occurred in 2 patients (5.6%) after prolonged treatment for nephroblastomatosis; anaplasia was not reported. Nephrogenic rests were present in 78.9%. Among patients with WT, the 5‐year event‐free survival rate was 84.3% (95% confidence interval, 72.4%‐98.1%), and the overall survival rate was 91.2% (95% confidence interval, 82.1%‐100%). Events (n = 6) did not include relapse, but contralateral tumor development (n = 3) occurred up to 7 years after the initial diagnosis, and 3 deaths were related to hepatotoxicity (n = 2) and obstructive ileus (n = 1). Conclusions: Patients with WAGR have a high rate of bilateral disease and no metastatic or anaplastic tumors. Although they can be treated according to existing WT protocols, intensive monitoring of toxicity and surveillance of the remaining kidney(s) are advised. Lay Summary: WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare genetic condition with an increased risk of developing Wilms tumor.In this study, 43 patients with WAGR and Wilms tumor (or Wilms tumor precursor lesions/nephroblastomatosis) were identified through the international registry of the International Society of Pediatric Oncology Renal Tumor Study Group (SIOP‐RTSG) and the SIOP‐RTSG network. In many patients (37.5%), both kidneys were affected. Disease spread to other organs (metastases) did not occur.Overall, this study demonstrates that patients with WAGR syndrome and Wilms tumor can be treated according to existing protocols. However, intensive monitoring of treatment complications and surveillance of the remaining kidney(s) are advised. Patients with WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) and Wilms tumor/nephroblastomatosis, identified through the International Society of Pediatric Oncology Renal Tumor Study Group registries and network, have a high rate of bilateral disease and no metastatic or anaplastic tumors. Although they can be treated according to existing Wilms tumor protocols, intensive monitoring of toxicity and surveillance of the remaining kidney(s) are advised. [ABSTRACT FROM AUTHOR]

Platinum is extensively used in the treatment of several childhood cancers. However, ototoxicity is one of the most notable adverse effects, especially in children. Several studies suggest that genetics may predict its occurrence. Here, polymorphisms associated with platinum-induced ototoxicity were selected from the literature and were investigated in a pediatric population treated with platinum-based agents. In this retrospective study, patients treated with cisplatin and/or carboplatin were screened. The patients with pre- and post-treatment audiogram (Brock criteria) available were included. We selected polymorphisms that have previously been associated with cisplatin ototoxicity with a minor allele frequency ≥30%. Deletion of GSTM1 and GSTT1 , rs1799735 ( GSTM3 ), rs1695 ( GSTP1 ), rs4880 ( SOD2 ), rs2228001 ( XPC ), rs1799793 ( XPD ) and rs4788863 ( SLC16A5 ) were investigated. Data of one hundred and six children matching the eligible criteria were analyzed. Thirty-three patients (31%) developed ototoxicity (with a Brock grade ≥2). The probability of hearing loss increased significantly in patients carrying the null genotype for GSTT1 (P = 0.03), A/A genotype at rs1695 (P = 0.01), and C/C genotype at rs1799793 (P = 0.008). We also showed an association of the cumulative doses of carboplatin with cisplatin ototoxicity (P <0.05). To conclude, deletion of GSTT1 , rs1695 and rs1799793 may constitute potential predictors of platinum-induced ototoxicity., Competing Interests: CONFLICTS OF INTEREST The authors declare no conflicts of interest.

Within the second "Cancer plan" 2009-2013, the French national institute of cancer (INCa) recommended the implementation of programs dedicated to adolescents and youngs adults (AYA) with cancer. In this context and in parallel to the specific medical care developed for AYA, the Oscar-Lambret center created a psycho-social-educational team including among others a social worker (SW) and a special educational teacher (SET), offering multidisciplinary qualifications and views. The social approach, realized as a pair by SW-SET, takes into account every aspect of each AYA (family, academic, career/professional, personal, cultural aspects…). We expose the first 2 years' experience of this special program for AYA through diagnosis to remission time. For this period, 164 AYA were seen by the social professionals, with a total of 602 consultations in the unit. The number of these consultations depended on the needs of AYA and their family. Nevertheless, only 10 AYA required no further intervention (6.1 %). The study highlights that the social interventions are most frequently about scolarity, work and disability recognition. These 2 years of experience of the SW-SET team offered a way to reflect upon our values and our culture, and on the role of the social worker in a medical setting. Each AYA has a personal story, which affects significantly the way to overcome the challenges that come with the disease. Our findings underscore the need for AYA with cancer to have access to personalized supportive care, encouraging them in pursing their personal goals and rewarding themselves., (Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Therapeutic education (TE) is a practice developed over 40 years at an international level to give people with chronic illness the skills necessary to help them better manage their disease. The lengthening survival time of cancer patients as well as changes in the patient-caregiver relationship have contributed to the development of TE in oncologic diseases. Every year in France, about 1900 adolescents and young adults (15-25 years old) are diagnosed with cancer which is the second leading cause of death in this age group. The observed survival rates for these patients are lower when compared with children's. Some of the hypotheses put forward to explain this difference include a lack of constancy in care and a non-following treatment, as failure to adhere to therapies is common in this age group. "Go-AJA", an interdisciplinary national organization established in 2012, aims to improve the quality of care and treatment results for AYA living with cancer. Therapeutic education for AYA in oncology is an active working group of "Go-AJA" and intends to draw recommendations and to improve adapted communication on different education topics. Elaboration and preparation of TE programs by skilled multidisciplinary teams engaged in interactive educational actions is the first and most crucial step., Materials and Methods: The TE "Go-AJA" working group has federated pediatric and adult oncologists, nurses, psychologists, TE professionals, and resource patients, thanks to the commitment of professionals from the 8 national teams supported by the National Cancer Institute. Physical meetings and conference calls were organized from 2012 to 2015 to construct TE tools and programs for AYA with cancer., Results: A competence referential was built and adapted to AYA population with cancer, after focused groups organized in 2 main oncology centers with on-therapy sarcoma patients and members of the multidisciplinary TE working group. Tools were validated and adapted to adolescents or young adults with cancer, to help in the 4 stages of TE: the "educational diagnosis" allowing the caregiver to better understand the patient in his life journey with the disease; the "therapeutic alliance" allowing to agree with the patient on his/her priorities; the "implementation" which is an action step: information, awareness, learning and psychosocial support. The final step called "assessment" allows the caregiver to take stock on the changes and difficulties with the patient. TE for AYA with cancer included individual and/or group sessions to improve self-care skills: knowledge about the disease (group sessions "what is cancer?" with use of microscopes to visualize sarcoma cells, and guided tours in a tumor research laboratory), and details about the treatment and its consequences (workshops about "management of febrile neutropenia"). Moreover, TE aimed to enrich the field of coping skills, in particular to improve the coordination and experience of cares between the different complex and varied network of care (group and/or individual sessions focused on physical rehabilitation, and adapted school/professional orientation)., Conclusion: Regardless of the care system, care workers dedicated to AYA with cancer should use TE-specific actions to reinforce treatment participation and therapeutic relationships. This active multidisciplinary TE working group dedicated to AYA with cancer elaborated TE programs by skilled multidisciplinary teams engaged in interactive educational actions. After this work of a national TE organization, more studies using methodological tools are still required to evaluate the impact of such implemented programs on the treatment results and the quality of life., (Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Clear cell sarcoma of the kidney (CCSK) is a rare tumor that is diagnosed most often in children between 2- and 4-years-old of age. Usually, patients with CCSK are treated in international study for intrarenal tumors, preferentially Wilms tumor, according to bad histopronostic group. The purpose of this paper is to review the most important features in 2015 about epidemiology, radiology, anatomopathology and genetic of CCSK, and above all a synthesis about successive treatment strategies with their results. Second most common pediatric renal tumor in children less than 5-years-old, its prognosis has improved dramatically in recent years with the use of anthracyclines., (Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Impaired renal function may occur following multimodal treatment of cancer in childhood. Renal late effects caused by chemotherapy, renal surgery and/or radiotherapy are now well described; but little is known about their prevalence and time of development. Herein, we provide a synthesis of the different renal complications that may occur with their physiopathology in relation with specific treatment exposures. This review summarized the literature that supported the recommendations issued by the long-term follow-up group of the "Société française des cancers de l'enfant (SFCE)" for childhood cancer survivors at risk for nephrotoxicity (www.sfce.org ; www.soc-nephrologie.org/SNP/index.htm). We developed these monitoring elements and the lifestyle recommendations for all asymptomatic survivors., (Copyright © 2015 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Given the excellent survival rate of children and teenagers treated for cancer, careful should be given in order to minimize long-term late effects of treatments and to improve quality of life. In this regard, fertility preservation is particularly important. Age, sex and concomitant therapies have an impact on the level of risk of hypofertility, with a stronger risk for chemotherapeutic regimens including alkylating agents, and gonads or hypothalamo-pituitary axis radiotherapy. Assessment of gonadotoxic impact of treatment strategy and fertility preservation techniques are crucial for limiting late effects. The objective of this literature review is to develop guidance for pediatric oncologists and hematologists relative to fertility preservation methods, in children and teenagers receiving gonadotoxic treatment. Specific follow-up after treatment is also proposed.

Germ cell tumours include a group of highly heterogeneous tumours regarding to their clinical and histological appearance. Altogether, they represent 3% of cancers diagnosed in children and adolescent younger than 15 years. A bimodal age distribution is observed with a small peak during infancy and a larger peak after puberty. Non-seminomateous germ cell tumours are largely predominant as compared to seminomateous tumours, rarely seen before puberty. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or yolk sac tumours in infants above three months. In adolescents, mixed germ cell tumours predominate with either gonadal, mediastinal or intracranial tumour. Surgical resection of the tumour is fundamental and must be carcinologic and conservative at the same time. Neoadjuvant chemotherapy may help to decrease the volume of the tumour making possible a delayed sparing surgery. Indeed, except for teratomas, these tumours are highly sensitive to chemotherapy, in particular to platinum salts. Prognosis is good even in metastatic diseases. This raises the question of a therapeutic de-escalation in an attempt to decrease long-term toxicity, in particular audiologic and renal impairment. On the opposite, recurrent or refractory diseases after chemotherapy carry a dismal prognosis and therapeutic strategies remain to be defined in such situations.