Massimo Provenzi

Treatment Adaptation to Surgical Timing in Pediatric Hepatoblastoma: A 19‐Year Single‐Center Experience With Resection and Liver Transplantation

Claudia Pellegrinelli, Laura Cavalleri, Michela Bravi, Paolo Marra et autres

ABSTRACT Background The management of hepatoblastoma (HB) relies on chemotherapy combined with surgery, either liver resection (LR) or liver transplantation (LT), with reported overall survival (OS) of 80%, cardiac toxicity of 6%, nephrotoxicity of 21%, ototoxicity of 32%–46%. In clinical practice, deviations …

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Pharmacokinetics of Pegaspargase with a Limited Sampling Strategy for Asparaginase Activity Monitoring in Children with Acute Lymphoblastic Leukemia

Cristina Matteo, Antonella Colombini, Marta Cancelliere, Tommaso Ceruti et autres

Background: Asparaginase (ASPase) plays an important role in the therapy of acute lymphoblastic leukemia (ALL). Serum ASPase activity (SAA) can be modified and even abolished by host immune responses; therefore, current treatment guidelines recommend to monitor SAA during treatment administration. The SAA …

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“Amici per la pelle”: un caso di leishmaniosi

Marialetizia Fantasia, Laura Cavalleri, Federica Bruni, Massimo Provenzi

Leishmaniasis is an infectious disease caused by protozoa of the Leishmania genus, parasites of humans and several domestic and wild animals, and transmitted via vectors. The authors describe the case of an 18-month-old girl, presenting to the Emergency Room for persistent fever, …

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Case Report: Early diagnosis of LAD-III in newborn with persistent leukocytosis and hemangioma-like lesion of the urinary bladder

Laura Lorioli, Francesca Favini, Eleonora Severi, Marco Salvi et autres

Leukocyte Adhesion Defects (LADs) are a group of rare autosomal recessive immune disorders characterized by constitutional defects in the process of leukocyte migration. Among these, LAD-III is the rarest, with only a few cases documented in scientific literature. It is caused by …

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SARS-CoV-2 Infection in the Pediatric Oncology Population: The Definitive Comprehensive Report of the Infectious Diseases Working Group of AIEOP

Daniele Zama, Andrea Zanaroli, Agnese Corbelli, Andrea Lo Vecchio et autres

OBJECTIVE: The objective of this study was to assess the clinical impact and outcome of the SARS-CoV-2 infection on children with cancer or those who received a hematopoietic stem cell transplantation. METHODS: AIEOP (Italian Association of Pediatric Hematology and Oncology) performed a …

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Therapeutic Targeting of ALK in Neuroblastoma: Experience of Italian Precision Medicine in Pediatric Oncology

Fabio Pastorino, Mario Capasso, Chiara Brignole, Vito Alessandro Lasorsa et autres

Neuroblastoma (NB) is the most common extracranial solid tumor in childhood. Patients with relapsed/refractory disease have a poor prognosis, and additional therapeutic options are needed. Mutations and amplifications in the ALK (Anaplastic Lymphoma Kinase) gene constitute a key target for treatment. Our …

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Phosphoproteomic Analysis Reveals a Different Proteomic Profile in Pediatric Patients With T-Cell Lymphoblastic Lymphoma or T-Cell Acute Lymphoblastic Leukemia

Giulia Veltri, Federica Lovisa, Giuliana Cortese, Marta Pillon et autres

T-cell lymphoblastic lymphoma (T-LBL) and lymphoblastic leukemia (T-ALL) arise from the transformation of precursor T-cells sharing common morphological and immunophenotypic features. Despite this, T-LBL and T-ALL show different genomic/transcriptomic profiles and whether they represent two distinct disease entities or variant manifestations of …

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Outcome of Children and Adolescents with Recurrent Classical Hodgkin Lymphoma: The Italian Experience

Alberto Garaventa, Stefano Parodi, Giulia Guerrini, Piero Farruggia et autres

The objective of this study was to identify prognostic factors for children and adolescents with relapsed or progressive classical Hodgkin’s lymphoma (cHL) to design salvage therapy tailored to them. We analyzed a homogeneous pediatric population, diagnosed with progressive/relapsed cHL previously enrolled in …

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Bilateral adrenal primary tumor in Stage 4S neuroblastoma: The Italian experience and review of the literature

Shana Montalto, Fiammetta Sertorio, Marta Giorgia Podda, Stefania Sorrentino et autres

Bilateral adrenal neuroblastoma (NB) is rare and is mainly stage 4S. Its incidence, presenting features, management, and outcome have not been fully defined yet. We searched the Italian NB Registry (RINB) for stage 4S NB infants with bilateral adrenal primary tumor to …

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Global characteristics and outcomes of SARS-CoV-2 infection in children and adolescents with cancer (GRCCC): a cohort study

Sheena Mukkada, Nickhill H. Bhakta, Guillermo Luis Chantada, Yi‐Chen Chen et autres

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Malignant sacrococcygeal germ cell tumors in childhood: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience

Paolo D’Angelo, Maria Debora De Pasquale, Francesco Barretta, Maria Carmen Affinita et autres

PURPOSE: To evaluate clinical features at diagnosis, prognostic factors, and outcomes of malignant sacrococcygeal germ cell tumors (SC-GCTs) in patients enrolled in the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) TCG 2004 protocol. PATIENTS AND METHODS: A prospective analysis was conducted on all …

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A novel homozygous disruptive PRF1 variant (K285Sfs*4) causes very early-onset of familial hemophagocytic lymphohystiocytosis type 2

Francesco Saettini, Ilaria Castelli, Massimo Provenzi, Grazia Fazio et autres

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory multisystemic disease. Familial hemophagocytic lymphohistiocytosis (FHL) represents a group of rare autosomal recess...

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