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Author Norbert Graf

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Prognostic Significance Of Histopathological Response To Preoperative Chemotherapy In Unilateral Wilms Tumor: An Analysis Of 899 Patients Treated On The Siop Wt 2001 Protocol In The Uk Cclg And Gpoh Studies.

Gordan M Vujanić, Ellen D'Hooghe, Norbert Graf, Christian Vokuhl, Reem Al-Saad, Tanzina Chowdhury, Kathy Pritchard-Jones, Rhoikos Furtwängler,

In the SIOP Wilms tumor (WT) studies preoperative chemotherapy is used as primary treatment, and tumors are classified thereafter by pathologists. Completely necrotic WTs (CN-WTs) are classified as low-risk tumors. The aim of the study was to evaluate whether a subset of regressive type WTs (RT-WTs)(67-99% chemotherapy-induced changes - CIC) ... Read more >>

Int J Cancer (International journal of cancer)
[2021, :]

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Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations.

Julian Thalhammer, Gerhard Kindle, Alexandra Nieters, Stephan Rusch, Mikko R J Seppänen, Alain Fischer, Bodo Grimbacher, David Edgar, Matthew Buckland, Nizar Mahlaoui, Stephan Ehl, ,

<h4>Background</h4>Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.<h4>Objective</h4>We sought to analyze ... Read more >>

J Allergy Clin Immunol (The Journal of allergy and clinical immunology)
[2021, :]

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MyPal-Child study protocol: an observational prospective clinical feasibility study of the MyPal ePRO-based early palliative care digital system in paediatric oncology patients.

Marcel Meyerheim, Christina Karamanidou, Sheila Payne, Tina Garani-Papadatos, Annette Sander, Julia Downing, Kostas Stamatopoulos, J Ling, Cathy Payne, Lydia Scarfò, Petr Lokaj, Christos Maramis, Norbert Graf,

<h4>Introduction</h4>Electronic patient-reported outcomes (ePROs) have tremendous potential to optimise palliative and supportive care for children with cancer, their families and healthcare providers. Particularly, these children and their families are subjected to multiple strains caused by the disease and its treatment. The MyPal digital health platform is designed to address these ... Read more >>

BMJ Open (BMJ open)
[2021, 11(4):e045226]

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Management of the First COVID-19 Wave in 45 Pediatric Practices in Saarland.

Karsten Theiß, Arne Simon, Norbert Graf, Tilman Rohrer,

<h4>Hintergrund</h4>Das Saarland ist in der ersten Welle der COVID-19-Pandemie eines der am stärksten betroffenen Bundesländer. Niedergelassene Kinder- und Jugendärzte sind für pädiatrische Patienten und ihre Familien erste Ansprechpartner.<h4>Fragestellung</h4>Darstellung der Herausforderungen und Maßnahmen zur Aufrechterhaltung der Patientenversorgung sowie der Zusammenarbeit während der COVID-19-Pandemie.<h4>Methoden</h4>Internet-basierte Befragung der niedergelassenen Kinder- und Jugendärzte sowie papierbasierte ... Read more >>

Gesundheitswesen (Gesundheitswesen (Bundesverband der Arzte des Offentlichen Gesundheitsdienstes (Germany)))
[2021, 83(4):258-264]

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Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature.

Sophie E van Peer, Corine J H Pleijte, Ronald R de Krijger, Marjolijn C J Jongmans, Roland P Kuiper, Marc R Lilien, Martine van Grotel, Norbert Graf, Marry M van den Heuvel-Eibrink, Janna A Hol,

In children presenting with a predominantly cystic renal tumor, the most likely diagnoses include cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma (CN). Both entities are rare and limited information on the clinical and molecular characteristics, treatment, and outcome is available since large cohort studies are lacking. We performed an ... Read more >>

Cancers (Basel) (Cancers)
[2021, 13(5):]

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Inter-clinician delineation variation for a new highly-conformal flank target volume in children with renal tumors: A SIOP-Renal Tumor Study Group international multicenter exercise.

Joeri Mul, Patrick Melchior, Enrica Seravalli, Daniel Saunders, Stephanie Bolle, Alison L Cameron, Kristin Gurtner, Semi Harrabi, Yasmin Lassen-Ramshad, Naomi Lavan, Henriette Magelssen, Henry Mandeville, Tom Boterberg, Petra S Kroon, Alexis N T J Kotte, Bianca A W Hoeben, Peter S N van Rossum, Martine van Grotel, Norbert Graf, Marry M van den Heuvel-Eibrink, Christian Rübe, Geert O Janssens,

<h4>Background and purpose</h4>Recently, the SIOP-RTSG developed a highly-conformal flank target volume definition for children with renal tumors. The aims of this study were to evaluate the inter-clinician delineation variation of this new target volume definition in an international multicenter setting and to explore the necessity of quality assurance.<h4>Materials and methods</h4>Six ... Read more >>

Clin Transl Radiat Oncol (Clinical and translational radiation oncology)
[2021, 28:39-47]

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School and kindergarten attendance and home schooling of pediatric cancer patients before and during the SARS-CoV-2 pandemic: results of a survey of the German Society for Pediatric Oncology and Hematology.

Arne Simon, Benjamin Siebald, Walther Stamm, Norbert Graf, Stephan Meier, Martin Schrappe, Andreas H Groll, Hans-Jürgen Laws, Thomas Lehrnbecher,

In this multicenter survey (July 07 to August 08, 2020) in pediatric oncology centers (POCs) belonging to the German Society for Pediatric Oncology and Hematology (GPOH), 36 POCs participated (response rate 70.6%). Home schooling practice was judged as satisfying by 79% prior to and by 38% during the pandemic (<i>P</i>=0.0007). ... Read more >>

GMS Hyg Infect Control (GMS hygiene and infection control)
[2021, 16:Doc10]

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[Prospektives Audit des Gentamicin Drug Monitorings in einem Kinderkrebszentrum].

Sarah Herberger, Nadine Oberkircher, Gentiana I Wenzel, Dietmar Hecker, Gudrun Wagenpfeil, Rhoikos Furtwängler, Sören L Becker, Cihan Papan, Norbert Graf, Arne Simon,

<h4>Background</h4>Many pediatric cancer centers still use Gentamicin as first line combination treatment in patients with fever and neutropenia. Since 2011, our center has implemented a dosing regimen with 250 mg/m<sup>2</sup> BSA (max. 10 mg/kg, max. 400 mg) as a single daily infusion according to the German guideline.<h4>Patients and methods</h4>In this ... Read more >>

Klin Padiatr (Klinische Padiatrie)
[2021, 233(3):123-126]

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Diffusion-weighted MRI and histogram analysis: assessment of response to neoadjuvant chemotherapy in nephroblastoma.

Andreas M Hötker, Yousef Mazaheri, André Lollert, Jens-Peter Schenk, Junting Zheng, Marinela Capanu, Oguz Akin, Norbert Graf, Gundula Staatz,

<h4>Purpose</h4>To assess the value of diffusion-weighted MRI (DW-MRI) in the non-invasive prediction of blastemal remnant after neoadjuvant chemotherapy in nephroblastoma.<h4>Methods</h4>This IRB-approved study included 32 pediatric patients with 35 tumors who underwent DW-MRI prior and after completion of neoadjuvant chemotherapy and subsequent surgical resection. Two blinded radiologists volumetrically assessed each tumor ... Read more >>

Abdom Radiol (NY) (Abdominal radiology (New York))
[2021, :]

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[Consequences of the lockdown during the first COVID-19 wave in 34 pediatric practices in Saarland].

Karsten Theiß, Arne Simon, Norbert Graf, Tilman Rohrer,

<h4>Background</h4>In the first COVID-19 pandemic wave Saarland was one of the most affected federal states. Pediatric practices are the primary point of contact regarding questions about risk factors, symptoms and suspicion of illness. The lockdown could have a severe impact on overall number of consultancies, preventive medical check-ups and vaccinations.<h4>Objective</h4>The impact ... Read more >>

Monatsschr Kinderheilkd (Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde)
[2021, :1-9]

Cited: 2 times

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Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol.

Raquel Dávila Fajardo, Rhoikos Furtwängler, Martine van Grotel, Harm van Tinteren, Claudia Pasqualini, Kathy Pritchard-Jones, Reem Al-Saadi, Beatriz de Camargo, Gema L Ramírez Villar, Norbert Graf, Xavier Muracciole, Patrick Melchior, Daniel Saunders, Christian Rübe, Marry M van den Heuvel-Eibrink, Geert O Janssens, Arnauld C Verschuur,

<h4>Objective</h4>Wilms tumour (WT) patients with a localised completely necrotic nephroblastoma after preoperative chemotherapy are a favourable outcome group. Since the introduction of the SIOP 2001 protocol, the SIOP- Renal Tumour Study Group (SIOP-RTSG) has omitted radiotherapy for such patients with low-risk, local stage III in an attempt to reduce treatment ... Read more >>

Cancers (Basel) (Cancers)
[2021, 13(5):]

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[Diagnosis and therapy of tumors with NTRK gene fusion].

Albrecht Stenzinger, Cornelis M van Tilburg, Ghazaleh Tabatabai, Florian Länger, Norbert Graf, Frank Griesinger, Lukas C Heukamp, Michael Hummel, Thomas Klingebiel, Simone Hettmer, Christian Vokuhl, Sabine Merkelbach-Bruse, Friedrich Overkamp, Peter Reichardt, Monika Scheer, Wilko Weichert, C Benedikt Westphalen, Carsten Bokemeyer, Philipp Ivanyi, Sonja Loges, Peter Schirmacher, Bernhard Wörmann, Stefan Bielack, Thomas T W Seufferlein,

NTRK gene fusions are sporadic genetic alterations that can occur across tumor entities. Whereas they are quite rare in most solid tumors they are present at much higher frequencies in certain rare tumors such as infantile fibrosarcoma, congenital mesoblastic nephroma, secretory breast, or salivary gland carcinoma. NTRK gene fusions or ... Read more >>

Pathologe (Der Pathologe)
[2021, 42(1):103-115]

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Clear cell sarcoma of the kidney in Austrian children: Long-term survival after relapse.

Waltraud Friesenbichler, Roswitha Lüftinger, Gabriele Kropshofer, Martin Henkel, Gabriele Amann, Rhoikos Furtwängler, Norbert Graf, Leo Kager,

<h4>Introduction</h4>Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce.<h4>Patients and methods</h4>Retrospective data analysis of all Austrian children with CCSK. ... Read more >>

Pediatr Blood Cancer (Pediatric blood & cancer)
[2021, 68(5):e28860]

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Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93-01, 2001 and UK-IMPORT database: A report of the SIOP-Renal Tumor Study Group.

Justine N van der Beek, Janna A Hol, Aurore Coulomb-l'Hermine, Norbert Graf, Harm van Tinteren, Kathy Pritchard-Jones, Maite E Houwing, Ronald R de Krijger, Gordan M Vujanic, Kristina Dzhuma, Jens-Peter Schenk, Annemieke S Littooij, Gema L Ramírez-Villar, Dermot Murphy, Satyajit Ray, Reem Al-Saadi, Manfred Gessler, Jan Godzinski, Christian Ruebe, Paola Collini, Arnaud C Verschuur, Tony Frisk, Christian Vokuhl, Christina A Hulsbergen-van de Kaa, Beatriz de Camargo, Bengt Sandstedt, Barbara Selle, Godelieve A M Tytgat, Marry M van den Heuvel-Eibrink,

In children, renal cell carcinoma (RCC) is rare. This study is the first report of pediatric patients with RCC registered by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93-01, 2001 and UK-IMPORT databases, were included. Event-free survival (EFS) ... Read more >>

Int J Cancer (International journal of cancer)
[2021, 148(11):2724-2735]

Cited: 1 time

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New approaches to risk stratification for Wilms tumor.

Marie V Nelson, Marry M van den Heuvel-Eibrink, Norbert Graf, Jeffrey S Dome,

<h4>Purpose of review</h4>The treatment of Wilms tumor is one of the great achievements in the field of oncology. One of the key success factors has been improved risk stratification, enabling augmentation or reduction of therapy depending on a patient's risk of relapse. This article highlights the evolution of clinical and ... Read more >>

Curr Opin Pediatr (Current opinion in pediatrics)
[2021, 33(1):40-48]

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[Alice in the digital wonderland-pediatric teaching during the COVID-19 pandemic].

Martin Häusler, Hans Martin Bosse, Thomas Fischbach, Norbert Graf, Jürgen-Christoph von Kleist-Retzow, Joachim Kreuder,

The COVID-19 pandemic led to a rapid switch from undergraduate classroom teaching to online-teaching; a challenging process for teachers and students. Based on a recent online survey among German pediatric university hospitals the "AG Lehre der DGKJ" (teaching working group of the German Society of Pediatrics and Adolescent Medicine) summarizes latest experiences with ... Read more >>

Monatsschr Kinderheilkd (Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde)
[2020, :1-8]

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Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK).

Karolina Nemes, Susanne Bens, Denis Kachanov, Margarita Teleshova, Peter Hauser, Thorsten Simon, Stephan Tippelt, Wilhelm Woessmann, Olaf Beck, Christian Flotho, Lorenz Grigull, Pablo H Driever, Paul-Gerhardt Schlegel, Claudia Khurana, Kathrin Hering, Reinhard Kolb, Alfred Leipold, Floor Abbink, Maria J Gil-Da-Costa, Martin Benesch, Kornelius Kerl, Stephen Lowis, Carmen H Marques, Norbert Graf, Karsten Nysom, Christian Vokuhl, Patrick Melchior, Thomas Kröncke, Reinhard Schneppenheim, Uwe Kordes, Joachim Gerss, Reiner Siebert, Rhoikos Furtwängler, Michael C Frühwald,

<h4>Introduction</h4>Extracranial rhabdoid tumours are rare, highly aggressive malignancies primarily affecting young children. The EU-RHAB registry was initiated in 2009 to prospectively collect data of rhabdoid tumour patients treated according to the EU-RHAB therapeutic framework.<h4>Methods</h4>We evaluated 100 patients recruited within EU-RHAB (2009-2018). Tumours and matching blood samples were examined for SMARCB1 ... Read more >>

Eur J Cancer (European journal of cancer (Oxford, England : 1990))
[2021, 142:112-122]

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Unmet needs for relapsed or refractory Wilms tumour: Mapping the molecular features, exploring organoids and designing early phase trials - A collaborative SIOP-RTSG, COG and ITCC session at the first SIOPE meeting.

Jesper Brok, Annelies M C Mavinkurve-Groothuis, Jarno Drost, Daniela Perotti, James I Geller, Amy L Walz, Birgit Geoerger, Claudia Pasqualini, Arnauld Verschuur, Angela Polanco, Kathy P Jones, Marry van den Heuvel-Eibrink, Norbert Graf, Filippo Spreafico,

Wilms tumour (WT) accounts for about 6% of all childhood cancers and overall survival of WT is about 90% in international protocols. However, for WT subgroups with much poorer prognoses, i.e. typically high-risk (unfavorable) histology and/or relapse, there is an unmet need to better understand the biology of WT and to translate ... Read more >>

Eur J Cancer (European journal of cancer (Oxford, England : 1990))
[2021, 144:113-122]

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Clinical characteristics and outcomes of children with WAGR syndrome and Wilms tumor and/or nephroblastomatosis: The 30-year SIOP-RTSG experience.

Janna A Hol, Marjolijn C J Jongmans, Hélène Sudour-Bonnange, Gema L Ramírez-Villar, Tanzina Chowdhury, Catherine Rechnitzer, Niklas Pal, Gudrun Schleiermacher, Axel Karow, Roland P Kuiper, Beatriz de Camargo, Simona Avcin, Danka Redzic, Antonio Wachtel, Heidi Segers, Gordan M Vujanic, Harm van Tinteren, Christophe Bergeron, Kathy Pritchard-Jones, Norbert Graf, Marry M van den Heuvel-Eibrink, ,

<h4>Background</h4>WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare contiguous gene deletion syndrome with a 45% to 60% risk of developing Wilms tumor (WT). Currently, surveillance and treatment recommendations are based on limited evidence.<h4>Methods</h4>Clinical characteristics, treatments, and outcomes were analyzed for patients with WAGR ... Read more >>

Cancer (Cancer)
[2021, 127(4):628-638]

Cited: 1 time

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Robustness of brain tumor segmentation.

Sabine Müller, Joachim Weickert, Norbert Graf,

<b>Purpose:</b> The segmentation of brain tumors is one of the most active areas of medical image analysis. While current methods perform superhuman on benchmark data sets, their applicability in daily clinical practice has not been evaluated. In this work, we investigate the generalization behavior of deep neural networks in this ... Read more >>

J Med Imaging (Bellingham) (Journal of medical imaging (Bellingham, Wash.))
[2020, 7(6):064006]

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Diffusion-weighted MRI in the assessment of nephroblastoma: results of a multi-center trial.

Andreas M Hötker, André Lollert, Yousef Mazaheri, Sabine Müller, Jens-Peter Schenk, Philipp C Mildenberger, Oguz Akin, Norbert Graf, Gundula Staatz,

PURPOSE:To assess the value of diffusion-weighted MRI in the pre-therapeutic evaluation of pediatric renal cortical tumors. METHODS:This IRB-approved, retrospective multi-center study included 122 pediatric patients with 130 renal tumors, who underwent MRI including DWI before neoadjuvant chemotherapy and nephrectomy. Two radiologists independently assessed each tumor volumetrically, and apparent diffusion coefficient ... Read more >>

Abdom Radiol (NY) (Abdominal radiology (New York))
[2020, 45(10):3202-3212]

Cited: 1 time

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The SIOP-Renal Tumour Study Group consensus statement on flank target volume delineation for highly conformal radiotherapy.

Geert O Janssens, Patrick Melchior, Joeri Mul, Daniel Saunders, Stephanie Bolle, Alison L Cameron, Line Claude, Kristin Gurtner, Kees P van de Ven, Martine van Grotel, Semi Harrabi, Yasmin Lassen-Ramshad, Naomi Lavan, Henriette Magelssen, Xavier Muracciole, Tom Boterberg, Henry Mandeville, Jan Godzinski, Norbert Graf, Marry M van den Heuvel-Eibrink, Christian Rübe,

For decades, radiotherapy with two opposing photon beams has been the standard technique used to cover the flank target volume in paediatric patients with renal tumours. Nowadays, many institutes are implementing advanced radiotherapy techniques that spare healthy tissue. To decrease the radiotherapy dose to healthy structures while preserving oncological efficacy, ... Read more >>

Lancet Child Adolesc Health (The Lancet. Child & adolescent health)
[2020, 4(11):846-852]

Cited: 2 times

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Functional loss of a noncanonical BCOR-PRC1.1 complex accelerates SHH-driven medulloblastoma formation.

Lena M Kutscher, Konstantin Okonechnikov, Nadja V Batora, Jessica Clark, Patricia B G Silva, Mikaella Vouri, Sjoerd van Rijn, Laura Sieber, Britta Statz, Micah D Gearhart, Ryo Shiraishi, Norman Mack, Brent A Orr, Andrey Korshunov, Brian L Gudenas, Kyle S Smith, Audrey L Mercier, Olivier Ayrault, Mikio Hoshino, Marcel Kool, Katja von Hoff, Norbert Graf, Gudrun Fleischhack, Vivian J Bardwell, Stefan M Pfister, Paul A Northcott, Daisuke Kawauchi,

Medulloblastoma is a malignant childhood brain tumor arising from the developing cerebellum. In Sonic Hedgehog (SHH) subgroup medulloblastoma, aberrant activation of SHH signaling causes increased proliferation of granule neuron progenitors (GNPs), and predisposes these cells to tumorigenesis. A second, cooperating genetic hit is often required to push these hyperplastic cells ... Read more >>

Genes Dev (Genes & development)
[2020, 34(17-18):1161-1176]

Cited: 4 times

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Persistent aseptic meningitis in a child-think patch.

Nasenien Nourkami-Tutdibi, Arne Simon, Rhoikos Furtwängler, Norbert Graf, Umut Yilmaz, Joachim Oertel, Sascha Meyer,

<h4>Background</h4>In patients with neurosurgical interventions requiring dura reconstruction, the use of bovine graft material may be required.<h4>Patients and methods</h4>Case report.<h4>Results</h4>We present a 12-year-old girl with a profound graft reaction with severe neurologic symptoms mimicking post-neurosurgical bacterial meningitis after resection of an infra-tentorial brain tumour.<h4>Conclusion</h4>It is important to take into consideration this rare ... Read more >>

Wien Med Wochenschr (Wiener medizinische Wochenschrift (1946))
[2021, 171(1-2):38-40]

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Characteristics and Outcome of Children with Renal Cell Carcinoma: A Narrative Review.

Justine N van der Beek, James I Geller, Ronald R de Krijger, Norbert Graf, Kathy Pritchard-Jones, Jarno Drost, Arnauld C Verschuur, Dermot Murphy, Satyajit Ray, Filippo Spreafico, Kristina Dzhuma, Annemieke S Littooij, Barbara Selle, Godelieve A M Tytgat, Marry M van den Heuvel-Eibrink,

Pediatric renal cell carcinoma (RCC) is a rare type of kidney cancer, most commonly occurring in teenagers and young adolescents. Few relatively large series of pediatric RCC have been reported. Knowledge of clinical characteristics, outcome and treatment strategies are often based on the more frequently occurring adult types of RCC. ... Read more >>

Cancers (Basel) (Cancers)
[2020, 12(7):]

Cited: 1 time

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