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BackgroundThis report documents the clinical characteristics, molecular grouping, and outcome of young children with ependymoma treated prospectively on a clinical trial.MethodsFifty-four children (aged ≤3 y) with newly diagnosed ependymoma were treated on the St Jude Young Children 07 (SJYC07) trial with maximal safe surgical resection, 4 cycles of systemic chemotherapy, consolidation therapy using focal conformal radiation therapy (RT) (5-mm clinical target volume), and 6 months of oral maintenance chemotherapy. Molecular groups were determined by tumor DNA methylation using Infinium Methylation EPIC BeadChip and profiled on the German Cancer Research Center/Molecular Neuropathology 2.0 classifier.ResultsOne of the 54 study patients had metastases (cerebrospinal fluid positive) at diagnosis. Gross or near-total resection was achieved in 48 (89%) patients prior to RT. At a median follow-up of 4.4 years (range, 0.2-10.3 y), 4-year progression-free survival (PFS) was 75.1% ± 7.2%, and overall survival was 92.6% ± 4.4%. The molecular groups showed no significant difference in PFS (4-year estimates: posterior fossa ependymoma group A [PF-EPN-A; 42/54], 71.2% ± 8.3%; supratentorial ependymoma positive for v-rel avian reticuloendotheliosis viral oncogene homolog A [ST-EPN-RELA; 8/54], 83.3% ± 17.0%; and supratentorial ependymoma positive for Yes-associated protein [4/54], 100%, P = 0.22). Subtotal resection prior to RT was associated with an inferior PFS compared with gross or near-total resection (4-year PFS: 41.7% ± 22.5% vs 79.0% ± 7.1%, P = 0.024), as was PF-EPN-A group with 1q gain (P = 0.05). Histopathologic grading was not associated with outcomes (classic vs anaplastic; P = 0.89).ConclusionsIn this prospectively treated cohort of young children with ependymoma, ST-EPN-RELA tumors had a more favorable outcome than reported from retrospective data. Histologic grade did not impact outcome. PF-EPN-A with 1q gain and subtotal resection were associated with inferior outcomes.

PURPOSE:The Children's Oncology Group trial ACNS0121 estimated event-free survival (EFS) and overall survival for children with intracranial ependymoma treated with surgery, radiation therapy, and-selectively-with chemotherapy. Treatment was administered according to tumor location, histologic grade, and extent of resection. The impacts of histologic grade, focal copy number gain on chromosome 1q, and DNA methylation profiles were studied for those undergoing surgery and immediate postoperative conformal radiation therapy (CRT). METHODS:ACNS0121 included 356 newly diagnosed patients (ages 1 to 21 years). Patients with classic supratentorial ependymoma were observed after gross total resection (GTR). Those undergoing subtotal resection received chemotherapy, second surgery, and CRT. The remaining patients received immediate postoperative CRT after near-total resection or GTR. CRT was administered with a 1.0-cm clinical target volume margin. The cumulative total dose was 59.4 Gy, except for patients who underwent GTR and were younger than age 18 months (who received 54 Gy). Patients were enrolled between October 2003 and September 2007 and were observed for 5 years. Supratentorial tumors were evaluated for RELA fusion; infratentorial tumors, for chromosome 1q gain. Classification of posterior fossa groups A and B was made by methylation profiles. RESULTS:The 5-year EFS rates were 61.4% (95% CI, 34.5% to 89.6%), 37.2% (95% CI, 24.8% to 49.6%), and 68.5% (95% CI, 62.8% to 74.2%) for observation, subtotal resection, and near-total resection/GTR groups given immediate postoperative CRT, respectively. The 5-year EFS rates differed significantly by tumor grade (P = .0044) but not by age, location, RELA fusion status, or posterior fossa A/posterior fossa B grouping. EFS was higher for patients with infratentorial tumors without 1q gain than with 1q gain (82.8% [95% CI, 74.4% to 91.2%] v 47.4% [95% CI, 26.0% to 68.8%]; P = .0013). CONCLUSION:The EFS for patients with ependymoma younger than 3 years of age who received immediate postoperative CRT and for older patients is similar. Irradiation should remain the mainstay of care for most subtypes.

Background Methods Results Conclusions Survival rates from childhood cancer continue to increase, with an ongoing interest in long‐term survivorship. Although infertility and gonadal failure are well recognized in Hodgkin lymphoma (HL) survivors, sexual dysfunction is less studied. The objective of this study was to compare the prevalence of sexual dysfunction in HL survivors with that in matched community controls.Long‐term survivors of HL (n = 186; female, 51.61%; mean age at diagnosis. 14.41 years [range, 3.01–22.60 years]; current mean ± standard deviation age, 36.73 ± 7.93 years) and matched community controls (n = 182; female, 50.55%; mean ± standard deviation age, 36.41 ± 9.02 years) completed a comprehensive, in‐person clinical assessment, laboratory battery, and the International Index of Erectile Function or the Female Sexual Function Index questionnaire.Male survivors had increased levels of erectile dysfunction (18.89% vs. 6.67%; p = .0239) but indicated no difference in sexual desire. Female survivors had a higher prevalence of sexual dysfunction compared with female controls (46.88% vs. 15.22%; p < .0001) and an increased prevalence of moderate‐to‐severe loss of sexual desire (38.04% vs. 23.26%; p = .0361). Female survivors with sexual dysfunction indicated increased levels of anxiety (p = .0184), depression (p = .0153), and worse physical and mental health (p = .0141 and p = .0419, respectively). Male survivors with erectile dysfunction had higher rates of anxiety and impaired physical health (p = .0147 and p = .0266, respectively).Sexual dysfunction was prevalent in this childhood and adolescent Hodgkin lymphoma survivor cohort and was associated with effects on quality of life. Health care providers must recognize the need for screening and intervention in this group to hopefully contribute to improved overall quality of life. [ABSTRACT FROM AUTHOR]

Background: Data on primary hypothyroidism and its long‐term impact on the health, cognition, and quality of life (QOL) of childhood cancer survivors are limited. This study examined the prevalence of and risk factors for primary hypothyroidism and its associations with physical, neurocognitive, and psychosocial outcomes. Methods: This was a retrospective study with a cross‐sectional health outcome analysis of an established cohort comprising 2965 survivors of childhood cancer (52.8% male; median current age, 30.9 years, median time since cancer diagnosis, 22.3 years). Multivariable logistic regression estimated odds ratios (ORs) and 95% confidence intervals (CIs) for associations between primary hypothyroidism and cancer‐related risk factors, cardiovascular disease risk factors, frailty, neurocognitive and QOL outcomes, social attainment, and subsequent thyroid carcinoma. Associations between serum free thyroxine and thyrotropin levels at assessment and health outcomes were explored. Results: The prevalence of primary hypothyroidism was 14.7% (95% CI, 13.5%‐16.0%). It was more likely in females (OR, 1.06; 95% CI, 1.03‐1.08), was less likely in non‐Whites (OR, 0.96; 95% CI, 0.93‐0.99), was associated with thyroid radiotherapy (higher risk at higher doses), and was more common if cancer was diagnosed at an age ≥ 15.0 years versus an age < 5 years (OR, 1.05; 95% CI, 1.01‐1.09). Primary hypothyroidism was associated with frailty (OR, 1.54; 95% CI, 1.05‐2.26), dyslipidemia (OR, 1.52; 95% CI, 1.14‐2.04), impaired physical QOL (OR, 1.66; 95% CI, 1.12‐2.48), and having health care insurance (OR, 1.51; 95% CI, 1.07‐2.12). Conclusions: Primary hypothyroidism is common in survivors and is associated with unfavorable physical health and QOL outcomes. The impact of thyroid hormone replacement practices on these outcomes should be investigated further. In this study, primary hypothyroidism affects 14.7% of 2965 survivors of childhood cancer, and it is associated with adverse health and quality of life. Associations between thyroid function tests and health outcomes are explored. [ABSTRACT FROM AUTHOR]

11-17 Health outcomes research findings have had a major influence on changes in paediatric cancer therapy and have informed health-screening strategies of childhood cancer survivors. 7-10 Current understanding of late health outcomes in adult survivors of childhood cancer has largely been the result of a number of cohort studies that have been instrumental in identifying, quantifying and characterizing cancer treatment-related health risks. Robust characterization of health outcomes and cancer-, cancer treatmentand health behaviour-related exposures provide an unparalleled opportunity to identify novel associations and to refine our understanding of the effects of cancer and its treatment on the health of aging survivors. Whereas SJLIFE does not specify age at cancer diagnosis, SJCRH generally restricts acceptance to children<25 years of age at the time of cancer diagnosis. [Extracted from the article]