Publications by authors named "Orbach D"

Several legal acts mandate that management agencies regularly assess biological populations. For species with distinct markings, these assessments can be conducted noninvasively via capture-recapture and photographic identification (photo-ID), which involves processing considerable quantities of photographic data. To ease this burden, agencies increasingly rely on automated identification (ID) algorithms.

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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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Pharmaceuticals prevent and treat diseases, yet inappropriate intake can result in harmful effects including mortality. Contaminants have become recurrent public and wildlife health concerns. Bioaccumulation of contaminants can occur throughout trophic levels of the food web.

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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.

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The editorial board of the Bulletin du cancer has compiled a summary of the news from 2024 in oncology, based on the main results presented at international congresses or published over the past year. After a year marked by the success of the Olympic Games, the selection of data is presented and discussed in podiums of three main results by topic. Emphasis is placed on studies that have an immediate impact on practice and on data that raise important questions for the year 2025.

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Background: Spetzler-Martin (SM) Grade III brain arteriovenous malformations (BAVMs) represent a transitional risk zone between low- and high-grade BAVMs, characterized by diverse angioarchitecture. The primary treatment options are endovascular embolization, microsurgical resection (MS), and stereotactic radiosurgery (SRS). This study compares the efficacy and outcomes of MS and SRS.

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Bow-riding occurs when dolphins swim in the pressure waves at the front of a vessel. Bow-riding is hypothesized to be "fun" for dolphins or to save them energy although the energetics have not been explored. An UAS (Unoccupied Aerial System) was used to follow and video-record adult dusky dolphins (Lagenorhynchus obscurus) bow-riding in front of a research vessel or free-swimming off Kaikoura, New Zealand.

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Purpose: TRK fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven CNS tumors.

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Background: The prognosis for patients with relapse of localized rhabdomyosarcoma (RMS) remains poor, with limited evidence for optimal second-line therapy. This study describes the management and outcomes of relapsed RMS patients in France.

Methods: We retrospectively reviewed all nonmetastatic RMS patients enrolled in France in the RMS 2005 study who relapsed between 2006 and 2019 after achieving complete local control, defined as complete remission or stable residue ≥ 6 months after treatment completion.

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Purpose: Infantile myofibromatosis is characterized by the development of myofibroblastic tumors in young children. In most cases, the disease is caused by somatic gain-of-function variants in platelet-derived growth factor (PDGF) receptor beta (PDGFRB). Here, we reported a novel germline intronic PDGFRB variant, c.

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Bottlenose dolphins () inhabit environments with variable natural salinities and experience physiological imbalances from extreme salinity fluctuations. Low salinity exposure (≤10) disrupts osmoregulation and increases the production of steroid hormones aldosterone (electrolyte balance) and cortisol (stress response). The effect(s) of high salinity exposure (>35) on steroid hormone production in bottlenose dolphins has not previously been assessed.

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Background: Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.

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Objective: To assess the feasibility, accuracy, and safety of ultrasound (US)-guided coaxial core-needle biopsy (CNB) for histomolecular diagnosis of extra-ocular orbital soft tissue tumours as a minimally invasive alternative to surgical biopsy.

Methods: This retrospective study was conducted at a single Comprehensive Cancer Center and included all consecutive patients referred to our center between 2015 and 2023 for the diagnosis and treatment of orbital soft tissue tumours. All patients underwent US-guided transconjunctival coaxial CNB using a semiautomatic 18-gauge biopsy gun.

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Pediatric cerebrovascular diseases have distinct clinical presentations, pathophysiology, and management compared to the adult counterparts. This introductory article discusses the imaging techniques and neurovascular conditions unique to each age group from the fetal stages through childhood, including vascular malformations, arteriopathy, and strokes. The article also underscores the importance of genetic factors and the need for a multidisciplinary approach in the diagnosis and treatment of pediatric neurovascular disorders.

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Article Synopsis
  • - Gastrointestinal stromal tumours (GIST) are digestive tract tumors primarily affecting adults, with limited management guidelines for pediatric cases.
  • - An updated literature review reveals that GIST in children often links to syndromic or familial contexts, with over 170 documented cases, primarily involving genetic alterations in Succinate Dehydrogenase (SDH).
  • - Management recommendations for pediatric GIST are similar to those for adults, but specific biological differences necessitate tailored therapeutic approaches, prompting international collaborations to enhance understanding of this rare condition.
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Objective: To assess whether white matter injuries differ in symptomatic vs asymptomatic moyamoya-affected hemispheres using diffusion magnetic resonance imaging since there is controversy regarding when or if to revascularize children with asymptomatic moyamoya.

Study Design: We conducted a cross-sectional study of children with moyamoya who underwent diffusion magnetic resonance imaging before revascularization surgery as well as controls without moyamoya. We measured the fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity of white matter tracts in the watershed regions.

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Background: Arteriovenous malformations (AVMs) are uncommon cerebral lesions that can cause significant neurological complications. Surgical resection is the gold standard for treatment, but endovascular embolization and stereotactic radiosurgery (SRS) are viable alternatives.

Objective: To compare the outcomes of endovascular embolization versus SRS in the treatment of AVMs with Spetzler-Martin grades I-III.

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Article Synopsis
  • For 88 years, researchers have tried to solve Gray's paradox, which questions how dolphins can swim so fast without expending too much energy.
  • Using an uncrewed aerial vehicle, the study measured the drag and power needed for dusky dolphins swimming at various speeds, revealing that drag coefficients decreased exponentially as speed increased.
  • Findings indicated that at higher speeds, the reduced drag allowed dolphins to swim efficiently, making their high-speed swimming feasible without violating physiological limits, thus providing an explanation for Gray's paradox.
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Purpose: Within the Paediatric Rare Tumours Network-European Registry (PARTNER) project, we aimed to evaluate the situation on the registration and management of paediatric patients affected by very rare tumours (VRT) in the European low health expenditure average rates (LHEAR) countries.

Methods: A survey regarding infrastructure, organisation, and clinical decision-making information on VRT was designed. This survey was distributed to the representatives of LHEAR countries involved in the activities of the PARTNER Work Package 7.

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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: The authors report the prospective evaluation of reduced dose alkylator chemotherapy combined with radiotherapy for European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) standard risk nonalveolar rhabdomyosarcoma (NA-RMS).

Patients And Methods: Localized node negative Intergroup Rhabdomyosarcoma Study (IRS) II/III NA-RMS at favorable sites (subgroup C), <25 years old, received five cycles of ifosfamide, vincristine, and dactinomycin (IVA) chemotherapy (30 g/m ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m ifosfamide) ± radiotherapy. Delayed primary tumor excision was considered for IRS III tumors.

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Article Synopsis
  • Desmoid tumors (DT) are rare, aggressive growths that have historically been treated primarily with surgery, but recent trends suggest a shift towards less invasive treatment options.
  • A consensus meeting held in Milan in June 2023 aimed to update global guidelines for DT management, bringing together over 90 experts and patient advocates to discuss new strategies and treatments.
  • The updated guidelines emphasize the importance of local therapies and include information on the latest medical agents, particularly γ-secretase inhibitors, to ensure informed and effective management of DT in specialized referral centers.
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Background And Aims: Alveolar soft part sarcoma (ASPS) is an ultra-rare chemo-resistant sarcoma in children, occurring preferentially in young adults. We aimed to describe and compare its clinical presentation and behaviour in children and young adults to determine whether the same therapeutic strategy should be addressed for both populations.

Methods: National retrospective multicentre study of children (0-18 years) vs.

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Article Synopsis
  • Four types of vascular anomalies affect the fetal brain, including vein of Galen malformations and dural sinus malformations, and they typically don't manifest after birth.
  • * MRI is crucial for diagnosing these conditions, evaluating potential brain injuries, and planning treatments, especially for high-risk cases.
  • * A new investigational procedure involves transuterine embolization for vein of Galen malformations, offering a promising treatment alternative to conventional methods.
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