Atypical Neuroblastoma With Absent Urinary Catecholamine Excretion and ImIBG Avidity Are of Favorable Outcome: A Retrospective French Single-Center Study.

Background: In neuroblastoma (NB), urinary catecholamine excretion and ImIBG avidity-depending on tumor enzymatic activity and norepinephrine transporter expression, respectively-are diagnostic standards. The prognostic impact of atypical NB, without urinary catecholamine excretion and/or ImIBG avidity, remains to be determined. We sought to determine the frequency and prognosis of atypical NB and investigate the significance of catecholamine profiles and ImIBG avidity at diagnosis.

Regional lymph node invasion in pediatric non-rhabdomyosarcoma soft tissue sarcoma: an international cohort study from the International Soft Tissue Sarcoma Consortium.

Background: In pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), the frequency and prognostic impact of regional lymph node involvement (N1) are not clearly defined and may vary according to histological type. We therefore to analyze the rate of N1 at diagnosis, the pattern of nodal relapse, and the prognostic impact of N1 in pediatric patients with NRSTS.

Methods: Data were collected and analyzed through the International Soft Tissue SaRcoma ConsorTium (INSTRuCT).

Early radiologic tumour volume response in non-metastatic rhabdomyosarcoma is not predictive for survival.

Background: There is an urgent need for early radiological markers predicting survival in paediatric rhabdomyosarcoma patients.

Objective: To analyse the predictive value of early radiological tumour volume response to chemotherapy in non-metastatic rhabdomyosarcoma patients.

Methods: The European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study prospectively included non-metastatic rhabdomyosarcoma patients.

Surgical Lymph Node Staging in Extremity Rhabdomyosarcoma: The EpSSG RMS 2005 Trial Experience.

Background: The European pediatric soft tissue Sarcoma Study Group (EpSSG) RMS 2005 study recommends a lymph node biopsy for extremity rhabdomyosarcoma (RMS). The aim of our study was to analyze the results of the lymph node sampling strategies used, such as sentinel node biopsy (SNB) and nodal sampling (NS), and compare the outcome of patients undergoing different nodal staging techniques.

Methods: All non-metastatic (M0) patients registered in the EpSSG RMS 2005 study with an RMS of the extremity, presenting between 2005 and 2016, were included for analysis of the lymph node sampling techniques used.

Desmoid-Type Fibromatosis Tumors in Children After First-Line Failure: Clinical Aspects and Approaches for Subsequent Therapeutic Lines.

Background: Desmoid-type fibromatosis (DTF) is a rare intermediate malignancy with high local aggressiveness and recurrence in children after first-line methotrexate-vinblastine (Mtx-Vbl) regimen. The objective is to describe refractory DTF to standardize second-line therapy.

Methods: This national multicenter retrospective study included patients (<25 years) with progressive/refractory DTF after Mtx-Vbl first-line.

Acquisition matters - how do scan parameters affect apparent diffusion coefficient estimates in pediatric rhabdomyosarcoma.

Background: The apparent diffusion coefficient (ADC) derived from diffusion-weighted imaging (DWI) is a potential biomarker for treatment response in pediatric rhabdomyosarcoma. Due to its rarity, investigations into this marker require multicenter approaches, which can result in variability in acquisition parameters.

Objective: To evaluate the impact of different acquisition parameters on ADC estimates in a multicenter dataset of rhabdomyosarcoma patients.

Appendiceal neuroendocrine tumors in children and adolescents: The European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) diagnostic and therapeutic recommendations.

Neuroendocrine tumors of the appendix, formerly known as carcinoid tumors, represent a rare entity. They are slow-growing tumors, characterized by an indolent clinical course. In pediatric patients, the 5-year overall survival is estimated to be 100% and the event-free survival slightly less than 100%, with only one reported case of local relapse to date.

Bronchial Carcinoid Tumors in Children and Adolescents.

Purpose: Carcinoid neoplasms, even rare, are the most common pulmonary tumors during childhood. They are classically divided into typical and atypical tumors according to pathology pattern. Cornerstone therapy is to obtain a complete tumor resection.

Impact of maintenance treatment on male gonadal function in patients treated for localized rhabdomyosarcoma in RMS2005 trial in France.

Background: Maintenance treatment with vinorelbine and oral cyclophosphamide (oral-CPM) improves outcome of nonmetastatic high-risk (HR) and very-high risk (VHR) rhabdomyosarcoma (RMS) patients. However, gonadal toxicity of maintenance was not yet investigated.

Methods: The authors focused their analysis on male gonadal toxicity in HR/VHR groups of RMS2005 trial, in France.

Maintenance therapy for pediatric sarcoma: full throttle ahead?

Purpose Of Review: Maintenance therapy (MT), particularly antiangiogenic approaches such metronomic chemotherapy (MC), correspond to the continuous administration of low-dose anticancer agents in a context of minimal residual disease. While widely used in pediatric acute lymphoblastic leukemia for decades, MT has recently shown promise in solid tumors. Additionally, antivascular endothelial growth factor (VEGF) tyrosine kinase inhibitor (TKI) are increasingly explored in pediatric sarcomas.

Pediatric Head and Neck Germ Cell Tumors: Current Management and Risk of Malignant Transformation.

Background And Aims: Head and neck germ cell tumors (HN-GCTs), excluding the central nervous system, are rare and frequently contain mature or immature teratoma (MIT) compounds. The aims of this study were to analyze the risk of malignant transformation after MIT HN-GCTs, to describe treatments and sequelae, and to propose recommendations for the follow up of these patients.

Methods: National multicentric retrospective study of all patients aged from birth to 17 years, treated in France between 2000 and 2021 for a HN-GCT of all histotypes.

Epidemiology, incidence, and outcome of childhood cancers in the Afro-descendant population of the French West Indies and French Guiana: a population-based study.

Background: The epidemiology of childhood cancer in Afro-descendant (AD) populations is poorly described. We performed a descriptive study of the distribution, incidence, and survival of children with cancer in the French West Indies (FWI) and French Guiana (FG).

Methods: We included all patients aged 0-17 diagnosed with cancer or benign intracranial tumor between January 2011 and December 2021 and living in the FWI/FG area at time of diagnosis.

Maintenance Chemotherapy in Patients With High-Risk Rhabdomyosarcoma: Long-Term Survival Analysis of the European Soft Tissue Sarcoma Study Group RMS 2005 Trial.

The European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) RMS 2005 trial evaluated maintenance chemotherapy in high-risk rhabdomyosarcoma (RMS). Patients were randomly assigned to either discontinue treatment (standard arm) or receive six 28-day cycles of vinorelbine (25 mg/m) once per day on days 1, 8, and 15, plus once daily low-dose cyclophosphamide (25 mg/m; experimental arm). Initial results showed improved overall survival (OS), but disease-free survival (DFS) improvement was not statistically significant.

Parotid Keratocystoma: Fine Needle Aspiration Cytomorphology With Ancillary Testing Results.

Keratocystoma is a rare benign salivary gland tumor described in 1999. Only 14 cases have been reported in the histopathological literature, with three of them having had a preoperative fine needle aspiration (FNA) performed. All cytological diagnoses were not specific.

NUT carcinoma in children and adolescents: An analysis of the European Cooperative Study Group on pediatric rare tumors (EXPeRT).

Background And Aims: NUT carcinoma (NC) is a sporadic, highly aggressive tumor that primarily affects children, adolescents, and young adults and is characterized by the presence of somatic NUTM1 rearrangements. This analysis by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) aims to fill the knowledge gap regarding the clinical characteristics of children with NC.

Methods: A retrospective case series of NC-patients aged 0-18 years treated between 2011 and 2023 was conducted using the EXPeRT database.

Cervical Lymph Node Invasion in Pediatric Salivary Gland Malignancies: Clinical Overview and Therapeutic Implications.

Background And Aims: Pediatric salivary gland malignancies (SGM) present challenges in managing cervical nodes. We aimed to characterize lymph node invasion to inform decisions regarding the need of systematic wide lymph node dissection (WLND).

Methods: International retrospective study, conducted across seven large French and American pediatric centers, including pediatric patients (0-18 years) diagnosed with SGM from 2000 to 2020.

Elective Discontinuation of Larotrectinib in Pediatric Patients With TRK Fusion Sarcomas and Related Mesenchymal Tumors.

Larotrectinib is a highly selective tropomyosin receptor kinase (TRK) inhibitor with efficacy in children with TRK fusion tumors. We evaluated patient outcomes after elective discontinuation of larotrectinib in the absence of disease progression in a protocol-defined wait-and-see subset analysis of eligible patients where treatment resumption with larotrectinib was allowed if disease progressed. We also assessed the safety and efficacy of larotrectinib in all pediatric patients with sarcoma.

Lymph Node Staging and Treatment in Pediatric Patients With Soft Tissue Sarcomas: A Consensus Opinion From the Children's Oncology Group, European paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

Accurate staging of nodal involvement in pediatric sarcoma patients is important to determine correct systemic and local therapy, with the goal to reduce subsequent recurrences. However, differences in lymph node staging strategies, definitions, and treatment protocols between the Children's Oncology Group (COG), European paediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS) complicate comparisons. In this article, we aim to establish internationally recognized recommendations for lymph node assessment and treatment of children and adolescents diagnosed with rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) according to the Consensus Conference Standard Operating Procedure methodology.

Pulmonary Consequences of Surgical Treatment in Children's Primary Lung Tumors: A National Retrospective Study.

Background And Aims: Primary lung tumors (PLTs) in children are rare, and surgery remains the key to ensure remission. Here we describe the PLTs clinical characteristics, their management, and the pulmonary outcome following surgery.

Methods: We carried out a French national cohort of pediatric PLTs from 2013 to 2023 from the FRACTURE rare pediatric tumors national database.

Prognostic role of bone erosion in orbital RMS: a report from the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG).

Background: Orbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment.