Publications by authors named "Elisabetta Schiavello"

Background: Craniofacial osteosarcoma (CFOS) is a rare tumor, typically affecting patients over 60, with a low incidence of metastasis at diagnosis as well as at relapse.

Patients And Methods: This study analyzed patients treated for high-grade CFOS at Fondazione IRCCS Istituto Nazionale dei Tumori between 1991 and 2021, focusing on histological characteristics, systemic and local treatment, and outcome.

Results: Eleven patients (7F/4M) were included, with a median age of 15 years.

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Background: Central nervous system (CNS) tumors are the leading cause of cancer-related deaths in children aged 0-14 years. Despite significant efforts, targeted therapies based on identified pathways have not improved survival rates. Research has shown that the gut microbiota (GM) can influence brain tumor cell proliferation, suggesting that the microbiota-gut-brain axis plays a role in CNS cancer.

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Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive sarcoma often associated with neurofibromatosis type 1, whose clinical management remains complex and challenging. Few publications exist on pediatric MPNST, and limited data are available on the best treatment approach, in particular regarding local therapy.

Methods: This retrospective analysis concerned 45 patients less than 18 years old with MPNST, treated at a referral center for pediatric sarcomas from 1983 to 2023.

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Background/objectives: Astroblastoma is a rare glial neoplasm more frequent in young female patients, with unclear clinical behaviors and outcomes. The diagnostic molecular alteration is a rearrangement of the Meningioma 1 () gene. MicroRNAs (miRNAs) are important gene expression regulators with strong implications in biological processes.

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Purpose: Tropomyosin receptor kinase (TRK) fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven central nervous system tumors.

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Article Synopsis
  • - The study focused on treating high-risk M+ medulloblastoma patients through a specific chemotherapy and radiation protocol, including multiple high-dose drugs and tailored dosages based on age and response to treatment.
  • - Out of 89 enrolled patients, the median age was 8.8 years, and the study reported 5-year overall survival at 75.9% and 15-year event-free survival at 66.5%, with some negative outcomes linked to disease progression during treatment.
  • - Subgroup analysis indicated that patients with Sonic Hedgehog (SHH) tumors and those with certain genetic mutations had significantly worse event-free survival rates compared to other subgroups in the study.
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This paper describes the complexity of the clinical management of foreign minors suffering from cancer, through the clinical experience of an Italian referral center. The study includes 50 patients less than 18 years (22% of the patients admitted to the unit in 2023), 32 foreigners who were Italian resident and 18 who had come to Italy specifically to receive cancer treatment. Patients who migrate for healthcare reasons often arrive at the referral center with advanced disease or relapse.

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Background And Aims: Since the beginning of the war in Ukraine on February 24, 2022, many pediatric oncology centers welcomed evacuated patients. To better understanding the needs of patients and families arriving at two Lombardy hospitals in the period March to November 2022, an anonymous questionnaire investigated the families' backgrounds, feelings, and impressions about hospitality and care.

Methods: Twenty questions investigated how patients had reached Italy, from whom they had received help (logistically/financially); the emotions regarding their status as war refugees; the knowledge, expectations, and opinions about Italy and Italians; the quality of medical care received and the relationships with the healthcare staff; lastly, suggestions to improve assistance.

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Background: The term gliomatosis cerebri (GC), a radiology-defined highly infiltrating diffuse glioma, has been abandoned since molecular GC-associated features could not be established.

Methods: We conducted a multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical and (epi-)genetic characterization.

Results: Median overall survival (OS) was 15.

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Article Synopsis
  • The 2021 fifth edition of the WHO Classification of Tumors of the CNS introduced updated naming and grading systems, focusing on integrated diagnoses and detailed reports for better understanding of tumors.
  • This edition put a stronger emphasis on molecular diagnostics alongside traditional methods, introducing new tumor types based on advanced techniques like DNA methylome profiling.
  • The review highlights significant changes in classifying pediatric CNS tumors, detailing relevant molecular alterations that are crucial for diagnosis, prognosis, and treatment strategies important for both patients and oncologists.
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Article Synopsis
  • Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are tough tumors that don’t have the best treatment options for some patients, especially if they can’t be surgically removed at first.
  • A study looked at 71 patients under 21 who were treated for NRSTS from 1990 to 2021, finding that many had large, dangerous tumors and most received chemotherapy before surgery.
  • Results showed that while the survival rates were not great, patients who responded well to chemotherapy and had successful surgeries had a better chance of living longer, highlighting the need for better treatment plans.
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Article Synopsis
  • PATZ1-rearranged CNS tumors may or may not be a distinct tumor type, as researchers investigated a pediatric series of 7 cases using various advanced analysis techniques to understand their characteristics.* -
  • MRI results showed these tumors mainly occur in the occipital lobe and have two major types: one resembling glial cells (NET) and another showing spindle cell features (SM), both identified via histologic analysis.* -
  • Although both groups share molecular similarities, gene expression analysis revealed they form two distinct subgroups, with those in the SM group showing important genes related to tumor progression; most patients are disease-free following treatment.*
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Introduction: The severe acute respiratory syndrome coronavirus-2 pandemic significantly affected clinical practice, also in pediatric oncology units. Cancer patients needed to be treated with an adequate dose density despite the SARS-CoV-2 infection, balancing risks of developing severe COVID-19 disease.

Areas Covered: Although the pandemic spread worldwide, the prevalence of affected children was low.

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Background: First-line therapies for medulloblastoma(MBL) are obtaining higher survival-rates while decreasing late-effects, but treatment at relapse is not standardized. We report here the experience with MBL re-irradiation(re-RT), its timing and outcome in different clinical settings and tumor groups.

Methods: Patient's staging/treatment at diagnosis, histotypes/molecular subgroups, relapse site/s, re-treatments outcome are reported.

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Background: The 2022 World Health Organization (WHO) classification redefines the concept of gray zone lymphoma (GZL), restricting it in practice to cases of mediastinal/thymic origin (mediastinal gray zone lymphoma, MGZL) with overlapping features between primary mediastinal B-cell lymphoma (PMBCL) and classical Hodgkin lymphoma (CHL). Cases with histological characteristics of GZL but occurring without mediastinal involvement are better classified as diffuse large B-cell lymphoma, not otherwise specified (DLBCL NOS), with few exceptions.

Procedure: We collected clinical and pathological data about all Italian pediatric patients diagnosed with GZL over a 20-year period.

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Objectives: Although transfusion support is commonly used in oncological palliative care, there is still a paucity of literature. We examined the transfusion support provided in the terminal stage of the disease and compared the approach at a pediatric oncology unit and a pediatric hospice.

Case Description: This case series analyzed patients treated at the Fondazione IRCCS Istituto Nazionale dei Tumori di Milano (INT)'s pediatric oncology unit who died between January 2018 and April 2022.

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Each year approximately 35,000 children and adolescents are diagnosed with cancer in Europe. Five-year survival rates have improved and now reach 80% in most European countries, thanks to a combination of chemotherapy, radiotherapy, and surgery. To date, there are more than 44,000 Italians still living several years after being diagnosed with cancer in developmental age.

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Background: Methotrexate is renally excreted. HDMTX (high dose-methotrexate)-induced acute kidney injury (AKI) is a non-oliguric decrease in glomerular filtration rate (GFR) heralded by an acute rise in serum creatinine. Moreover, AKI is also a frequent complication of COVID-19.

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Hearing loss (ototoxicity) is a major adverse effect of cisplatin and carboplatin chemotherapy. The aim of this study is to identify novel genetic variants that play a role in platinum-induced ototoxicity. Therefore, a genome-wide association study was performed in the Genetics of Childhood Cancer Treatment (GO-CAT) cohort (n = 261) and the United Kingdom Molecular Genetics of Adverse Drug Reactions in Children Study (United Kingdom MAGIC) cohort (n = 248).

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Objectives: Cancer remains the leading cause of mortality by disease in childhood in high-income countries. For terminally ill children, care focuses on quality of life, and patient management fundamentally affects grieving families. This paper describes our experience of palliative sedation (PS) for children with refractory symptoms caused by solid tumours, focusing on the drugs involved.

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Background: Patients with rhabdomyosarcoma (RMS) whose disease relapses have little chance of being cured, so front-line treatments are usually followed up with surveillance imaging in an effort to detect any recurrences as early as possible, and thereby improve post-relapse outcomes. The real benefit of such routine surveillance imaging in RMS remains to be demonstrated, however. This retrospective, single-center study examines how well surveillance imaging identifies recurrent tumors and its impact on post-relapse survival.

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Background: The risk of survivors developing a secondary bone sarcoma after being treated for pediatric cancers is well established. The aim of this study was to examine the clinical characteristics and outcomes of patients with secondary osteosarcoma (SOS).

Methods: The study concerns survivors of childhood and adolescence primary neoplasms (PN) treated with chemotherapy, with or without radiotherapy and surgery, subsequently diagnosed with SOS.

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