Showing papers by "Sabine Sarnacki published in 2021"

Thoracoscopic Management of Pediatric Patients with Congenital Lung Malformations: Results of a European Multicenter Survey.

Abstract: Background: This study aimed to report a European multi-institutional experience about thoracoscopic management of children with congenital lung malformations. Methods: The records of 102 patients ...

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

Abstract: 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 Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry)

Robotic Surgery in Pediatric Oncology: Lessons Learned from the First 100 Tumors-A Nationwide Experience.

Abstract: While robotics has become commonplace in adult oncology, it remains rare in pediatric oncology due to the rarity of childhood cancers. We present the results of a large nationwide experience with robotic oncology, with the aim of providing practical and feasible guidelines for child selection. This was a prospective analysis performed over a period of 4 years. Treatment was delivered according to the Societe Internationale d’Oncologie Pediatrique/International Society of Paediatric Oncology Europe Neuroblastoma Group (SIOP/SIOPEN) protocols. Indications were approved by a certified tumor board. Overall, 100 tumors were resected during 93 procedures (abdomen, 67%; thorax, 17%; pelvis, 10%; retroperitoneum, 6%) in 89 children (56 girls). The median age at surgery was 8.2 years (range 3.6–13); 19 children (21%) harbored germinal genetic alterations predisposing to cancer. No intraoperative tumor ruptures occurred. Seven conversions (8%) to an open approach were performed. Neuroblastic tumors (n = 31) comprised the main group (18 neuroblastomas, 4 ganglioneuroblastomas, 9 ganglioneuromas) and renal tumors comprised the second largest group (n = 24, including 20 Wilms’ tumors). The remaining 45 tumors included neuroendocrine (n = 12), adrenal (n = 9), germ-cell (n = 7), pancreatic (n = 4), thymic (n = 4), inflammatory myofibroblastic (n = 4), and different rare tumors (n = 5). Overall, 51 tumors were malignant, 2 were borderline, and 47 were benign. The median hospital stay was 3 days (2–4), and five postoperative complications occurred within the first 30 days. During a median follow-up of 2.4 years, one child (Wilms’ tumor) presented with pleural recurrence. One girl with Wilms’ tumor died of central nervous system metastasis. Robotic surgery for pediatric tumors is a safe option in highly selected cases. Indications should be discussed by tumor boards to avoid widespread and uncontrolled application.

Thymoma and thymic carcinoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations.

Abstract: Thymic tumors are epithelial tumors arising from the anterior mediastinum and constitute 0.2-1.5% of all adult malignancies but are exceptional in pediatric population. Thymic epithelial tumors (TETs) encompass a variety of histologic subtypes associated with different clinical outcomes. Due to its rarity in children, TETs' management requires a multidisciplinary approach. However, prognosis remains still poor, especially among patients with thymic carcinoma. This study presents the internationally recognized recommendations for the diagnosis and treatment of thymic tumors in children and adolescents, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) group within the EU-funded project Paediatric Rare Tumours Network - European Registry (PARTNER).

Anaesthesia management during Paediatric Robotic Surgery - Preliminary results from a single centre multidisciplinary experience.

Abstract: Introduction Paediatric robotic surgery is gaining popularity across multiple disciplines and offers technical advantages in complex procedures requiring delicate dissection. To date, limited publications describe its perioperative management in children. Material & methods We retrospectively analysed the prospectively collected anaesthetic data of the first 200 robotic-assisted surgery procedures in our paediatric university hospital as part of a multidisciplinary program from October of 2016 to February of 2019. Anaesthetic technique and monitoring were based on guidelines initially derived from adult data. We examined adverse events and particular outcomes including blood loss and analgesic requirements. Results Fifty-one different surgical procedures were performed in patients aged 4 months to 18 years (weight 5–144 kg). Operative times averaged 4 h and conversion rate was 3%. Neither robotic arm nor positional injury occurred. Limited access to the patient did not lead to any complication. Hypothermia was frequent and mostly self-limiting. Negative physiological effects due to positioning, body cavity insufflation or surgery manifesting as significant respiratory and haemodynamic changes occurred in 14% and 11% of patients, respectively. Overt haemorrhage complicated one case. Eighty per cent of 170 patients did not require level 3 analgesics postoperatively, while thoracic and certain tumour cases had greater analgesic requirements. Conclusion These preliminary results show that paediatric robotic surgery is well tolerated with a low bleeding risk and that major intraoperative events are uncommon. A consistent anaesthetic approach is effective across a broad range of procedures. Analgesic requirements are low excluding thoracic and some complex abdominal cases. Future studies should focus on the rehabilitative aspects of robotic surgery technique.

Ultrasound detected prenatal hyperechoic lung lesions and concordance with postnatal findings: A common aspect for multiple diagnoses.

Abstract: Objective Hyperechoic lung lesions are largely detected prenatally but their underlying etiology is still poorly defined. The aim of the study was to determine the concordance between pre and postnatal diagnosis of hyperechoic lung lesions. Methods Prenatal ultrasound (US) evaluation was performed by a fetal medicine specialist. Postnatal diagnosis was based on CT-scan. Pre- and postnatal features were retrieved from medical charts. Results Seventy five patients were included from January 2009 to December 2018. Main prenatal diagnoses were bronchopulmonary sequestrations (BPS) (n = 24%-32%), pulmonary cystic malformations (PCM) (n = 19%-25%), congenital lobar emphysemas (CLE) (n = 15%-20%). Mediastinal shift was observed in 18 cases (24%). The prenatal detection of a systemic arterial supply had a diagnostic accuracy of 90%, while the prenatal detection of a cystic component had a diagnostic accuracy of 76.5%. All 16 neonates with prenatal isolated mediastinal shift were asymptomatic at birth. Seven neonates showed respiratory distress that was not predicted prenatally. Conclusions Hyperechoic lung malformations reflect a heterogeneous group of lesions with a good concordance for bronchopulmonary sequestration, but not a satisfying prediction for cystic lesions.

Automatic Size And Pose Homogenization With Spatial Transformer Network To Improve And Accelerate Pediatric Segmentation

Abstract: Due to a high heterogeneity in pose and size and to a limited number of available data, segmentation of pediatric images is challenging for deep learning methods. In this work, we propose a new CNN architecture that is pose and scale invariant thanks to the use of Spatial Transformer Network (STN). Our architecture is composed of three sequential modules that are estimated together during training: (i) a regression module to estimate a similarity matrix to normalize the input image to a reference one; (ii) a differentiable module to find the region of interest to segment; (iii) a segmentation module, based on the popular UNet architecture, to delineate the object. Unlike the original UNet, which strives to learn a complex mapping, including pose and scale variations, from a finite training dataset, our segmentation module learns a simpler mapping focusing on images with normalized pose and size. Furthermore, the use of an automatic bounding box detection through STN allows saving time and especially memory, while keeping similar performance. We test the proposed method in kidney and renal tumor segmentation on abdominal pediatric CT scanners. Results indicate that the estimated STN homogenization of size and pose accelerates the segmentation (25h), compared to standard data-augmentation (33h), while obtaining a similar quality for the kidney (88.01% of Dice score) and improving the renal tumor delineation (from 85.52% to 87.12%).

Robotic Surgery in Pediatric Oncology: Lessons Learned from the First 100 Tumours. A Nationwide Experience

Abstract: Background: The use of robotics has become commonplace in adult oncology but remains rare in paediatric oncologic surgery. We present the results of a large nationwide experience with robotic surgery in children with benign and malignant tumours. Methods: Prospective analysis (ClinicalTrials.gov NCT03274050) of robotic procedures performed between 2008 and 2019. Children received treatment according to the ongoing SIOP/SIOPEN protocols. Indications were approved by a certified tumour board. Findings: A total of 100 tumours were resected during 92 procedures in 87 children (56 girls). The median age at surgery was 7·4 years (3·5-12); 20% of children harboured germinal genetic alterations predisposing to cancer. Procedures were performed by laparoscopy for abdominal (71%) or pelvic tumours (10%) and by retroperitoneoscopy (5%) or thoracoscopy (14%). No intraoperative tumour rupture occurred during robotic-assisted dissection. Eight conversions (9%) to an open approach occurred. Neuroblastic tumour (n=29) was the main group (19 neuroblastomas, three ganglioneuroblastomas, seven ganglioneuromas). Renal tumour was the second largest group (n=24, including 20 Wilms’ tumours). The other 47 tumours were neuroendocrine (n=13), adrenal (n=11), pancreatic (n=3), germ-cell (n=7), thymic (n=4), inflammatory myofibroblastic (n=4) and five different rare tumours. Overall, 50 tumours were malignant, 2 were borderline and 48 were benign. The median hospital stay was 3 days (2-4). Five post-operative surgical complications occurred within the first 30 days in four patients. During a follow-up of 1 to 28 months, one child (Wilms’ tumour) presented pleural recurrence. One girl with Wilms’ tumour died of central nervous system metastasis. Interpretation: Robotic surgery for paediatric tumours is feasible and may be an option in highly selected cases. Indications should be discussed by tumour boards to avoid widespread and uncontrolled application of the approach. Children with genetic predisposition to cancer seem good candidates for the robotic approach. Trial Registration: ClinicalTrials.gov NCT03274050 Funding: The sponsor was Assistance Publique–Hopitaux de Paris (APHP, Clinical Research and Innovation Delegation) and this project was funded by a grant from Necker Hospital. Declaration of Interests: None to declare. Ethics Approval Statement: This prospective analysis received approval from institutional review boards and an independent ethics committee (Comite de Protection des Personnes, CPP Ile de France VII). The sponsor was Assistance Publique–Hopitaux de Paris (APHP, Clinical Research and Innovation Delegation).

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

Abstract: 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. DESIGN This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved. RESULTS Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not. CONCLUSION This study is a plea in favor of OSS as the first-choice treatment of OMT when possible. Close follow-up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors.

Automatic size and pose homogenization with spatial transformer network to improve and accelerate pediatric segmentation

Abstract: Due to a high heterogeneity in pose and size and to a limited number of available data, segmentation of pediatric images is challenging for deep learning methods. In this work, we propose a new CNN architecture that is pose and scale invariant thanks to the use of Spatial Transformer Network (STN). Our architecture is composed of three sequential modules that are estimated together during training: (i) a regression module to estimate a similarity matrix to normalize the input image to a reference one; (ii) a differentiable module to find the region of interest to segment; (iii) a segmentation module, based on the popular UNet architecture, to delineate the object. Unlike the original UNet, which strives to learn a complex mapping, including pose and scale variations, from a finite training dataset, our segmentation module learns a simpler mapping focusing on images with normalized pose and size. Furthermore, the use of an automatic bounding box detection through STN allows saving time and especially memory, while keeping similar performance. We test the proposed method in kidney and renal tumor segmentation on abdominal pediatric CT scanners. Results indicate that the estimated STN homogenization of size and pose accelerates the segmentation (25h), compared to standard data-augmentation (33h), while obtaining a similar quality for the kidney (88.01\% of Dice score) and improving the renal tumor delineation (from 85.52\% to 87.12\%).

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

Abstract: Introduction Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. Materials & Methods: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. Results A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. Conclusions MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Childhood ovarian non-seminomatous germ cell tumors: A Highly Curable Disease With Few Long-Term Treatment-Related Toxicities. Results of the French TGM95 study

Abstract: We report survival and late effects analysis of TGM95 study for childhood (≤18years) ovarian non-seminomatous 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-cisplatinum) in intermediate-risk group (IR: FIGO-stage IC-II-III and AFP 15years (median=26years) with remaining ovarian tissue, all had developed spontaneous puberty and 21 (41%) had at least one pregnancy (including two with infertility treatment). Among 69 patients treated with platinum-based chemotherapy, chronic-kidney-disease was diagnosed in 4 patients (3 after VIP) and significant ototoxicity occurred in 3 (all grade-2). Childhood ovarian NS-GCTs have an excellent prognosis with few late effects. The low-intensive etoposide-free VBP regimen could be an alternative in children with IR disease especially in cases of tumor rupture. The risk of contralateral mature teratoma needs regular monitoring of the remaining ovary. This article is protected by copyright. All rights reserved.