Introduction. The H3K27M-mutant diffuse midline glioma (DMG) was first included in the WHO Classification of CNS (central nervous system) tumors in 2016, and confirmed in its fifth edition. The biological behavior and dismal prognosis of this tumor resemble DIPG (diffuse intrinsic pontine gliomas). Homogeneously-treated series are rarely reported. Methods. From 2016 onwards, we treated patients with DMG with radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus re-irradiation at relapse, as already done for DIPG (DOI10.1007/s11060-014-1428-z). Results. We treated nine patients, seven females, median age at diagnosis of 13 years-old. Tumor sites were: thalamic in five cases, pontocerebellar in two, pineal in one, and paratrigonal with nodular/leptomeningeal dissemination in one. Three patients were biopsied, and six had partial tumor resections. Central review of the pathologists’ diagnoses was performed. The median time to local progression was 12.7 months, and the median overall survival was 17.8 months. Six patients died of tumor progression, one of cerebral bleeding whose tumor was progressing. Two were alive, one in continuous remission, the other after a relapse, at 38.6 and 46.3 months after diagnosis, respectively. Progression-free survival was 33.3% at one year. Overall survival was 88.9%, 33.3% and 22.2% at 1, 2 and 3 years, respectively. Conclusions. This is one of only a handful of reports on homogeneously-treated series. The results obtained are comparable with those seen in patients with DIPG. Given the phenotypically- and molecularly-defined setting of DMG and severe outcome in this orphan population, they should be treated and included in registries and protocols of DIPG.

During SARS-CoV-2 pandemic, oncologists manage patients at higher risk of having a severe course of this infection. This raises new questions about their correct management, as well as the difficulty of distinguishing tumor/treatments complications from those related to Coronavirus disease 2019 (COVID-19). We report a case of an 11 year-old boy undergoing treatment for T cell lymphoblastic lymphoma who experienced a prolonged SARS-CoV-2 infection. Oncological therapy was continued without significant changes compared to the initially planned treatment. No relevant complications occurred. COVID-19 convalescent plasma (CP) was administered, resulting in a positive antibody titer after 24 days.

Background: Postoperative hypocalcemia is a frequent complication after thyroid surgery. Hypoparathyroidism may develop as transient (TtHP), with normalization within six months from surgery, or permanent (PtHP) if the patient requires replacement therapy. The present study analyzes rates and factors associated with the development of TtHP or PtHP following thyroid surgery in a pediatric population. Procedure: A retrospective multicenter study analyzing 363 patients was carried out. We recorded gender, age, tumor size, type of surgery, lymph node dissection, histology. Calcium levels were acquired daily for 72 hours after discharge. Subsequent sample collection was customized on the patient’s hypocalcemia severity. Results: We analyzed 363 patients aged ≤18 years (mean age 14.2 years) who underwent thyroid surgery clustered into age groups (≤15 or >15). Patients mean follow-up was 5.8 years (1-11yrs). At histology 310 (85%) were papillary carcinoma, 32 (9%) were follicular carcinoma, 6 (2%) presented diffuse sclerosing variant of papillary thyroid carcinoma whilst 15 (4%) had familial medullary carcinoma. TtHP developed in 36 (9,9%), PtHP in 20 (5.5%) cases. TtHP was more frequent in younger patients (p=0,009). Both PtHP and TtHP were increased in case of larger tumors (≥2 cm) (p=0,001). All TtHP and PtHP were in TT group. PtHP rate was increased if lymph node dissection was carried out (p<0.001). Conclusions: The risk of hypoparathyroidism is related to younger age, tumor size, TT and lymph node dissection therefore surgeons should tailor surgery as much as possible to avert such complication.

Introduction. Mutations of the APC (adenomatous polyposis coli) gene correlate mainly with familial adenomatous polyposis (FAP), but can occasionally be pathogenic for medulloblastoma (MBL) WNT subtype as well, the course of which has only recently been described. Methods. We retrospectively retrieved all patients with documented germline APC mutations and a centrally-reviewed diagnosis of MBL to examine the outcome of their MBL, late effects of its treatment, and further oncological events. Results. Between 2007-2016 we diagnosed and treated 6 patients, all with a pathogenic APC variant mutation, who all had MBL, classic histotype. None had metastatic disease. All patients were in complete remission a median 65 months after treatment with craniospinal irradiation at 23.4 Gy, plus a boost on the posterior fossa/tumor bed up to 54 Gy, followed by cisplatin/carboplatin, lomustine and vincristine for a maximum of 8 courses. Five of 6 diagnostic revised MRI were suggestive of the WNT molecular subgroup typical aspects. Four of 6 patients had a positive family history of FAP, while gastrointestinal symptoms prompted its identification in the other 2 cases. Four patients had developed other tumors (desmoid, MELTUMP, melanoma, pancreatoblastoma, thyroid Tir3) from 5 to 7 years after MBL. Discussion. Our data confirm a good prognosis for patients with MBL associated with FAP. Patients’ secondary tumors may or may not be related to their syndrome or treatment, but warrant adequate attention when planning shared guidelines for these patients.

Background: Children and adolescents with cancer are at risk of a poor health-related quality of life. Exercise interventions to enhance movement may be a valid strategy for managing some symptoms, including fatigue. Methods: Forty-four consecutive patients (20 females; aged 5-21 years old, median 15.5 years), without any contraindications significantly limiting their movements were invited to join an in-hospital 6-week supervised exercise program, and asked afterwards to complete the PedsQL-4.0 quality of life Generic Core Scales and the PedsQL Multidimensional Fatigue Scale. The program consisted of personalized workout sessions of aerobic, resistance and flexibility exercises. The results obtained on the scales were compared between patients who engaged in the exercise program (GYM group, n=21) and those who did not (No-GYM, n=23), with the aim of examining the different dimensions of health-related quality of life (physical, emotional, cognitive, social) and fatigue (general, sleep/rest, cognitive) comparing the two groups. Results: For 43 of 44 patients, being diagnosed with cancer initially prompted a drop-out from previous physical exercise or sports routines despite no contraindications to their continuation. After 6 weeks, the scores for patients in the GYM group showed a statistically significant better perceived emotional functioning, and a possible indication of improved social functioning compared with the No-GYM group. Conclusion: These findings suggest that to exercise improves the satisfaction of children and adolescents with cancer with their physical, mental and social functioning. It is worth further investigating the value of systematically including exercise workouts in their routine cancer practices.