not-yet-known not-yet-known not-yet-known unknown Background: Timely diagnosis is critical in pediatric oncology to optimize treatment outcomes. Diagnostic delays may impact tumor extension and prognosis, necessitating analysis of diagnostic intervals across different neoplasms. Methods: We analyzed data from 749 pediatric patients diagnosed with rhabdomyosarcoma between 1996 and 2016. Diagnostic interval (DI) was defined as days from symptom onset to diagnosis, and treatment interval (TI) from symptom onset to treatment initiation. Factors influencing DI and TI were collected, including patient age, histology, tumor characteristics, and protocol of treatment. Survival outcomes were assessed using Kaplan-Meier analysis. Results: Median DI was 32 days, decreasing insignificantly from 1996-2004 to 2005-2016. Longer DI was associated with metastatic disease (p=0.0021). The proportion of patients diagnosed within one month increased over time, but remained lower for metastatic cases. Median TI was 48 days, unchanged over time. Longer TI correlated with larger tumors (p<0.0001). Adolescents had prolonged DI (>2 months) more frequently. The quantile regression models showed that on univariate analysis DI was associated with age at diagnosis, unfavourable histology and metastatic diaeses, but not confirmed in multivariate Five-year event-free survival (EFS) and overall survival (OS) were 59.7% and 69.3%, respectively. Conclusions: This study evaluated the role of timely diagnosis and treatment initiation in pediatric patients with rhabdomyosarcoma . Our data highlights that DI and TI are crucial in adolescents and often longer in metastatic patients. Future efforts should focus on streamlining access to diagnostic facilities and improving processes to ensure timely interventions, especially for patients presenting with more advanced disease.

In the era of big data, young patients are overwhelmed by artificial intelligence-based tools, such as chatbots. Five clinical experts were asked to evaluate the performance of four of the most currently used chatbots at providing information on a rare cancer affecting young people, such as rhabdomyosarcoma. In general, despite their high performance at giving general information about the disease, these chatbots were considered by the experts inappropriate at providing suggestion on cancer treatments and specialized centers, also lacking of "sensitivity". Efforts are envisioned by the pediatric oncology community to improve the quality of data used to train these tools.

Background and Aims. Since the war in Ukraine which began on February 2022, many pediatric oncology centers have welcomed patients from Ukraine. To understand the needs of patients and families arriving at our hospital, an anonymous questionnaire investigated the families’ backgrounds, feelings and impressions about hospitality and care. Methods Twenty items investigated how patients had reached Italy, from whom they received help (logistically/economically); emotions regarding their statusas war refugees; knowledge, expectations, and opinions about Italy and Italians; the quality of medical care received and relationships with the healthcare staff; suggestions to improve assistance. Results Questionnaires were completed by 19/32 patient/parents in the time interval May-November 2022 in two different pediatric-oncology centers in the north of Italy. Most families had reached Italy (58%) and received medical care (95%) thanks to the help of Charities and the Italian Public Health Care System. Many of them (69%) declared themselves to be satisfied by the assistance. The Italian population appeared friendly (95%) and generous (58%). The improvement of their stay correlated with the positive outcome of their children (15%), the presence of the whole family (15%), the end of the war (10%), and the overcoming of language barriers (10%) Conclusions. Taking care of children from a different country suffering the traumatic experience of fleeing their country in addition to the equally traumatic condition of cancer disease, is a huge task. Our questionnaire aimed at obtaining a better understanding of families’ conditions, not at bridging the relational gap due to different culture and experiences.

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: Irinotecan is a drug active against pediatric sarcomas with a toxicity profile that theoretically allows for its association with more myelotoxic drugs. We examined the feasibility of a dose-density strategy integrating irinotecan in standard chemotherapy regimens for patients with high-risk sarcomas. METHODS: Between November 2013 and January 2020, 23 patients < 21 years old with metastatic (11 children) or recurrent (12 children) sarcomas were treated with 9 IrIVA/IrVAC cycles. All newly-diagnosed patients received IrIVA (ifosfamide 3g/m2 on days 1 and 2, vincristine 1.5 mg/m2 on day 1, actinomycin D 1.5 mg/m2 on day 1, irinotecan 20 mg/m2 for 5 consecutive days starting on day 8). Two relapsed patients received IrIVA and 10 IrVAC (cyclophosphamide 1.5 g/m2 on day 1 instead of ifosfamide). Feasibility was assessed in terms of toxicity and time to complete the treatment. RESULTS: 17 rhabdomyosarcomas, 4 Ewing sarcomas, 2 desmoplastic round cell tumors received a total of 181 cycles (range 2-10). Grade 4 neutropenia occurred in 62.4% of the cycles. 13 patients had febrile neutropenia. Diarrhea occurred in 14 cycles. The median time to complete the treatment was 195 days (range 170-231), 83.4% of cycles were administered on time or with a delay <1 week. With a median follow-up of 2.6 years (range 0.2-5.0), 12 patients are alive, 9 complete remissions, 3 with the disease. Conclusions: A dose density strategy combining irinotecan with standard chemotherapy is feasible. This approach will be investigated in the next trial coordinated by the European pediatric Soft tissue sarcoma Study Group.

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.

We analyzed the nationwide hospital discharge records of adolescent inpatients in Italy to map where adolescents with melanoma are treated and identify expert centers. We identified 137 hospitals pointing out a dispersion of these patients that must be addressed to ensure to all adolescents with melanoma adequate treatment and enrolment in clinical studies.