Nephron-Sparing Surgery for Patients With Wilms Tumor, a Surgical Delphi Study Consensus Statement.

Background And Aim: Current pediatric renal tumor treatment protocols allow for nephron-sparing surgery (NSS) for unilateral disease only under strict conditions. Oncological guidelines do not account for surgical feasibility, however, possibly reducing the utilization of NSS. To potentially change this, a definition of surgical feasibility is required.

Nodular Fasciitis With Malignant Morphology and a Fusion: A Case Report (of a 10-Year-old Boy).

Nodular fasciitis is usually a benign lesion genetically characterized by ubiquitin-specific protease 6 () rearrangements. We present a case of a 10-year-old boy with a 1.5-week history of a painless mass on the right chest wall, which was excised.

A Novel Standard for Systematic Reporting of Neuroblastoma Surgery: The International Neuroblastoma Surgical Report Form (INSRF): A Joint Initiative by the Pediatric Oncological Cooperative Groups SIOPEN∗, COG∗∗, and GPOH∗∗∗.

Objective: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes.

Summary Of Background Data: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated.

Rectal Omeprazole in Infants With Gastroesophageal Reflux Disease: A Randomized Pilot Trial.

Background And Objective: Omeprazole is a proton pump inhibitor that is used in acid suppression therapy in infants. Infants cannot swallow the oral tablets or capsules. Since, infants require a non-standard dose of omeprazole, the granules or tablets are often crushed or suspended in water or sodium bicarbonate, which may destroy the enteric coating.

Intra- and inter-fraction uncertainties during IGRT for Wilms' tumor.

Background And Purpose: To assess intra- and inter-fraction motion uncertainties, due to displacements of the tumor bed (TB) and organs at risk (OAR), as well as intra- and inter-fraction patient set-up uncertainties, due to positioning variations, during image-guided radiation therapy (IGRT) in children with Wilms' tumor.

Material And Methods: Four-dimensional computed tomography (4D-CT) and daily pre- and post-treatment cone-beam CT (CBCT)-scans of 15 patients (average 4, range 1-8 years) undergoing flank irradiation after nephrectomy were analyzed. TB (marked by four surgical clips) and OAR motion uncertainties were quantified by displacements of the center of mass in all orthogonal directions.

Apparent diffusion coefficient as it relates to histopathology findings in post-chemotherapy nephroblastoma: a feasibility study.

Background: Nephroblastomas represent a group of heterogeneous tumours with variable proportions of distinct histopathological components.

Objective: The purpose of this study was to investigate whether direct comparison of apparent diffusion coefficient (ADC) measurements with post-resection histopathology subtypes is feasible and whether ADC metrics are related to histopathological components.

Materials And Methods: Twenty-three children were eligible for inclusion in this retrospective study.

A Girl with Disseminated Thoracic Echinococcosis.

We present an unusual case of extensively disseminated pulmonary and thoracic echinococcosis in a 10-year-old girl. Thoracic echinococcosis should be considered in any child from an endemic country with cystic lesions on chest imaging. Confirmation of the diagnosis can be difficult and is important before surgery to reduce the risk of further dissemination and anaphylaxis.

Angular malalignment as cause of limitation of forearm rotation: an analysis of prospectively collected data of both-bone forearm fractures in children.

Introduction: Although limitation of pronation/supination following both-bone forearm fractures in children is often attributed to an angular malunion, no clinical study has compared pronation/supination and angular malalignment of the same child by analysis of prospectively collected clinical data.

Aim: The purpose of this trial is to explore whether limitation of pronation/supination can be predicted by the degree of angular malalignment in children who sustained a both-bone forearm fracture.

Methods: In four Dutch hospitals, children aged ≤16 years with a both-bone forearm fracture were prospectively followed up consecutive children for 6-9 months.

Which factors affect limitation of pronation/supination after forearm fractures in children? A prospective multicentre study.

Introduction: Both-bone forearm fractures in children frequently result in a limitation of pronation/supination, which hinders daily activities. The purpose of this prospective multicentre study was to investigate which clinical factors are related to the limitation of pronation/supination in children with a both-bone forearm fracture.

Methods: In four Dutch hospitals, consecutive children (<16 years) who sustained a both-bone forearm fracture were included.

Conversion to below-elbow cast after 3 weeks is safe for diaphyseal both-bone forearm fractures in children.

Background: It is unclear whether it is safe to convert above-elbow cast (AEC) to below-elbow cast (BEC) in a child who has sustained a displaced diaphyseal both-bone forearm fracture that is stable after reduction. In this multicenter study, we wanted to answer the question: does early conversion to BEC cause similar forearm rotation to that after treatment with AEC alone?

Children And Methods: Children were randomly allocated to 6 weeks of AEC, or 3 weeks of AEC followed by 3 weeks of BEC. The primary outcome was limitation of pronation/supination after 6 months.

Early conversion to below-elbow cast for non-reduced diaphyseal both-bone forearm fractures in children is safe: preliminary results of a multicentre randomised controlled trial.

Introduction: This multicentre randomised controlled trial was designed to explore whether 6 weeks above-elbow cast (AEC) or 3 weeks AEC followed by 3 weeks below-elbow cast (BEC) cause similar limitation of pronation and supination in non-reduced diaphyseal both-bone forearm fractures in children.

Materials And Methods: Children were randomly allocated to 6 weeks AEC or to 3 weeks AEC followed by 3 weeks BEC. The primary outcome was limitation of pronation and supination after 6 months.

Single-bone intramedullary fixation of unstable both-bone diaphyseal forearm fractures in children leads to increased re-displacement: a multicentre randomised controlled trial.

Introduction: Both-bone diaphyseal forearm fractures in children can be stabilised without cast by a flexible intramedullary nail in both the radius and the ulna. Adequate results with single-bone fixation combined with a complementary cast are also reported. However, because those results are based on a selection of children, this trial investigates whether single-bone intramedullary fixation, compared with both-bone intramedullary fixation, results in similar pronation and supination in children with an unstable diaphyseal both-bone forearm fracture.

Re-displacement of stable distal both-bone forearm fractures in children: a randomised controlled multicentre trial.

Introduction: Displaced metaphyseal both-bone fractures of the distal forearm are generally reduced and stabilised by an above-elbow cast (AEC) with or without additional pinning. The purpose of this study was to find out if re-displacement of a reduced stable metaphyseal both-bone fracture of the distal forearm in a child could be prevented by stabilisation with Kirschner wires.

Methods: Consecutive children aged <16 years with a displaced metaphyseal both-bone fracture of the distal forearm (n = 128) that was stable after reduction were randomised to AEC with or without percutaneous fixation with Kirschner wires.

Below-elbow cast for metaphyseal both-bone fractures of the distal forearm in children: a randomised multicentre study.

Introduction: Minimally displaced metaphyseal both-bone fractures of the distal forearm in children are often treated with an above-elbow cast (AEC). Treatment with a below-elbow cast (BEC) could give more comfort, but might lead to fracture displacement reducing pronation and supination. Because this has not been systematically investigated, we set up a randomised multicentre study.

Identification of Familial Adenomatous Polyposis carriers among children with desmoid tumours.

Objective: Desmoid tumours are rare mesenchymal tumours with unpredictable progression and high recurrence risk. They can occur sporadically or in association with Familial Adenomatous Polyposis (FAP), which is caused by germline APC mutations. The Wnt/β-catenin pathway has a central role in the pathogenesis of desmoid tumours.

Copy number detection in discordant monozygotic twins of Congenital Diaphragmatic Hernia (CDH) and Esophageal Atresia (EA) cohorts.

The occurrence of phenotypic differences between monozygotic (MZ) twins is commonly attributed to environmental factors, assuming that MZ twins have a complete identical genetic make-up. Yet, recently several lines of evidence showed that both genetic and epigenetic factors could have a role in phenotypic discordance after all. A high occurrence of copy number variation (CNV) differences was observed within MZ twin pairs discordant for Parkinson's disease, thereby stressing on the importance of post-zygotic mutations as disease-predisposing events.

Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP).

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor in children. DFSP is characterized by a specific fusion of the platelet-derived growth factor beta (PDGFbeta) with the collagen type 1alpha1 (COL1alpha1) gene which renders these tumors responsive to targeted therapy with tyrosine kinase inhibitors, such as imatinib mesylate, as is reported in adults. In the current report, we describe the first small pediatric DFSP series, in which response to imatinib mesylate contributed to successful treatment outcome.

Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate.

Purpose: Congenital diaphragmatic hernia (CDH) has traditionally been repaired via a laparotomy. More and more reports on thoracoscopic repair are being published. The aim of this study was to evaluate our series of thoracoscopic CDH repair and compare this group to an open repair group treated during the same period in the same institute.

Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature.

Background: Aggressive fibromatosis (AF) is a soft tissue tumor and is rare in childhood, with high potential for local invasiveness and recurrence. General recommendations for the clinical management of pediatric patients with AF remain undetermined.

Methods: The authors retrospectively analyzed 13 children with AF who were diagnosed from 1987 until 2004 in the Erasmus MC-Sophia Children's Hospital, and a review of the pediatric literature was conducted.

Strategy for management of newborns with cervical teratoma.

Background: Cervical teratomas are extremely rare tumors with high perinatal mortality and morbidity rates.

Objective: To compare our experience and outcome in newborns with cervical teratoma with similar reports from the literature, in order to propose a structured approach.

Methods: A retrospective review of seven patients treated between 1986 and 2000 was performed.