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.

Isolated Fallopian Tube Torsion in Children With Hydrosalpinx: Is Conservative Management an Option?

Background: Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX).

Methods: A retrospective review was conducted.

Reconstruction with antibiotic loaded single-side gore-tex "Tartine" methyl-methacrylate cementoplasty for pediatric chest wall reconstruction: A 10-case series.

Introduction: Chest wall reconstruction in children after large resection of tumors may be performed with rigid or soft materials. Cementoplasty is commonly used with the "Sandwich" method i.e.

Oncological and endocrinological outcomes for children and adolescents with testicular and ovarian sex cord-stromal tumors. Results of the TGM13 National Registry.

Rationale: Sex cord-stromal tumors (SCST) are hormonally active and rare. The aim was to describe their endocrinological presentation and outcomes.

Method: Patients (< 19 years) registered in the TGM13 registry between 2014 and 2021 for SCST were selected.

Pectus Arcuatum: A Pectus Unlike Any Other.

Background: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers.

Extracranial germ cell tumours in children and adolescents: Results from the French TGM13 protocol.

Background: Chemotherapy for non-seminomatous germ cell tumours (NSGCT) exposes to dose-dependent toxicities. The TGM13-NS protocol (EudraCT 2013-004039-60) aimed to decrease the chemotherapy burden compared to the previous TGM95 protocol while maintaining the 5-year event-free survival (EFS) at 80% or more.

Procedure: Patients less than 19 years of age with disseminated NSGCT were enrolled (May 2014 to May 2019) and stratified into four groups: two intermediate-risk (IR: localised tumour with low tumour markers [TM]) groups treated with VBP (vinblastine-bleomycin-cisplatin): three courses for IR1 (ovarian tumour any age/testis tumour less than or equal to 10 years) and four courses for IR2 (extragonadal tumour 10 years or less) groups, and two high-risk (HR: metastatic and/or high TM) groups treated with etoposide-cisplatin and either ifosfamide (VIP) or bleomycin (BEP): three courses for HR1 (ovarian tumour any age/testis tumour less than or equal to 10 years and low TM/testis tumour more than 10 years and very low TM) groups and four courses for HR2 (remainder) groups.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

Introduction: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication.

Lessons from a large nationwide cohort of 350 children with ovarian mature teratoma: A study in favor of ovarian-sparing surgery.

Background: Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%-20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian-sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed.

Predicting the risk of respiratory distress in newborns with congenital pulmonary malformations.

Objectives: Most children with prenatally diagnosed congenital pulmonary malformations (CPMs) are asymptomatic at birth. We aimed to develop a parsimonious prognostic model for predicting the risk of neonatal respiratory distress (NRD) in preterm and term infants with CPM, based on the prenatal attributes of the malformation.

Methods: MALFPULM is a prospective population-based nationally representative cohort including 436 pregnant women.

Excision of Wilms' Tumor With Atrial Extension Under Moderate Hypothermia and Cerebral Perfusion.

Background: Wilms' tumor is the most common pediatric renal tumor. Almost half of all cases have involvement of the inferior vena cava, which must be addressed at the time of surgical excision. Further extension into the right atrium may pose an immediate vital risk and necessitates special operative techniques that employ cardiopulmonary bypass.

Childhood ovarian nonseminomatous germ cell tumors: A highly curable disease with few long-term treatment-related toxicities-Results of the French TGM95 study.

We report survival and late effects analysis of TGM95 study for childhood (≤18 years) ovarian nonseminomatous germ cell tumors (NS-GCT). Patients with localized tumors (FIGO-stage IA) had no adjuvant treatment (low-risk, LR). Patients with advanced-stage received 3-5 VBP (vinblastin-bleomycin-cisplatin) in intermediate-risk group (IR: FIGO-stage IC-II-III and AFP < 15 000 ng/mL) or 4-6 VIP (etoposide-ifosfamide-cisplatin) in high-risk group (HiR: metastatic or AFP ≥ 15 000 ng/mL).

Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations.

Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy.

Testicular transposition in children before scrotal external radiotherapy.

Testicular transposition (TT) before scrotal external radiotherapy (RT) is poorly reported in children with cancer, with only rare case reports published. TT surgical techniques, dosimetric parameters, and testicular functions are retrospectively reported in 12 children, median age 12.8 years, after scrotal RT for sarcomas.

Pure pediatric ovarian immature teratomas: The French experience.

Objective: To describe characteristics and outcome of pediatric ovarian immature teratomas (IT) to better define the place of chemotherapy.

Methods: Children with ovarian IT enrolled in TGM95 and TGM2013 studies were analyzed. Norris grading and International Federation of Gynecology and Obstetrics staging system were used.

Exploring heterogeneity of adrenal cortical tumors in children: The French pediatric rare tumor group (Fracture) experience.

Introduction: Pediatric adrenal cortical tumors are characterized by a wide spectrum of behavior. Questions remain regarding intermediate disease stages with isolated tumor rupture or relapse.

Objectives: To describe clinical characteristics, treatment strategy, and outcome of patients depending on disease stage, tumor rupture, or in case of a refractory tumor, to discuss optimal management.

Neonatal Soft Tissue Sarcoma with YWHAE-NUTM2B Fusion.

Neonatal soft tissues sarcoma is a rare entity that comprises heterogeneous types of tumors. In this article we describe a neonatal case of round-cell sarcoma with an YWHAE-NUTM2B fusion gene. The patient was treated just after birth with neoadjuvant chemotherapy, then surgical resection, but evolution was quickly fatal.

Multicentric evaluation of the adherence to Peristeen® transanal irrigation system in children.

Background: Since 2009 in France, the Peristeen® transanal irrigation (TAI) device has represented an alternative treatment of faecal incontinence (FI).

Objective: The primary objective of this study was to assess the mid-term adherence to TAI in paediatric patients. The secondary objective was to identify factors determining TAI continuation.

Low level myelomeningoceles: do they need prenatal surgery?

Background: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications.

Thoracoscopic Rib Resection in Children.

Objective: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy.

Materials And Methods: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted.

Postnatal Management of Myelomeningocele: Outcome with a Multidisciplinary Team Experience.

Introduction: Myelomeningocele (MMC) is a complex neural tube defect. Few studies report the results of modern postnatal management. The goal of this study was to report the long-term outcome of a multidisciplinary approach of patients with MMC.

[Type 1 neurofibromatosis: Onset of two tumors before the age of 5years].

Neurofibromatosis type 1 (NF1) is a frequent autosomal dominant genetic disorder that predisposes to the development of benign and malignant tumors. Mutation of the NF1 gene affects the RAS-MAPK signaling pathway and leads to a dysfunction in cell proliferation and induces tumor development. Epidemiology of cancer in children with NF1 is very different from the general pediatric population, which requires regular and specific monitoring.