| Autor(en) |
Titel |
Quelle |
Links |
| Oberlin O, Rey A, Anderson J, Carli M, Raney R, Treuner J, Stevens M |
Treatment of orbital rhabdomyosarcoma. |
J Clin Oncol 2001, 19: 197 |
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| Oberthuer A, Hero B, Spitz R, Berthold F, Fischer M |
The tumor-associated antigen PRAME is universally expressed in high-stage neuroblastoma and associated with poor outcome. |
Clin Cancer Res 2004, 10: 4307 |
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| Oberthuer A, Berthold F, Warnat P, Hero B, Kahlert Y, Spitz R, Ernestus K, König R, Haas S, Eils R, Schwab M, Brors B, Westermann F, Fischer M |
Customized oligonucleotide microarray gene expression-based classification of neuroblastoma patients outperforms current clinical risk stratification. |
Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2006, 24: 5070 |
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| PURPOSE: To develop a gene expression-based classifier for neuroblastoma patients that reliably predicts courses of the disease. PATIENTS AND METHODS: Two hundred fifty-one neuroblastoma specimens were analyzed using a customized oligonucleotide microarray comprising 10,163 probes for transcripts with differential expression in clinical subgroups of the disease. Subsequently, the prediction analysis for microarrays (PAM) was applied to a first set of patients with maximally divergent clinical courses (n = 77). The classification accuracy was estimated by a complete 10-times-repeated 10-fold cross validation, and a 144-gene predictor was constructed from this set. This classifier's predictive power was evaluated in an independent second set (n = 174) by comparing results of the gene expression-based classification with those of risk stratification systems of current trials from Germany, Japan, and the United States. RESULTS: The first set of patients was accurately predicted by PAM (cross-validated accuracy, 99%). Within the second set, the PAM classifier significantly separated cohorts with distinct courses (3-year event-free survival [EFS] 0.86 +/- 0.03 [favorable; n = 115] v 0.52 +/- 0.07 [unfavorable; n = 59] and 3-year overall survival 0.99 +/- 0.01 v 0.84 +/- 0.05; both P < .0001) and separated risk groups of current neuroblastoma trials into subgroups with divergent outcome (NB2004: low-risk 3-year EFS 0.86 +/- 0.04 v 0.25 +/- 0.15, P < .0001; intermediate-risk 1.00 v 0.57 +/- 0.19, P = .018; high-risk 0.81 +/- 0.10 v 0.56 +/- 0.08, P = .06). In a multivariate Cox regression model, the PAM predictor classified patients of the second set more accurately than risk stratification of current trials from Germany, Japan, and the United States (P < .001; hazard ratio, 4.756 [95% CI, 2.544 to 8.893]). CONCLUSION: Integration of gene expression-based class prediction of neuroblastoma patients may improve risk estimation of current neuroblastoma trials. |
| Oberthuer A, Warnat P, Kahlert Y, Westermann F, Spitz R, Brors B, Hero B, Eils R, Schwab M, Berthold F, Fischer M |
Classification of neuroblastoma patients by published gene-expression markers reveals a low sensitivity for unfavorable courses of MYCN non-amplified disease. |
Cancer letters 2007, 250: 250 |
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| Currently, Pubmed lists 385 marker genes for neuroblastoma outcome. Using a customized neuroblastoma-microarray, we evaluated the prognostic impact of the gene-expression pattern of 349 of these candidates (90.6%) in 127 neuroblastoma patients with divergent outcome. By significance analysis of microarrays (SAM) and both uncorrected and Bonferroni-corrected ANOVA, 166/349 (47.5%), 218/349 (62.5%) and 128/349 (36.4%) candidates showed significant differential expression between patients with contrasting outcome. By Prediction Analysis for Microarrays (PAM), a 38-gene-classifier was derived from all markers, which classified patients outcome with an overall accuracy of 78.5%. However, patients with unfavorable outcome of MYCN non-amplified disease were largely misclassified (accuracy: 35%), suggesting that these courses are not identified by current marker genes. |
| Oberthuer A, Kaderali L, Kahlert Y, Hero B, Westermann F, Berthold F, Brors B, Eils R, Fischer M |
Subclassification and individual survival time prediction from gene expression data of neuroblastoma patients by using CASPAR. |
Clinical cancer research : an official journal of the American Association for Cancer Research 2008, 14: 6590 |
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| PURPOSE: To predict individual survival times for neuroblastoma patients from gene expression data using the cancer survival prediction using automatic relevance determination (CASPAR) algorithm. EXPERIMENTAL DESIGN: A first set of oligonucleotide microarray gene expression profiles comprising 256 neuroblastoma patients was generated. Then, CASPAR was combined with a leave-one-out cross-validation to predict individual times for both the whole cohort and subgroups of patients with unfavorable markers, including stage 4 disease (n = 67), unfavorable genetic alterations, intermediate-risk or high-risk stratification by the German neuroblastoma trial, and patients predicted as unfavorable by a recently described gene expression classifier (n = 83). Prediction accuracy of individual survival times was assessed by Kaplan-Meier analyses and time-dependent receiver operator characteristics curve analyses. Subsequently, classification results were validated in an independent cohort (n = 120). RESULTS: CASPAR separated patients with divergent outcome in both the initial and the validation cohort [initial set, 5y-OS 0.94 +/- 0.04 (predicted long survival) versus 0.38 +/- 0.17 (predicted short survival), P < 0.0001; validation cohort, 5y-OS 0.94 +/- 0.07 (long) versus 0.40 +/- 0.13 (short), P < 0.0001]. Time-dependent receiver operator characteristics analyses showed that CASPAR-predicted individual survival times were highly accurate (initial set, mean area under the curve for first 10 years of overall survival prediction 0.92 +/- 0.04; validation set, 0.81 +/- 0.05). Furthermore, CASPAR significantly discriminated short (<5 years) from long survivors (>5 years) in subgroups of patients with unfavorable markers with the exception of MYCN-amplified patients (initial set). Confirmatory results with high significance were observed in the validation cohort [stage 4 disease (P = 0.0049), NB2004 intermediate-risk or high-risk stratification (P = 0.0017), and unfavorable gene expression prediction (P = 0.0017)]. CONCLUSIONS: CASPAR accurately forecasts individual survival times for neuroblastoma patients from gene expression data. |
| Oberthuer A, Theissen J, Westermann F, Hero B, Fischer M |
Molecular characterization and classification of neuroblastoma. |
Future oncology (London, England) 2009, 5: 625 |
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| For many decades, neuroblastoma has remained a challenging disease for both clinicians and researchers. Now, techniques that efficiently specify both comprehensive genetic and gene-expression alterations of neuroblastoma tumors have provided molecular markers that indicate tumor behavior and patient outcome with very high accuracy. Once the anticipated value of these markers has been confirmed in ongoing studies, patients may profit from more accurate risk assessment by integrating these markers into clinical routine. Moreover, disclosing further tumor-initiating events, such as the recently revealed oncogenic mutations of ALK, will further promote the elucidation of the genetic etiology of the disease. Together with recent information on altered signaling pathways in aggressively growing tumors, this knowledge will help to establish therapeutic strategies specifically targeting molecular key factors of neuroblastoma tumor progression. |
| Oberthuer A, Berthold F, Hero B, Till H |
Neuroblastome, in: Solide Tumoren im Kindesalter. Fuchs J (Hrsg.) |
Schattauer GmbH: Stuttgart 2012, 77 |
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| Oda Y, Jürgens H, Roessner A |
Expression of multidrug resistance-associated protein gene in Ewing's sarcoma and malignant peripheral neuroectodermal tumor of bone. |
J Cancer Res Clin Oncol 1997, 123: 237 |
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| Oehme I, Linke JP, Böck BC, Milde T, Lodrini M, Hartenstein B, Wiegand I, Eckert C, Roth W, Kool M, Kaden S, Gröne HJ, Schulte JH, Lindner S, Hamacher-Brady A, Brady NR, Deubzer HE, Witt O |
Histone deacetylase 10 promotes autophagy-mediated cell survival. |
Proc Natl Acad Sci U S A 2013, 110:E2592 |
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| Tumor cells activate autophagy in response to chemotherapy-induced DNA damage as a survival program to cope with metabolic stress. Here, we provide in vitro and in vivo evidence that histone deacetylase (HDAC)10 promotes autophagy-mediated survival in neuroblastoma cells. We show that both knockdown and inhibition of HDAC10 effectively disrupted autophagy associated with sensitization to cytotoxic drug treatment in a panel of highly malignant V-MYC myelocytomatosis viral-related oncogene, neuroblastoma derived-amplified neuroblastoma cell lines, in contrast to nontransformed cells. HDAC10 depletion in neuroblastoma cells interrupted autophagic flux and induced accumulation of autophagosomes, lysosomes, and a prominent substrate of the autophagic degradation pathway, p62/sequestosome 1. Enforced HDAC10 expression protected neuroblastoma cells against doxorubicin treatment through interaction with heat shock protein 70 family proteins, causing their deacetylation. Conversely, heat shock protein 70/heat shock cognate 70 was acetylated in HDAC10-depleted cells. HDAC10 expression levels in high-risk neuroblastomas correlated with autophagy in gene-set analysis and predicted treatment success in patients with advanced stage 4 neuroblastomas. Our results demonstrate that HDAC10 protects cancer cells from cytotoxic agents by mediating autophagy and identify this HDAC isozyme as a druggable regulator of advanced-stage tumor cell survival. Moreover, these results propose a promising way to considerably improve treatment response in the neuroblastoma patient subgroup with the poorest outcome. |
| Oevermann L, Firnkorn M, Michaelis S, Müller S, Schaeffeler E, Schrappe M, Cario G, Stanulla M, Schwab M, Handgretinger R, Mezger M |
No association between the presence of killer-cell immunoglobulin-like receptor genes and susceptibility to childhood ALL. |
Blood 2015, 125: 3355 |
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| Offenmueller S, Leiter U, Bernbeck B, Garbe C, Eigentler T, Borkhardt A, Classen CF, Corbacioglu S, Dirksen U, Ebetsberger-Dachs G, Heinzerling L, Jorch N, Kuhlen M, Lawlor J, Niggli F, Streiter M, Schneider DT, Brecht I |
Clinical characteristics and outcome of 60 pediatric patients with malignant melanoma registered with the German Pediatric Rare Tumor Registry (STEP). |
Klinische Padiatrie 2017, 229: 322 |
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| Malignant melanoma (MM) is a common malignancy in adults while it is rare in children. Thus, information on clinical behavior of pediatric MM is incomplete. The German Pediatric Rare Tumor Registry (STEP) presents a prospective analysis of 60 childhood MM cases diagnosed between June 2006 and December 2014. Patients' ages ranged between 0 and 17 years at initial diagnosis (median age 9.6 years). Information on patient's and tumor characteristics was obtained by standardized documentation. Three-year overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier test. Follow-up ranged from 0 to 116 months with a median of 36.5 months, however, univariate analysis was performed for 46 cases with a follow-up > 3 months, only. Cases with spitzoid histotype (40%) did not show a significantly different outcome compared to cases with non-spitzoid MM. Breslow thickness ≤ 2.00 mm was identified in 30% of the cases and 18% were Clark level I to III. Adjuvant therapy was used in 45% of cases. OS at 3 years was 100%, EFS 95.2%. We present a series of cases with a high number of spitzoid malignant melanoma and advanced pediatric melanoma, but surprisingly good overall survival rates. Spitzoid and non-spitzoid MM do not differ in clinical behavior and survival. |
| Ogawa S |
Clonal hematopoiesis in acquired aplastic anemia. |
Blood 2016 Jul 21; 128: 337 |
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| Okcu M, Munsell M, Treuner J, Mattke A, Pappo A, Cain A, Ferrari A, Casanova M, Ozkan A, Raney B |
Synovial sarcoma of childhood and adolescence. |
J Clin Oncol 2003, 21: 1602 |
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| Okpanyi V, Schneider DT, Zahn S, Sievers S, Calaminus G, Nicholson JC, Palmer RD, Leuschner I, Borkhardt A, Schönberger S |
Analysis of the adenomatous polyposis coli (APC) gene in childhood and adolescent germ cell tumors. |
Pediatric blood & cancer 2011, 56: 384 |
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| Aberrant Wnt signaling due to deregulation of Wnt regulators is implicated in the development and progression of numerous embryonal tumors. This study addresses the questions if activation of Wnt signaling in germ cell tumors (GCTs) arising during childhood and adolescence is associated with aberrations of the tumor suppressor adenomatous polyposis coli (APC), and whether APC aberrations might be responsible for progression from benign teratoma to malignant yolk sac tumor (YST). |
| Oldenburg J, Barthels M |
Angeborene Koagulopathien am Beispiel der Hämophilie A und B, Hemmkörperhämophilie. |
Hämostaseologie 2008, 28: 335 |
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| Es wird eine Übersicht über die Klinik und Therapie angeborener Koagulopathien am Beispiel der Hämophilie A und B gegeben. Diese Übersicht umfasst das heutige Krankheitsbild der behandelten Hämophilie, Strategien und Empfehlungen bei der Diagnostik und Therapie. Von besonderer Bedeutung ist die Pathogenese der hämophilen Gelenkarthropathie als wichtigste Folgeerkrankung der Hämophilie. Sichere aus Plasma oder rekombinant hergestellte Gerinnungsfaktorkonzentrate erlauben heute eine wirksame Blutungsbehandlung und auch eine prophylaktische Therapie zur präventiven Vermeidung von Blutungen und deren Folgen. Hierdurch haben sich Lebensqualität und Lebenserwartung von heute geborenen Hämophilepatienten weitgehend normalisiert. Die Hemmkörperentwicklung zu Beginn der Substitutionstherapie mit Faktor-VIII-Konzentraten stellt die heutzutage schwerwiegendste Komplikation der Hämophiliebehandlung dar. Die einzige Möglichkeit den Hemmkörper dauerhaft zu eradizieren ist die Induktion einer Immuntoleranz, für die verschiedene Protokolle publiziert sind. Die erfolgreiche Immmuntoleranztherapie (ITT) ist Voraussetzung, um dem Hämophilen ein reguläres prophylaktisches Therapieregime zu ermöglichen. Die Querschnittsleitlinien der Bundesärztekammer, die Deutschen Empfehlungen zur ITT und die Konsensusempfehlungen einer internationalen Arbeitsgruppe geben wichtige Anhaltspunkte für die Durchführung der ITT. |
| Oldenburg J, Barthels M |
Congenital coagulopathies and coagulation factor inhibitors. |
Hämostaseologie 2008, 18 ; 255 |
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| An overview is given on the clinical picture and therapy of the haemophilias A and B as an example for congenital coagulopathies. The survey deals with the special problems of today's „treated haemophilia” and its recommendations on diagnosis and treatment regimens. A special focus is put on the pathogenesis of the haemophilic joint arthropathy, that is affecting most haemophiliacs earlier or later during life. Safe plasma derived and recombinant clotting factor concentrates allow an efficient on-demand and also a prophylactic treatment for the prevention of bleeding sequelae. As a consequence life quality and life expectancy of today born haemophiliacs have nearly normalised. The problems of haemophilia with inhibitors and future therapeutic options are discussed. The development of inhibitors at start of therapy with factor VIII concentrates represent the nowadays most serious complication of haemophilia treatment. The only way to a long lasting eradication of the inhibitor ist the induction of immune tolerance that can be achieved by various protocols. A successful immune tolerance therapy (ITT) is the precondition for a regular prophylactic treatment regime. The Guidelines of the Federal Chamber of Physicians , the German recommendations for ITT and the consensus recommendations of an international working group provide an important lead for the conduction of immune tolerance therapy. |
| Oommen PT, Linden T, Romahn A, Bucsky P |
UICC-2002 TNM-classification is not suitable for differentiated thyroid carcinoma in children and adolescent - interim results of GPOH-MET 97 trial. |
Pediatric Blood & Cancer 2005, 45: 482 P.D. 111 |
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| Oommen PT, Romahn A, Linden T, Frühwald MC, Bucsky P |
UICC-2002 TNM classification is not suitable for differentiated thyroid cancer in children and adolescents. |
Pediatr Blood Cancer 2008, 50: 1159-62. |
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| BACKGROUND: Recently the UICC-TNM classification for differentiated thyroid cancer (DTC) was changed neglecting the special circumstances for children affected by the disease. While the 1997 TNM classification grouped tumours |
| Opocher E, Kremer LC, Da Dalt L, van de Wetering MD, Viscardi E, Caron HN, Perilongo G |
Prognostic factors for progression of childhood optic pathway glioma: a systematic review. |
European journal of cancer 2006, 42: 1807 |
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| A systematic literature review was carried out to evaluate best existing evidence on prognostic factors for progression of childhood optic pathway glioma. Databases were searched for relevant articles and articles selected independently by two authors. Information about study design, population, treatment, outcome and prognostic analysis were abstracted and the quality of each article was assessed. A total of 23 articles met the inclusion criteria. Many studies had important methodological limitations, regarding external and internal validity. Eleven studies evaluated possible prognostic factors in a multivariate analysis. Three high-quality studies indicated age<1 year as an independent prognostic factor for a worse progression-free survival. Three studies with multivariate analysis, including one high-quality study, found that children with neurofibromatosis type 1 (NF-1) have a better progression-free survival than those without NF-1. Two studies with multivariate analysis found tumour site to be a prognostic factor, both with some methodological limitations. In conclusion, this systematic review demonstrates that only a few of the prognostic factors proposed have been proven to be clinically relevant. Age<1 year is a clear and independent prognostic factor for progression-free survival. Other prognostic factors, such as NF-1, tumour site and others, are suggested, but are still without solid evidence and need further high-quality studies to be clearly proven. |
| Øra I, Eggert A |
Progress in treatment and risk stratification of neuroblastoma: impact on future clinical and basic research. |
Seminars in cancer biology 2011, 21: 217 |
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| Close international collaboration between pediatric oncologists has led to marked improvements in the cure of patients, seen as a long-term overall survival rate of about 80%. Despite this progress, neuroblastoma remains a challenging disease for both clinicians and researchers. Major clinical problems include lack of acceptable cure rates in high-risk neuroblastoma and potential overtreatment of subsets of patients at low and intermediate risk of the disease. Many years of intensive international cooperation have recently led to a promising joint effort to further improve risk classification for treatment stratification, the new International Neuroblastoma Risk Group Classification System. This approach will facilitate comparison of the results of clinical trials performed by different international collaborative groups. This, in turn, should accelerate refinement of risk stratification and thereby aid selection of appropriate therapies for individual patients. To be able to identify new therapeutic modalities, it will be necessary to elucidate the pathogenesis of the different subtypes of neuroblastoma. Basic and translational research have provided new tools for molecular characterization of blood and tumor samples including high-throughput technologies for analysis of DNA, mRNAs, microRNAs and other non-coding RNAs, as well as proteins and epigenetic markers. Most of these studies are array-based in design. In neuroblastoma research they aim to refine risk group stratification through incorporation of molecular tumor fingerprints and also to enable personalized treatment modalities by describing the underlying pathogenesis and aberrant signaling pathways in individual tumors. To make optimal use of these new technologies for the benefit of the patient, it is crucial to have a systematic and detailed documentation of both clinical and molecular data from diagnosis through treatment to follow-up. Close collaboration between clinicians and basic scientists will provide access to combined clinical and molecular data sets and will create more efficient steps in response to the remaining treatment challenges. This review describes the current efforts and trends in neuroblastoma research from a clinical perspective in order to highlight the urgent clinical problems we must address together with basic researchers. |
| Orbach D, Sparber-Sauer M, Laetsch TW, Minard-Colin V, Bielack SS, Casanova M, Corradini N, Koscielniak E, Scheer M, Hettmer S, Bisogno G, Hawkins DS, Ferrari A |
Spotlight on the treatment of infantile fibrosarcoma in the era of neurotrophic tropomyosin receptor kinase inhibitors: International consensus and remaining controversies. |
European journal of cancer 2020, 137: 183 |
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| Targeted neurotrophic tropomyosin receptor kinase (TRK) inhibitors offer a highly specific therapeutic option for patients with infantile fibrosarcoma (IFS) carrying the NTRK gene translocation. International recommendations are needed to define the role of TRK inhibitors (TRKI) for infants with IFS. We analysed retrospective data for all published patients with IFS in the European Paediatric Soft tissue sarcoma Study Group and Cooperative Weichteilsarkomstudiengruppe (CWS) experience and developed a consensus strategy with the Children's Oncology Group. Therapies consisted of tumour resection and/or perioperative chemotherapy for extensive tumours. Among the 172 European patients treated, 162 were alive at the end of the follow-up. Sixty-five patients (40% of all survivors) were treated with surgery alone and 64 patients (39%) with surgery combined with chemotherapy. Radiotherapy was delivered to 3% of survivors (five patients). In addition, 28 survivors (17%) exclusively received chemotherapy. Among the 129 patients treated with surgery, 91% had conservative surgery (118 cases). Overall, nine patients died of disease, one from toxicity (6%) and 20 patients (12%) survived with major functional deficits or had mutilating surgery. Overall, conventional conservative strategies before the era of targeted therapy, even in the case of extensive tumours, demonstrate efficacy in IFS, but are associated with acute and some chronic side effects. TRKI have demonstrated very rapid responses in the vast majority of children with IFS with limited acute toxicity. With the current state of our knowledge, both conventional chemotherapy and TRKI should be regarded as options for patients with localised disease at the physician's and parent's discretion. TRKI should be considered in patients with metastatic disease, and before mutilating surgery when conventional chemotherapy fails. Outside a clinical trial, additional data are needed to resolve the lack of consensus about front-line use of conventional chemotherapy versus TRKI in patients with localised disease. |
| Orbach D, André N, Brecht IB, López Almaraz R, Ben-Ami T, Vermersch S, Carton M, Virgone C, Bisogno G, Schneider DT, Bajciova V, Reguerre Y, Galateau-Salle F, Stachowicz-Stencel T, Dvir R, Rees H, Bien E, Ferrari A, Ben Arush M |
Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. |
European journal of cancer 2020, 140: 63 |
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| Very little is known about the characteristics of mesothelial tumours in the paediatric population. In adults with malignant mesothelioma, the pemetrexed-based regimen with cytoreductive surgery (CRS) is a standard of care in limited tumours, but long-term survival is uncommon. |
| Orbach D, Ferrari A, Schneider DT, Reguerre Y, Godzinski J, Bien E, Stachowicz-Stencel T, Surun A, Almaraz RL, Dragomir M, Jani D, Ami TB, Roganovic J, Brecht IB, Ladenstein R, Bisogno G |
The European Paediatric Rare Tumours Network - European Registry (PARTNER) project for very rare tumors in children. |
Pediatric blood & cancer 2021, 68 Suppl 4:e29072 |
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| The PARTNER project (Paediatric Rare Tumours Network - European Registry) was launched in 2016. PARTNER aims to create a European Registry dedicated to children and adolescents with very rare tumors (VRT). It links existing national registries and provides a registry for those countries in which a VRT registry has not yet been created. This consortium is composed of the various national cooperative groups and their respective member institutions. The strategic value of this project is based on the Europe-wide data collection concerning the treatment of VRTs. These data are provided to experts and constitute the basis for new clinical practice guidelines for use by ERN (European Reference Network) and non-ERN institutions. The proposed tasks and milestones will increase collaboration in the field of pediatric oncology among member states and will also facilitate the inclusion of low health expenditure average rate (LHEAR) countries in this process. In addition, this project creates a platform for VRTs that may represent a model on how to elaborate a comprehensive approach (case registration, international case consultation and treatment recommendations, and website to provide information for parents/patients) for rare diseases. |
| Origa R, Sollaino MC, Borgna-Pignatti C, Piga A, Feliu Torres A, Masile V, Galanello R |
α-Globin Gene Quadruplication and Heterozygous β-Thalassemia: A Not So Rare Cause of Thalassemia Intermedia. |
Acta haematologica 2013, 131: 162 |
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| Orr LC, Fleitz J, McGavran L, Wyatt-Ashmead J, Handler M, Foreman NK |
Cytogenetics in pediatric low-grade astrocytomas. |
Med Pediatr Oncol 2002, 38: 173 |
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| Oschlies I, Klapper W, Zimmermann M, Krams M, Wacker HH, Burkhardt B, Harder L, Siebert R, Reiter A, Parwaresch R |
Diffuse large B-cell lymphoma in pediatric patients belongs predominantly to the germinal-center type B-cell lymphomas: a clinicopathologic analysis of cases included in the German BFM (Berlin-Frankfurt-Münster) Multicenter Trial. |
Blood 2006, 107: 4047 |
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| Ostertag CB |
Stereotactic interstitial radiotherapy for brain tumors. |
J Neurosurg Sci 1989, 33: 83 |
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| Ottinger HD et al. |
Empfehlungen zur immungenetischen Spenderauswahl für die allogene Transplantation von Knochenmark und peripheren Blutstammzellen. |
Leitlinien und Empfehlungen der Deutschen Gesellschaft für Immungenetik 1999 |
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| Otte JB, Pritchard J, Aronson DC, Brown J, Czauderna P, Maibach R, Perilongo G, Shafford E, Plaschkes J, International Society of Pediatric Oncology (SIOP) |
Otte JB, Pritchard J, Aronson DC, et al.: Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience. |
Pediatr Blood Cancer 2004, 42: 74-83. |
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| BACKGROUND: For hepatoblastoma (HB) that remains unresectable by partial hepatectomy after chemotherapy, total hepatectomy with orthotopic liver transplantation (LTX) has been advocated as the best treatment option. The role of LTX in the overall management of HB is still, however, unclear.
PROCEDURE: The results of LTX from the first study of HB by the International Society of Pediatric Oncology, SIOPEL-1, were analyzed. In addition, the world experience of LTX for HB was extensively reviewed. Twelve patients in the SIOPEL-1 study underwent a LTX. Median (range) follow-up at Dec. 31, 2001 was 117 months (52-125) since LTX.
RESULTS: Overall survival at 10 years post-LTX was 85% for the seven children who received a "primary LTX" and 40% for the 5 children who underwent a "rescue LTX" after previous partial hepatectomy. In the world experience (147 cases), the overall survival rate at 6 year post-LTX was 82% for 106 patients who received a "primary LTX" and 30% for 41 patients who underwent a "rescue LTX." Multivariate analysis of patients undergoing primary LTX showed that only macroscopic venous invasion had a significant impact (P-value: 0.045 with a hazard ratio of 2.96) on overall survival.
CONCLUSIONS: Orthotopic LTX has added a new dimension to the treatment of HB unresectable by partial hepatectomy. Because of the rarity of the disease and to optimize results, children with extensive HB should be treated in centers with surgical expertise in pediatric major liver resection and LTX, in close collaboration with pediatric oncologists, radiologists, and histopathologists. |
| Ottensmeier H, Zimolong B, Wolff JE, Ehrich J, Galley N, von Hoff K, Kuehl J, Rutkowski S |
Neuropsychological short assessment of disease- and treatment-related intelligence deficits in children with brain tumours. |
European journal of paediatric neurology 2015, Epub ahead of print |
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| The Wuerzburger Psychologische Kurz-Diagnostik (WUEP-KD) is a short screening battery for cognitive deficits in children with brain tumour. We report on its psychometric quality and testing efficiency. |
| Ottensmeier H, Schlegel PG, Eyrich M, Wolff JE, Juhnke BO, von Hoff K, Frahsek S, Schmidt R, Faldum A, Fleischhack G, von Bueren A, Friedrich C, Resch A, Warmuth-Metz M, Krauss J, Kortmann RD, Bode U, Kühl J, Rutkowski S |
Treatment of children under 4 years of age with medulloblastoma and ependymoma in the HIT2000/HIT-REZ 2005 trials: Neuropsychological outcome 5 years after treatment. |
PloS one 2020, 15:e0227693 |
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| Young children with brain tumours are at high risk of developing treatment-related sequelae. We aimed to assess neuropsychological outcomes 5 years after treatment. This cross-sectional study included children under 4 years of age with medulloblastoma (MB) or ependymoma (EP) enrolled in the German brain tumour trials HIT2000 and HIT-REZ2005. Testing was performed using the validated Wuerzburg Intelligence Diagnostics (WUEP-D), which includes Kaufman-Assessment-Battery, Coloured Progressive Matrices, Visual-Motor Integration, finger tapping "Speed", and the Continuous Performance Test. Of 104 patients in 47 centres, 72 were eligible for analyses. We assessed whether IQ was impacted by disease extent, disease location, patient age, gender, age at surgery, and treatment (chemotherapy with our without craniospinal irradiation [CSI] or local radiotherapy [LRT]). Median age at surgery was 2.3 years. Testing was performed at a median of 4.9 years after surgery. Patients with infratentorial EPs (treated with LRT) scored highest in fluid intelligence (CPM 100.9±16.9, mean±SD); second best scores were achieved by patients with MB without metastasis treated with chemotherapy alone (CPM 93.9±13.2), followed by patients with supratentorial EPs treated with LRT. In contrast, lowest scores were achieved by patients that received chemotherapy and CSI, which included children with metastasised MB and those with relapsed MB M0 (CPM 71.7±8.0 and 73.2±21.8, respectively). Fine motor skills were reduced in all groups. Multivariable analysis revealed that type of treatment had an impact on IQ, but essentially not age at surgery, time since surgery or gender. Our results confirm previous reports on the detrimental effects of CSI in a larger cohort of children. Comparable IQ scores in children with MB treated only with chemotherapy and in children with EP suggest that this treatment strategy represents an attractive option for children who have a high chance to avoid application of CSI. Longitudinal follow-up examinations are warranted to assess long-term neuropsychological outcomes. |
| Ouachée-Chardin M, Elie C, de Saint Basile G, Le Deist F, Mahlaoui N, Picard C, Neven B, Casanova JL, Tardieu M, Cavazzana-Calvo M, Blanche S, Fischer A |
Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients. |
Pediatrics 2006, 117:e743 |
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| OBJECTIVES: Familial hemophagocytic lymphohistiocytosis (FHLH) is a genetically determined disorder characterized by the early onset of fever, hepatosplenomegaly, central nervous system disease, thrombocytopenia, coagulation disorders, and hemophagocytosis. It is caused by genetic defects that impair T cell-mediated and natural cytotoxicity. Chemotherapy- or immunotherapy-based treatments can achieve remission. Hematopoietic stem cell transplantation (HSCT), however, is the only curative option, but optimal modalities and long-term outcome are not yet well known. METHODS: We retrospectively analyzed the outcome of HSCT that was performed in 48 consecutive patients who had FHLH and were treated in a single center between 1982 and 2004. RESULTS: The overall survival was 58.5% with a median follow-up of 5.8 years and extending to 20 years. A combination of active disease and haploidentical HSCT had a poor prognosis because in this situation, HLH disease is more frequently associated with graft failure. Twelve patients received 2 transplants because of graft failure (n = 7) or secondary graft loss that led to HLH relapse (n = 5). Transplant-related toxicity essentially consisted in veno-occlusive disease, which occurred in 28% of transplants and was associated with young age, haploidentical transplantation, and the use of antithymocyte globulin (ATG) in the conditioning regimen. A sustained remission was achieved in all patients with a donor chimerism > or = 20% of leukocytes. Long-term sequelae were limited, because only 2 (7%) of 28 patients experienced a mild neurologic disorder. CONCLUSIONS: This survey demonstrates the long-term efficacy of HSCT as a cure of FHLH. HSCT preserves quality of life. It shows that HSCT should be performed as early as a complete remission has been achieved. Additional studies are required to improve the procedure and reduce its toxic effects. |
| Ozaki T, Lindner N, Hoffmann C, Hillmann A, Rödl R, Blasius S, Link T, Winkelmann W, Jürgens H |
Ewing's sarcoma of the ribs. A report from the cooperative Ewing's sarcoma study. |
Eur J Cancer 1995, 31A: 2284 |
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| Ozaki T, Hillmann A, Hoffmann C, Rübe C, Blasius S, Dunst J, Jürgens H, Winkelmann W |
Significance of surgical margin on the prognosis of patients with Ewing's sarcoma. A report from the Cooperative Ewing's Sarcoma Study. |
Cancer 1996, 78: 892 |
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| Ozaki T, Hillmann A, Hoffmann C, Rübe C, Blasius S, Dunst J, Treuner J, Jürgens H, Winkelmann W |
Ewing's sarcoma of the femur - Prognosis in 69 patients treated by the CESS group. |
Acta Orthop Scand 1997, 68: 20 |
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| Ozaki T, Hillmann A, Rübe C, Rödl R, Hein M, Hoffmann C, Blasius S, Jürgens H, Winkelmann W |
The impact of intraoperative brachytherapy on surgery of Ewing's sarcoma. |
J Cancer Res Clin Oncol 1997, 123: 53 |
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| Ozaki T, Winkelmann W, Willich N, Jürgens H |
Treatment of Ewing's sarcoma in the Cooperative Ewing's Sarcoma Study group. |
J Orthop Sci 1997, 2: 180 |
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| Ozaki T, Paulussen M, Poremba C, Brinkschmidt C, Rerin J, Ahrens S, Hoffmann C, Hillmann A, Wai D, Schaefer K, Boecker W, Jürgens H, Winkelmann W, Dockhorn-Dworniczak B |
Genetic imbalances revealed by comparative genomic hybridization in Ewing tumors. |
Genes Chromosomes Cancer 2001, 32: 164 |
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| Ozaki T, Flege S, Liljenqvist U, Hillmann A, Delling G, Salzer-Kuntschik M, Jürgens H, Kotz R, Winkelmann W, Bielack S |
Osteosarcoma of the spine. |
Cancer 2002, 94: 1069 |
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| Ozaki T, Schaefer K, Wai D, Buerger H, Flege S, Lindner N, Kevric M, Diallo R, Bankfalvi A, Brinkschmidt C, Jürgens H, Winkelmann W, Dockhorn-Dworniczak B, Bielack S, Poremba C |
Genetic imbalances revealed by comparative genomic hybridization in osteosarcomas. |
Int J Cancer 2002, 102: 355 |
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| Ozaki T, Flege S, Kevric M, Lindner N, Maas R, Delling G, Schwarz R, von Hochstetter A, Salzer-Kuntschik M, Berdel W, Jürgens H, Exner G, Reichardt P, Mayer-Steinacker R, Ewerbeck V, Kotz R, Winkelmann W, Bielack S |
Osteosarcoma of the pelvis. |
J Clin Oncol 2003, 21: 334 |
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| Ozyurt J, Lorenzen A, Gebhardt U, Warmuth-Metz M, Müller HL, Thiel CM |
Remote effects of hypothalamic lesions in the prefrontal cortex of craniopharygioma patients. |
Neurobiology of learning and memory 2014, 111: 71 |
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| Albeit histologically low grade (WHO I(o)) brain tumors, craniopharyngiomas and/or their surgical removal frequently affect the hypothalamus, amongst other brain regions at risk. Due to rich hypothalamic connections with prefrontal and limbic regions, hypothalamic injury may adversely affect neural substrates of emotion processing and higher cognitive control, including memory and executive functions. The current study is the first to investigate the consequences of hypothalamic involvement on neural substrates of emotional and cognitive functioning. Ten patients with childhood craniopharyngioma and known hypothalamic involvement and fifteen age- and intelligence matched control subjects (median age: 17.8 and 17.3 yrs.) were studied with functional magnetic resonance imaging and an emotional face recognition task. During encoding, participants were asked to classify neutral and emotional faces. In a subsequent recognition phase, participants had to recognize these old faces within a set of new faces. Behavioral performance was comparable between patients and controls. Neural activity revealed, however, differential recruitment of fronto-limbic brain regions during recognition. Patients exhibited an abnormal pattern of task-induced activation and deactivation in the anterior and posterior rostral medial prefrontal cortex and a higher functional coupling between anterior rostral medial prefrontal cortex and the thalamus. Additionally, we found a higher reactivity in the patients' amygdala to emotional relative to neutral faces when compared to healthy controls. Our data provide first evidence that hypothalamic damage impacts neural correlates of memory retrieval in medial prefrontal cortex, indicating a less efficient use of an area involved in executive control processes. We propose that the deactivation failure in the patients' anterior rostral medial prefrontal cortex is related to an increased coupling with the thalamus and reflects a reduced efficiency to flexibly adapt to task demands. |
| Özyurt J, Thiel CM, Lorenzen A, Gebhardt U, Calaminus G, Warmuth-Metz M, Müller HL |
Neuropsychological outcome in patients with childhood craniopharyngioma and hypothalamic involvement. |
The Journal of pediatrics 2014, 164: 876-881.e4 |
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| To test memory performance and executive functions in patients with childhood craniopharyngioma and hypothalamic involvement. |
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