Publications by authors named "Sabine Sarnacki"

Background: Ovarian mature teratomas (OMTs) are the most common benign ovarian tumors in pediatric patients. Management in adolescents can be performed by pediatric (PSs) or gynecologic surgeons (GSs). The aim of this study was to assess the differences in OMT management and the repercussions according to the risk of secondary events.

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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.

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Introduction: Establishing a robotic program in pediatric surgery remains challenging, with evidence-based practices still evolving. Over the last five years, robot-assisted surgery (RAS) has become the standard approach for several pediatric indications. This study presents the first national analysis of pediatric RAS in France, aiming to evaluate indications, procedural trends, and technical considerations.

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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.

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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.

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Introduction: Denys-Drash syndrome (DDS) is a rare disease typically associated with a triad of early onset nephrotic syndromes (NS), susceptibility to Wilms tumor (WT), and genitourinary structural defects. DDS is caused by Wilms' tumor suppression gene () variants, with the most frequent variants in exons 8 and 9. This study aimed to evaluate the long-term clinical outcomes and genotype-to-phenotype correlations in a large, multicenter cohort of children with typical DDS.

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Rectoperineal fistula (RPF) and rectovestibular fistula (RVF) are the most common forms of low anorectal malformations (ARMs) in girls, and lead to difficult stooling, thus demanding early surgical correction. This study's aim was to assess early outcomes associated with one-step ventral and dorsal proctoplasty in RPF/RVF.All female infants who consecutively underwent one-step proctoplasty for RPF/RVF at our institution (2012-2022) were retrospectively included.

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Background/purpose: Pediatric cancer is a major global health challenge, with an estimated 400,000 new cases diagnosed each year. Access to specialized surgical care is particularly limited in low- and middle-income countries (LMICs), where a shortage of trained pediatric surgical oncologists exacerbates treatment disparities. This study aimed to develop a comprehensive global curriculum for pediatric surgical oncology to enhance training and improve patient outcomes.

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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.

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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.

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Background: Ovarian mature teratoma represents the most common benign neoplasm among pediatric germ cell tumors. This study reports the prevalence and characteristics of familial forms identified in a single center over 22 years in order to better understand possible familial predispositions to ovarian teratoma.

Methods: The records of all patients who were surgically treated for ovarian teratoma between 2000 and 2022 were retrospectively reviewed.

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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.

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Background And Aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability.

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Objective: Adreno CorticoTropic Hormone (ACTH)-independent Cushing's syndrome (CS) in children is very rare but potentially fatal. In bilateral nodular hyperplasia, synchronous bilateral adrenalectomy (SBA) represents the definitive treatment to correct hypercortisolism. We aim to report the multidisciplinary management of this rare condition.

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Article Synopsis
  • The article outlines the development and growth of the Multidisciplinary Paediatric Robotic Program at Hôpital Necker-Enfants Malades in Paris, emphasizing the importance of a safe environment for patients and staff throughout all surgical phases.
  • It highlights how robotic surgery enhances the precision of operations on tumours, with careful consideration of tumour characteristics and surgical experience to determine feasibility.
  • The program aims for excellent cancer outcomes using minimally invasive techniques, focusing on selected paediatric tumours such as endocrine, neuroblastic, and renal cancers, while anticipating the future integration of robotic and image-guided surgery.
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Article Synopsis
  • - Anorectal malformations (ARMs) are uncommon congenital issues affecting the anus and rectum, which require complicated management; European experts are working on unified guidelines for better care.
  • - The guidelines were created based on the Dutch Quality Standard for ARMs and involved a literature review and evaluation by a panel of experts from several European countries, leading to adaptations and new recommendations.
  • - Emphasizing the need for specialized care throughout a patient's life, the guidelines outline essential diagnostic and follow-up processes for children with ARMs to ensure comprehensive management.
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Introduction:  Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked area of patient care. The European Reference Network eUROGEN for rare and complex urogenital conditions assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

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Background And Aims: We aimed to review current literature on the impact of Preoperative Identification (POI) of the Adamkiewicz Artery (AKA) in solid pediatric Posterior Thoracic Tumors (PTT), comprising a spectrum of neuroblastic tumors and neuroblastoma, with particular focus on Complete Macroscopic Excision (CME) and Neurologic Complications/Sequelae (NCS).

Methods: A systematic review in accordance with PRISMA guidelines was undertaken. The study included reports on pediatric patients providing available data on POI of the AKA in PTT.

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Neuroblastoma, the most common extracranial solid tumor in children under the age of 5, has been described as early as the 19th century, and its complexity has continued to intrigue researchers, as well as medical and surgical specialists. At one end of the phenotypic spectrum, neuroblastoma is self-limiting with minimal to no intervention required, while on the opposite end exists the challenge of refractory disease despite aggressive management and toxic systemic treatments. The goal of this review is to describe a comprehensive surgical perspective and contemporary approach to neuroblastoma.

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Article Synopsis
  • * A group of 15 experts from Europe created guidelines to help doctors and hospitals properly treat patients with ARMs based on new research and existing standards.
  • * The guidelines cover treatments, after-surgery care, toilet training, and managing issues like incontinence, emphasizing the need for a well-coordinated healthcare team.
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Introduction:  Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

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Background: Minimally invasive surgery for paediatric adrenal tumours has evolved, but robot-assisted laparoscopic adrenalectomy (RALA) in children remains poorly studied. The current prospective study aims to demonstrate the safety and efficacy of RALA in treating children with adrenal tumours.

Methods: A prospective institutional analysis of children presenting with neuroblastic and endocrine tumours treated with RALA was undertaken over a six year-period.

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Article Synopsis
  • Pancreatic neuroendocrine neoplasms (pNENs) are rare in children, with a study examining cases between 2011 and 2020 to understand their clinical presentation and behavior.
  • The study analyzed 15 patients, mostly adolescents, and found that a significant number had cancer predisposition syndromes, with some diagnosed through screening.
  • Results showed that patients with low-grade tumors had better survival rates, while those with high-grade or metastatic cancers faced poorer outcomes, highlighting the need for improved treatment strategies for severe cases.
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Background: Image-defined risk factors (IDRFs) were promulgated for predicting the feasibility and safety of complete primary tumor resection in children with neuroblastoma (NB). There is limited understanding of the impact of individual IDRFs on resectability of the primary tumor or patient outcomes. A multicenter database of patients with high-risk NB was interrogated to answer this question.

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