Response assessment in paediatric intracranial ependymoma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group.

Response criteria for paediatric intracranial ependymoma vary historically and across different international cooperative groups. The Response Assessment in the Pediatric Neuro-Oncology (RAPNO) working group, consisting of an international panel of paediatric and adult neuro-oncologists, neuro-radiologists, radiation oncologists, and neurosurgeons, was established to address both the issues and the unique challenges in assessing the response in children with CNS tumours. We established a subcommittee to develop response assessment criteria for paediatric ependymoma. Current practice and literature were reviewed to identify major challenges in assessing the response of paediatric ependymoma to clinical trial therapy. For areas in which data were scarce or unavailable, consensus was reached through an iterative process. RAPNO response assessment recommendations include assessing disease response on the basis of changes in tumour volume, and using event-free survival as a study endpoint for patients entering clinical trials without bulky disease. Our recommendations for response assessment include the use of brain and spine MRI, cerebral spinal fluid cytology, neurological examination, and steroid use. Baseline postoperative imaging to assess for residual tumour should be obtained 24-48 h after surgery. Our consensus recommendations and response definitions should be prospectively validated in clinical trials.

Development and field testing of a neuro psychomotor multidimensional grid for the assessment of children with cns tumor.

Central Nervous System (CNS) tumors are the most common pediatric solid tumor and development neuro psychomotor (DNPM) therapy can contribute to the rehabilitation of these children. This paper describes the development of a DNPM multidimensional assessment grid for children with CNS tumor (DNPM-CNS grid).The development process included 4 phases: (P1) literature review and grid development (Version 1.0), (P2) two rounds consultations with experts (Version 1.1 and 2.0), (P3) field testing, (P4) final revision (Version 3.0).(P1) The DNPM-CNS grid was developed based on previous tools and manuals and on clinical experience with this patient population. (P2) A total of 52 questionnaires were filled in by experts about relevance of assessment areas, pertinence, comprehensibility and feasibility of the grid. Average scores ranged from 7.6 to 10. (P3) At case level, good inter-rater agreement scores (78%) and limited non-evaluability rates (18%) emerged. At item level, 27% of items reached high disagreement and 26% high not-evaluability rates. The qualitative assessment was judged clinically useful for planning the neuro-oncology rehabilitation treatment and a good feasibility of the DNPM-CNS grid emerged both for preschool and school-age children. (P4) The final version of the grid consists of 8 assessment areas with 133 items.The DNPM-CNS grid is a comprehensive tool that can guide the overall DNPM assessment in a limited amount of time. It can be used as a screening tool to customize more specific assessments. Further research is needed to better characterize grid psychometric properties.Supplemental data for this article is available online at https://doi.org/10.1080/08880018.2021.1948648 .

Adolescents with cancer on privacy: Fact-finding survey on the need for confidentiality and space.

BACKGROUND: Cancer diagnosis and related treatment can limit young patients' privacy. Their need for private physical and psychological spaces can be impeded by limited freedom, e.g., sharing hospital rooms with others and exposing their body to their parents and doctors. METHODS: We conducted a survey among young patients of our center to investigate how their perception of privacy changed after being diagnosed with cancer and their need for hospital dedicated physical spaces and time for patients to enjoy their privacy. A questionnaire with 16 items was developed by the staff involving psychologists and physicians. The relevance of changes before and after the cancer diagnosis was assessed by calculating two scores indicating the situation before and after diagnosis for each answer, as well as a delta score. RESULTS: Between May and June 2020, the questionnaire was completed by 60 patients aged 16-24 years. The median delta scores describing the changes before and after diagnosis were -8 points for perception of privacy, indicating a significant decrement of the level of privacy perceived. A major issue was the constant presence of parents being perceived as intrusive. Concerning hospital dedicated physical spaces and time for patients to enjoy their privacy, respondents requested dedicated spaces (50% of patients) and opportunities to have private interviews with medical personnel (88%). CONCLUSIONS: Our study offers a snapshot of how young people with cancer perceive the impact of the disease and its treatment on their privacy with the restrictions imposed on their individual freedom. Patients' personal needs must be taken into consideration to adopt appropriate measures and better organize wards.

Application of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric oncology patients: A multicenter study.

Posterior cranial fossa (PCF) tumors in childhood are often associated with ataxia as well as other motor, neurobehavioral and linguistic impairment. The use of a reliable outcome measure is mandatory to evaluate the severity of impairment and monitor rehabilitation effectiveness. The aim of this work is to explore the validity of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric subjects with ataxia secondary to PCF tumor resection and evaluate the influence of age and comorbidities. Seventy eight patients (3-18 years) were recruited in 5 centers from 2016 to 2018. The age effect on SARA was analyzed by correlating total SARA scores and item scores with age and gradually excluding youngest subjects. The comorbidity effect was evaluated by comparing the ataxia-only group vs a group of subjects with ataxia + dysfunction of cranial nerves or cerebellar mutism (CM) and a group of patients with ataxia + hemiparesis. Several negative correlations between SARA scores and age were found under age 9. Differences between ataxia-only group and the other two groups were closely associated with specific comorbidities (e.g. speech disturbance in cranial nerves or CM group (p value < 0.001) and gait, stance, sitting and finger chase in the hemiparetic group (mean p value 0.022)).

Response assessment in diffuse intrinsic pontine glioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group.

Optimising the conduct of clinical trials for diffuse intrinsic pontine glioma involves use of consistent, objective disease assessments and standardised response criteria. The Response Assessment in Pediatric Neuro-Oncology working group, consisting of an international panel of paediatric and adult neuro-oncologists, clinicians, radiologists, radiation oncologists, and neurosurgeons, was established to address issues and unique challenges in assessing response in children with CNS tumours. A working group was formed specifically to address response assessment in children and young adults with diffuse intrinsic pontine glioma and to develop a consensus on recommendations for response assessment. Response should be assessed using MRI of brain and spine, neurological examination, and anti-inflammatory or antiangiogenic drugs. Clinical imaging standards are defined. As with previous consensus recommendations, these recommendations will need to be validated in prospective clinical trials.

PD-L1 assessment in pediatric rhabdomyosarcoma: a pilot study.

BACKGROUND: Rhabdomyosarcomas (RMSs) are the most frequent soft tissue sarcoma in children and adolescents, defined by skeletal muscle differentiation and the status of FOXO1 fusions. In pediatric malignancies, in particular RMS, scant and controversial observations are reported about PD-L1 expression as a putative biomarker and few immune checkpoint clinical trials are conducted. METHODS: PD-L1 assessment was evaluated by immunohistochemistry (IHC) utilizing two anti-PDL1 antibodies, in a pilot cohort of 25 RMS. Results were confirmed in primary and commercial RMS cell lines by cytofluorimetric analysis and IHC. RESULTS: PD-L1 expression was detectable, by both anti-PD-L1 antibodies, in the immune contexture of immune cells infiltrating and/or surrounding the tumor, in 15/25 (60%) RMS, while absent expression was observed in neoplastic cells. Flow cytometry analysis and PD-L1 IHC of commercial and primary RMS cell lines confirmed a very small percentage of PD-L1 positive-tumor cells, under the detection limits of conventional IHC. Interestingly, increased PD-L1 expression was observed in the immune contexture of 4 RMS cases post chemotherapy compared to their matched pre-treatment samples. CONCLUSION: Here we identify a peculiar pattern of PD-L1 expression in our RMS series with scanty positive-tumor cells detected by flow cytometry, and recurrent expression in the immune cells surrounding or infiltrating the tumor burden.

Adolescents' Health Awareness and Understanding of Cancer and Tumor Prevention: When and Why an Adolescent Decides to Consult a Physician.

BACKGROUND: A feature often seen in adolescent patients with cancer is a lengthy symptom interval, especially in comparison with children. It has been suggested that inadequate awareness of cancer risk among adolescents may play an important role in this study. METHODS: The Società Italiana Adolescenti con Malattie Onco-ematologiche and the Fondazione Umberto Veronesi conducted a survey to investigate health awareness among healthy adolescents and their understanding of cancer and its signs and symptoms by commissioning a specialized agency (AstraRicerche). A questionnaire was administered to 500 Italian adolescents from the age group of 15 to 19 years using the computer-aided web interviewing method. RESULTS: Approximately 80% of the adolescents interviewed claimed to be well informed about their own health, 85% said they were aware that some lifestyle habits could influence their health, and 80% reported that they know that cancer can develop in adolescence too. It was also noted that, while some adolescents were worried about a given symptom, 22% of them reportedly preferred a wait-and-see approach (either to avoid alarming their parents, or they hoped that the symptom would be temporary). CONCLUSIONS: This study showed that a majority of adolescents are concerned about their own health, but sometimes prefer not to report their symptoms to anyone. Hence, it is important to develop information campaigns tailored to raise awareness among this age group and help them interpret their symptoms.

Female fertility preserving practices at a pediatric unit: a challenge of multiprofessional and multidisciplinary cooperation.

INTRODUCTION: Being able to have children could have an important positive effect on the future lives of pediatric cancer survivors. Working at a cancer institute makes us responsible for filling the gaps in our knowledge in this area of patient care. METHODS: We describe our activities in a series of young females diagnosed with cancer and evaluated for fertility preservation options. We discuss the developed skills and organization as well as the practical difficulties encountered in managing fertility preservation. RESULTS: Since September 2012, laparoscopy and cryopreservation of cortical ovarian tissue has been performed in 16 girls (with ovary transposition in 3, and after several cycles of chemotherapy in 5) and egg banking in 4 young women (before chemotherapy in 2 and several years after treatment in 2). CONCLUSIONS: Recommendations on fertility preservation indicate that discussing the problems early on is crucial to future success. It is unthinkable to simply provide information and offer the opportunity to choose a fertility preserving technique without helping and accompanying patients and their families in their decisions and choices on the matter.

[Adolescents with cancer: the "Youth Project" at the Istituto Nazionale Tumori in Milan]. / Gli adolescenti ammalati di tumore: il "Progetto Giovani" dell'Istituto Nazionale dei Tumori di Milano.

Adolescents with cancer are a particular group of patients who are less likely to gain access to optimal cancer services at comprehensive cancer Centers: many studies suggest adolescents fare less well than children with the same disease. The paper describes the key issues of the "Youth Project" of the Pediatric Oncology Unit IRCCS Fondazione Istituto Nazionale Tumori in Milan, dedicated to adolescents (over 15 years old) and young adults (up to 25 years old) with solid tumors. This project is a possible clinical and organizational model to address the unique needs of patients in this age group and for bridge the gap in access to care and in recruitment in clinical trials, in clinical and psycho-social management and in curves of healing. The paper also describes the activity of the Adolescent Commission established by the Italian Pediatric Hematology Oncology (AIEOP).

The Youth Project at the Istituto Nazionale Tumori in Milan.

The paper describes the key issues of the Youth Project launched in 2011 at the pediatric oncology unit of the Istituto Nazionale Tumori in Milan dedicated to adolescents (over 15 years old) and young adults (up to 25 years old) with solid tumors. The Youth Project was developed within the pediatric oncology unit in the conviction that adolescent patients may benefit from the multidisciplinary team typical of the pediatric oncology setting, as well as the expertise in treating pediatric-type malignancies and enrolling patients in clinical trials. The project was an offshoot of existing activities, making no major changes to the hospital's organization and posing no major demands on the institution's administration and board. Patients are managed by the pediatric oncology staff, but they have access to particular services (e.g., regarding their psychosocial support, fertility preserving measures, access to care after completing therapy); dedicated, adequately equipped multifunctional rooms have been provided. The location of the pediatric unit within a cancer referral center and the cooperation with divisions dedicated to adults have played an important role in the project's creation.

Psychological assessment of women on an early breast screening program after radiotherapy to the chest wall for childhood cancer.

AIMS AND BACKGROUND: This study investigates the psychological status in a population of female patients who received chest irradiation for a childhood cancer and were screened for second primary breast cancer. METHODS: Sixty-eight consecutive such young women were included. Compilation of the Crown-Crisp Index questionnaire was requested and 49/68 patients accepted to fill it in; 14 women in the sample had children (28%). RESULTS: Twenty-seven of 49 patients achieved a normal score, whereas in 22 the score was slightly above the normal range in at least one scale. Pathological scores were more frequent among the women without children. CONCLUSION: Quality of life in this series of long-term survivors does not seem to be severely affected by previous treatment for cancer nor by the concern for the onset of a second primary malignancy.