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Purpose: Children diagnosed with a brain tumor (BT) in the first years of their life are at high risk of cognitive and neuropsychological problems, more school difficulties, and an increased need for educational support. To improve this condition, it will be beneficial to be able to identify the neuropsychological variables that are early predictors of school competences at later ages., Methods: We longitudinally assessed 30 school-age BT children with a diagnosis before the age of 5 who were administered cognitive and neuropsychological evaluations before entering school or in the first 2 school years and who were followed up for academic performance at least one year after the first evaluation. A discriminant function analysis was conducted to detect the early neuropsychological profile that best predicted those children who turned out to need school support or not; we tested 5 block multiple regression models, one for each academic variable entering as predictors the neuropsychological variables that significantly discriminated the two groups., Results: A total of 93.3% of the cases were correctly classified according to the discriminant function in "with vs. without" educational support. Visual attention abilities were highly correlated with resulting school problems, both for reading (accuracy and speed) and math (operations) at school age., Conclusions: Analysis provided evidence that the early neuropsychological profile may predict academic difficulties for both reading and math at school age and that visual attention seems to play an important role in both these academic abilities, allowing clinicians to identify children with major difficulties in/from early years and to intervene beforehand.

Purpose: Effects of tumor location on cognitive performance of patients with brain tumor are controversial: some studies reported higher risks related to supratentorial locations, some to infratentorial locations, and still others did not find any differences. We aimed to address this issue by comparing school-aged children with supratentorial or infratentorial tumor with respect not only to cognitive outcomes but also to the associations between core cognitive domains and academic abilities., Methods: 32 children with infratentorial tumor and 22 with supratentorial tumor participated in the study. To detect relationships among cognitive domains, we tested which neuropsychological variable(s) predicted academic skills, controlling for the effects of radiotherapy and time since diagnosis., Results: Radiotherapy and time since diagnosis, but not tumor location, predicted cognitive outcomes. Radiotherapy negatively influenced attention and executive functioning, as well as reading speed and arithmetic operations accuracy. Unexpectedly, longer time since diagnosis was associated with improvement in attention and reading speed. Tumor location showed an effect on the relationships between core cognitive domains and academic skills: verbal and visual-spatial memory influenced reading and mathematical performance in supratentorial patients; in infratentorial patients, an only effect of visual-spatial memory on mathematical performance was detected., Conclusions: Tumor location seems not to influence cognitive performance, while radiotherapy constitutes a key risk factor for cognitive impairment. Attentional and reading abilities may improve over time, possibly due to the weakening of cancer care effects. Different patterns of cognitive associations seem to characterize supratentorial and infratentorial patients, probably associated with different neuroplastic reorganization processes after tumor occurrence.

Medulloblastoma and central nervous system (CNS) germ cell tumors are very rare in adults, while they account for 25% and 5% of brain tumors in children, respectively (Pastore et al. Eur J Cancer 42:2064-208, 2006). Pediatric experiences, mostly from randomized and controlled clinical trials, have led to different tailored treatments, based on various risk factors, including histology, and extent of disease. For medulloblastoma, biological features have recently emerged that enable therapies to be scaled down in some cases, or pursued more aggressively in the event of chromosomal and/or genetic alterations (Massimino et al. Crit Rev Oncol Hematol 105:35-51, 2016). Such refinements are still impossible for adult patients due to the lack of similar clinical trials that might provide the same or a different understanding regarding patients' prognosis, long-term survival, quality of life, and acute and late toxicities. This review aims to contribute to the debate on the treatment of adults with these two diseases and promote the creation of broad-based, national and international trials to advance our knowledge in this area and to share the skills between pediatric and adult oncologists as adolescent and young adults (AYA) brain tumor national boards are currently requiring.

Background: Primary pediatric extraspinal sacrococcygeal ependymoma (ESE) is a very rare disease, poorly described in literature, whose diagnostic, therapeutic, and follow-up approach is still controversial., Methods: We describe six cases of pediatric ESE treated at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers in Italy since 1983, with a review of the literature., Results: All six patients had primary sacrococcygeal disease (two presacral and four subcutaneous) with median age of 10 years. Three patients were males, and two of them are metastatic at diagnosis; 3/6 had myxopapillary ependymoma grade I and 3/6 had classic ependymoma grade II. Five patients underwent surgical resection with complete removal only in one case with coccygectomy. Adjuvant chemoradiotherapy was administered to one metastatic patient obtaining a complete remission. Two patients relapsed at 3 and 8 years from diagnosis: they were treated with salvage chemotherapy (high-dose sequential chemotherapy with myeloablative regimen in one case), surgery, and radiotherapy achieving complete remission (CR). All six patients are in complete continuous remission (CCR) at a median follow-up of 12.8 years., Conclusions: Pediatric patients with this peculiar disease need to be referred to specialized pediatric cancer centers that can provide multidisciplinary treatment after a centralized pathology review. Our experience highlights the role of chemotherapy and radiotherapy in adjuvant and relapse setting. The final prognosis is relatively optimistic, but with a careful follow-up due to the high risk of recurrence.

Purpose: An intensive therapeutic strategy for metastatic medulloblastoma was launched in 1998 in our Institution. The aim of this study was to examine the long-term quality of life (QoL) in survivor patients at least 3 years after the end of the treatment., Methods: Patients were asked to complete self-administered QoL questionnaires. An index of physical impairment (IPI) was scored (range 0-100; the lower the score the better) based on clinical objective observations. Patients were divided into two groups (lower IPI group, and higher IPI group) and descriptively compared accordingly., Results: The study was completed by 25/33 eligible patients. Despite patients with a higher IPI reported worse perceived health condition, they had better emotional and psychological scores than those with a lower IPI in all QoL questionnaires., Conclusion: In our sample, patients with more severe objective and perceived physical impairments reported a better psychosocial QoL, possibly because the greater attention paid to them by society and family contributes to a better adjustment in long-term survivors. On this base, it should be recommended that all survivors receive a strong support as the most impaired patients.

Introduction: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system is a highly aggressive neoplasm which prevalently affects children and is characterized by a severe prognosis., Case Presentation: The authors describe an extremely rare case of a primary spinal AT/RT that occurred in a young girl. The patient underwent a wide surgical resection of a lumbar mass, followed by aggressive chemotherapy, myeloablative treatment, and local radiotherapy. After 7 months from the end of the treatment, the patient experienced local recurrence and was treated with surgery and second-line chemotherapy with antiangiogenic purposes, consisting of oral vinorelbine, cyclophosphamide, and celecoxib. Treatment was well tolerated, and patient was still alive 36 months after diagnosis., Conclusion: The peculiarity of this case report is the clinical-radiological response to a metronomic therapy in a case of early-relapsing spinal AT/RT despite previous maximal surgery, chemotherapy, and radiotherapy.

Purpose: We retrospectively report strategies used for medulloblastoma patients progressing after craniospinal irradiation where we aimed for: symptom control, a satisfactory quality of life, accrual in phase 1-2 trials, when available, and the first two conditions could no longer be satisfied by already experienced second-line strategies., Methods: Surgery was used in cases of doubtful relapse or when only one site was affected. Radiotherapy was given whenever possible, especially to relieve symptoms. The main chemotherapy regimens were oral temozolomide/etoposide, intravenous (iv.) cisplatin/etoposide, iv. gemcitabine/oxaliplatin, an oral sonic hedgehog pathway inhibitor and oral melphalan., Results: Between 1998 and 2011, we treated 18 patients relapsed after median 20 months. Nine had relapsed locally, four had dissemination, three single metastases, and two had one synchronous local and metastatic recurrence. Responses to chemotherapy were seen in 32% of cases. The median hospital stay for treatments/complications was 19 days. The 1- and 3-year progression-free survival (PFS) rates were 28 ± 10% and 0%, respectively, for OS, they were 44 ± 12% and 22 ± 10% but no patient was cured. The median PFS after a first relapse was 7 months (range 1-29); the median OS was 7 months (range 4-44). No patients died due to treatment toxicity. Late recurrence (more than 1-2 years after diagnosis) and involvement of single sites were favorable prognostic factors., Conclusions: Without succeeding in patients cure, we ensured them further treatment with short hospital stay thus affording low personal and social costs. The chances of cure may emerge from tailored therapies according to genetic stratification.

To reduce the sequelae from CNS irradiation (RT), 16 children younger than 3 years with medulloblastoma-PNET (13 cases) and ependymoma (3 cases) were treated between 1987-1993 according to different postsurgical chemotherapy (CT) programs. None of these patients presented with metastases. Eleven patients were rendered disease-free by surgery, while 5 had residual tumor. Adjuvant therapy depended on patients' age, postsurgical status and parents' consent to radiotherapy (RT). Nine of the 16 infants remained alive in continuous complete remission from the first neoplasm (median follow-up 7 years). Three of them had been treated with CT alone and 6 with combined CT + RT (posterior fossa 4, whole CNS 2). Seven patients relapsed a median of 13 months after diagnosis, and all 7 of them died of their disease. Despite the omission of RT in 6 of the 16 patients and administration of only focal RT in 8 of the 16, the outcome of this series was satisfactory. Local failure (in 5/7 patients) was the major problem, despite the high dose of RT used in 2 of these 5. In 4 of 6 evaluable children school performance was satisfactory. One child in whom the entire CNS was irradiated developed glioblastoma multiforme 120 months after the first diagnosis of medulloblastoma.