| Autor(en) |
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| Faivre L, Meerpohl J, Da Costa L, Marie I, Nouvel C, Gnekow A, Bender-Gotze C, Bauters F, Coiffier B, Peaud, PY, Rispal P, Berrebi A, Berger C, Flesch M, Sagot P, Varet B, Niemeyer C, Tchernia G, Leblanc, T |
High-risk pregnancies in Diamond-Blackfan anemia: a survey of 64 pregnancies from the French and German registries. [+] |
Haematologica 2006, 91: 530-3. Epub 2006 Mar 15. |
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| We reviewed 64 pregnancies in 26 women with Diamond-Blackfan anemia (DBA) included in the French and German DBA registries. Complications were seen in 42 pregnancies (66%) and included abortion, pre-eclampsia, in utero fetal death, intrauterine growth retardation, retroplacental hematoma, pre-term delivery and fetal malformations. Of the 34 children (53%) born alive, 13 had DBA. No correlations were found between pregnancy outcome and features of either maternal or child DBA. Pregnancies in DBA-affected women are at high risk, especially for complications likely to be of vascular-placental origin. Careful monitoring with prevention of severe anemia and early introduction of aspirin is suggested. |
| Fanconi G |
Familiäre infantile perniziosaartige Anämie (perniziöses Blutbild und Konstitution). |
Jahrb Kinderheilk 1927,: 257 |
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| Fanconi G |
Familial constitutional panmyelocytopathy, Fanconi's anemia (F. A.). I. Clinical aspects. |
Semin Hematol 1967, 4: 233 |
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| Farahati J, Bucsky P, Parlowsky T, Mader U, Reiners C |
Characteristics of differentiated thyroid carcinoma in children and adolescents with respect to age, gender, and histology. |
Cancer 1997, 80: 2156 |
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| Farahati J, Parlowsky T, Mader U, Reiners C, Bucsky P |
Differentiated thyroid cancer in children and adolescents. |
Langenbecks Arch Surg 1998, 383: 235 |
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| Favrot M, Ambros P, Schilling F, Frappaz D, Combaret V, Berthold F, Dominici C, Erttmann R, Esteve J, Jenkner A, Kerbl R, Mann J, Mathieu P, Parker L, Powell J, Philip T |
Comparison of the diagnostic and prognostic value of biological markers in neuroblastoma. Proposal for a common methodology of analysis. SENSE group. |
Ann Oncol 1996, 7: 607 |
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| Favara B, Feller A, Pauli M, Jaffe E, Weiss L, Arico M, Bucsky P, Egeler R, Elinder G, Gadner H, Gresik M, Henter J, Imashuku S, Janka-Schaub G, Jaffe R, Ladisch S, Nezelof C, Pritchard J |
Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. |
Med Pediatr Oncol 1997, 29: 157 |
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| Fazekas T, Eickhoff P, Lawitschka A, Knotek B, Pötschger U, Peters C |
Exhaled nitric oxide and pulmonary complications after paediatric stem cell transplantation. [+] |
European journal of pediatrics 2012 Feb 16; |
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| Pulmonary complications are major causes of morbidity and mortality after haematopoietic stem cell transplantation (HSCT). We hypothesise that elevated exhaled nitric oxide (FeNO) levels early after HSCT in children are predictive for pulmonary complications. The present prospective study included 30 children (age, 4-18 years) before HSCT. FeNO levels were evaluated 10 days before transplant, at day 0, day +28 and day +60 after HSCT. During the follow-up period until day +100, pulmonary complications and lung function were assessed. Before HSCT, the mean FeNO levels were comparable in children with or without post-transplant pulmonary complications. However, they differed at day 0 and day +28 with a mean of 7 (±1.95) and 13 (±3.44) ppb at day 0 and a mean of 13 (±3.44) and 14 (±3.57) ppb at day +28, respectively. Conclusion: Children with pulmonary complications after day +28 have higher mean FeNO levels 28 days after HSCT than children without later pulmonary complications. Therefore, FeNO could be an important diagnostic tool for hyperinflammatory response in bronchial epithelium after paediatric HSCT. |
| Fazekas T, Attarbaschi A, Lawitschka A, Seidel M, Pötschger U, Peters C, Mann G, Gadner H, Matthes-Martin S |
Lethal pulmonary complications after pediatric allogeneic hematopoietic stem cell transplantation. [+] |
The Pediatric infectious disease journal 2012, 31: 115 |
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| Hematopoietic stem cell transplantation (HSCT) in children is accompanied by a transplant-related mortality of 10% to 30%, which is the result of lethal pulmonary complications (LPCs) in many cases. |
| Fengler R, Baumgarten E, Buchmann S, Creutzig U, Harbott J, Ludwig R, Henze G |
Biklonale Leukämie (O-ALL/AMoL) mit 11;19 Translokation und Trisomie X bei einem 8 Monate alten Mädchen. |
Klinische Pädiatrie 1986, 198: 178 |
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| Ferrari A, Casanova M, Bisogno G, Mattke A, Meazza C, Gronchi A, Cecchetto G, Fidani P, Kunz D, Treuner J, Carli M |
Hemangiopericytoma in pediatric ages. |
Cancer 2001, 92: 2692 |
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| Ferris Tortajada J, Garcia Castell J, Berbel Tornero O, Clar Gimeno S |
Risk factors for non-Hodgkin’s lymphomas. |
An Esp Pediatr 2001, 55: 230 |
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| Ferris Tortajada J, Garcia Castell J, Lopez Andreu JA, Clar Gimeno S, Berbel Tornero O |
Risk factors for Hodgkin's lymphomas. |
An Esp Pediatr 2001, 55: 239 |
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| Ferrari A, Bisogno G, Casanova M, Meazza C, Piva L, Cecchetto G, Zanetti I, Pilz T, Mattke A, Treuner J, Carli M |
Paratesticular rhabdomyosarcoma. |
J Clin Oncol 2002, 20: 449 |
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| Ferrari A, Casanova M, Bisogno G, Cecchetto G, Meazza C, Gandola L, Garaventa A, Mattke A, Treuner J, Carli M |
Malignant vascular tumors in children and adolescents. |
Med Pediatr Oncol 2002, 39: 109 |
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| Ferrari A, Casanova M, Bisogno G, Mattke A, Meazza C, Gandola L, Sotti G, Cecchetto G, Harms D, Koscielniak E, Treuner J, Carli M |
Clear cell sarcoma of tendons and aponeuroses in pediatric patients. |
Cancer 2002, 94: 3269 |
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| Ferrari A, Bisogno G, Casanova M, Brecht I, Alaggio R, Cecchetto G, Provenzi M, Koscielniak E, Treuner J, Carli M |
Is alveolar histotype a prognostic factor in paratesticular rhabdomyosarcoma? |
Pediatr Blood Cancer 2004, 42: 134 |
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| Ferrari A, Casanova M, Bisogno G, Carli M, Treuner J |
What chemotherapy should alveolar paratesticular rhabdomyosarcoma receive? |
Pediatr Blood Cancer 2004, 43: 295 |
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| Ferrari A, Brecht IB, Koscielniak E, Casanova M, Scagnellato A, Bisogno G, Alaggio R, Cecchetto G, Catania S, Meazza C, Int-Veen C, Kirsch S, Dantonello T, Carli M, Treuner J |
The role of adjuvant chemotherapy in children and adolescents with surgically resected, high-risk adult-type soft tissue sarcomas. [+] |
Pediatric blood & cancer 2005, 45: 128 |
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| PURPOSE: This analysis evaluates whether adjuvant chemotherapy can be recommended for high-risk, surgically-resected, adult-type non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) within the new European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol. The Italian and German Cooperative Groups reviewed their data-bases, analyzing patients classified as group I-II, with high-grade tumor (G3) larger than 5 cm in size. METHODS: The analysis included 36 patients, and compared the clinical features and outcome of the group of 21 patients who received chemotherapy versus the group of 15 patients treated with local therapies only. RESULTS: For the series as a whole, 5-year event-free survival (EFS), metastasis-free survival (MFS), and overall survival (OS) were 26.2%, 34.0%, and 37.5%, respectively. In patients treated with chemotherapy, MFS and OS were 49.5% and 41.5% (median time to relapse: 13 months). In patients who did not receive chemotherapy, MFS and OS were 0% and 23.8% (median time to relapse: 3 months). CONCLUSION: The role of adjuvant chemotherapy in NRSTS is still uncertain, however, the current retrospective analysis showed that: (1) despite the globally good prognosis of grossly-resected cases, patients with G3 and large-size have a high-risk of metastatic spread, and (2) MFS appears to be better in patients who had chemotherapy. Based in part on these results, and in accordance with recent suggestions coming from the literature on adult sarcomas, the EpSSG NRSTS protocol will recommend adjuvant chemotherapy in high-risk surgically-resected patients. |
| Ferrari A, Bisogno G, De Salvo GL, Indolfi P, Perilongo G, Cecchetto G, Italian Study on Rare Tumours in Paediatric Age (TREP),Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) |
The challenge of very rare tumours in childhood: the Italian TREP project. [+] |
European journal of cancer 2007, 43: 654 |
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| A national cooperative project on rare paediatric tumours (the TREP project) was launched in 2000 in Italy, with a view to improving the clinical management and the basic research on these 'orphan' tumours, defined as those childhood solid malignancies characterised by an annual incidence <2/million and not considered in other clinical trials. This paper describes the process that the group developed and the problems it had to face, and aims to stimulate a debate on the rationale, scientific relevance and feasibility of running scientific research programs on rare childhood neoplasms. In the first phase of its activity, the project developed diagnostic and therapeutic recommendations for each rare tumour and established a collaborative network between 'experts' dedicated to each histotype and other specialists (i.e. adult oncologists, surgeons) involved in the management of these tumours. From 2000 to 2005, 297 patients have been registered from 35 Italian centres. This experience demonstrates the feasibility of a national multidisciplinary cooperation on rare paediatric malignancies and suggests that international studies could be realised. |
| Feuchtinger T |
Susceptibility to childhood leukemia. |
Blood 2011 118: 1189 |
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| Fichtner I, Lemm M, Becker M, Berthold F |
Effects of amifostine (WR-2721, ethyol) on tumor growth and pharmacology of cytotoxic drugs in human xenotransplanted neuroblastomas. |
Anticancer Drugs 1997, 8: 174 |
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| Fichtner I, Paal K, Borgmann A, Badiali L, Wurm R, Henze G |
Chemo- and radiation sensitivity of xenografted acute lymphoblastic leukemias--correlation to the expression of multidrug resistance proteins. |
Anticancer Res 2003, 23: 2657 |
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| Filipovich AH, Mathur A, Kamat D, Shapiro RS |
Primary inmunodeficiencies: genetic risk factors for lymphoma. |
Cancer Res 1992, 52: 5465 |
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| Filipovich AH |
Life-threatening hemophagocytic syndromes: current outcomes with hematopoietic stem cell transplantation. [+] |
Pediatric transplantation 2005, 9 Suppl 7: 87 |
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| Life-threatening hemophagocytic syndromes represent a subset of genetic disorders of inflammation. Many are rapidly lethal and can only be definitively treated at the present time with allogeneic hematopoietic stem cell transplantation (HSCT). In this report, current results with allogeneic transplantation for Hemophagocytic Lymphohistiocytosis (HLH) are described. HLH typically presents symptomatically during infancy and early childhood and can be identified by a constellation of numerous physical findings and laboratory tests indicative of overwhelming inflammation. The majority of patients with familial HLH lack natural killer (NK) cell function; in approximately 50% of cases the specific underlying genetic cause can now be discerned. Effective treatment consists of initial combination therapy with proapoptotic chemotherapy (typically etoposide) and anti-inflammatory therapies (principally steroids) in addition to aggressive supportive care, followed by allogeneic HSCT from the best available donor. Over the past 25 yr, through collaborative worldwide efforts, survival of children with HLH and related disorders has improved from 5% at 1 yr after diagnosis to greater than 50% 3-5 yr after diagnosis. |
| Finger PT, Czechonska G, Demirci H, Rausen A |
Chemotherapy for retinoblastoma: a current topic. [+] |
Drugs 1999, 58: 983 |
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| Retinoblastoma is the most common primary intraocular tumour in children, with an incidence of 1 in 15,000 live births. Treatment strategies for retinoblastoma have gradually evolved over the past few decades. There has been a trend away from enucleation (removal of the eye) and external beam radiation therapy toward focal 'conservative' treatments. Every effort has been made to save the child's life with preservation of eye and sight, if possible. Primary enucleation continues to be the commonly used method of treatment for retinoblastoma. It is employed in situations where eyes contain large tumours, long standing retinal detachments, neovascular glaucoma and suspicion of optic nerve invasion or extrascleral extension. Most of these eyes either have or are expected to have no useful vision. Radiation therapy continues to be an effective treatment option for retinoblastoma. However, external beam radiotherapy has unfortunately been associated with secondary non-ocular cancers in the field of radiation (primarily in children carrying the RB-1 germline mutation). Ophthalmic plaque brachytherapy has a more focal and shielded radiation field, and may carry less risk. Unfortunately, its applicability is limited to small to medium-sized retinoblastomas in accessible locations. Cryotherapy and transpupillary thermotherapy (TTT) have been used to provide control of selected small tumours. TTT is an advanced laser system adapted to the indirect ophthalmoscope which provides flexible nonsurgical treatment for small retinoblastomas. Recent research in the treatment of retinoblastoma has concentrated on methods of combining chemotherapy with other local treatment modalities (TTT, radiotherapy, cryotherapy). This approach combines the principle of chemotherapeutic debulking in paediatric oncology with conservative focal therapies in ophthalmology. Termed chemoreduction, intravenous or subconjunctival chemotherapy is used to debulk the initial tumour volume and allow for local treatment with TTT, cryotherapy and plaque radiotherapy. Cyclosporin has been added to the chemotherapy regimen in several centres. Other clinical settings where chemotherapy is considered are situations where the histopathology suggests a high risk for metastatic disease and where there is extraocular extension. There is no consensus that chemotherapy is needed when choroidal invasion is observed on histopathology. However, in patients where the retinoblastoma is noted beyond the cut end of the optic nerve or if there is disruption of the sclera with microscopic invasion of the orbital tissue, treatment has been helpful. Systemic and intrathecal chemotherapy with local and cranial radiotherapy has improved the survival of these patients. Most recently, the use of new chemotherapy modalities with haematopoietic stem cell rescue or local radiotherapy has increased the survival of patients with distant metastasis. Nevertheless, the prognosis of patients with central nervous system involvement is still poor. |
| Fine B, Stanulla M, Schrappe M, Ho M, Viehmann S, Harbott J, Boxer L |
Gene expression patterns associated with recurrent chromosomal translocations in acute lymphoblastic leukemia. |
Blood 2004, 103: 1043 |
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| Fischer M, Berthold F |
Characterization of the gene expression profile of neuroblastoma cell line IMR-5 using serial analysis of gene expression. |
Cancer Lett 2003, 190: 79 |
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| Fischer M, Skowron M, Berthold F |
Reliable transcript quantification by real-time reverse transcriptase-polymerase chain reaction in primary neuroblastoma using normalization to averaged expression levels of the control genes HPRT1 and SDHA. [+] |
The Journal of molecular diagnostics 2005, 7: 89 |
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| Real-time reverse transcriptase-polymerase chain reaction (RT-PCR) represents a sensitive and efficient technique to determine expression levels of target genes in multiple samples and is increasingly used in clinical oncology to evaluate the patient's outcome or to detect minimal residual disease. Normalization of raw data are required to obtain comparable results between different specimens and is usually achieved by correlating transcript abundances of target genes with those of a single control gene with putatively stable expression levels. In this study, expression stability of six supposed control genes was evaluated in 64 samples of primary neuroblastoma and HPRT1 and SDHA mRNA levels were shown to exhibit the least expression variability among the samples. Because application of more than one control gene may enhance reliability of real-time RT-PCR results, various normalization factors consisting of the geometrical mean of multiple control gene expression values were calculated and evaluated by mRNA quantification of 14 target genes. Comparison with transcript levels determined by oligonucleotide-array expression analysis revealed that target gene mRNA quantification became most consistent after normalization to averaged expression levels of HPRT1 and SDHA. This normalization factor was in addition demonstrated to be not associated with stage of disease or MYCN amplification status of the tumor. Thus, these data indicate that the geometrical mean of HPRT1 and SDHA transcript levels represents a suitable internal control for biological and clinical studies investigating differential gene expression in primary neuroblastoma by real-time RT-PCR. |
| Fischer M, Oberthuer A, Brors B, Kahlert Y, Skowron M, Voth H, Warnat P, Ernestus K, Hero B, Berthold F |
Differential expression of neuronal genes defines subtypes of disseminated neuroblastoma with favorable and unfavorable outcome. [+] |
Clinical cancer research : an official journal of the American Association for Cancer Research 2006, 12: 5118 |
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| PURPOSE: Identification of molecular characteristics of spontaneously regressing stage IVS and progressing stage IV neuroblastoma to improve discrimination of patients with metastatic disease following favorable and unfavorable clinical courses. EXPERIMENTAL DESIGN: Serial analysis of gene expression profiles were generated from five stage IVS and three stage IV neuroblastoma. Differential expression of candidate genes was evaluated by real-time quantitative reverse transcription-PCR in 76 pretreatment tumor samples (stage IVS n=27 and stage IV n=49). Gene expression-based outcome prediction was determined by Prediction Analysis for Microarrays using 38 tumors as a training set and 38 tumors as a test set. RESULTS: Comparison of serial analysis of gene expression profiles from stage IV and IVS neuroblastoma revealed approximately 500 differentially expressed transcripts. Genes related to neuronal differentiation were observed more frequently in stage IVS tumors as determined by associating transcripts to Gene Ontology annotations. Forty-one candidate genes were evaluated by quantitative reverse transcription-PCR and 18 were confirmed to be differentially expressed (P |
| Fischer M, Spitz R, Oberthür A, Westermann F, Berthold F |
Risk estimation of neuroblastoma patients using molecular markers. [+] |
Klinische Padiatrie 2008, 220: 137 |
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| The pediatric tumor neuroblastoma is a heterogeneous disease: Patients' clinical courses can range from spontaneous regression to fatal progression of the disease. Accordingly, treatment protocols vary from |
| Fischer M, Bauer T, Oberthür A, Hero B, Theissen J, Ehrich M, Spitz R, Eils R, Westermann F, Brors B, König R, Berthold F |
Integrated genomic profiling identifies two distinct molecular subtypes with divergent outcome in neuroblastoma with loss of chromosome 11q. [+] |
Oncogene 2010, 29: 865 |
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| Imbalances in chromosome 11q occur in approximately 30% of primary neuroblastoma and are associated with poor outcome. It has been suggested that 11q loss constitutes a distinct clinico-genetic neuroblastoma subgroup by affecting expression levels of corresponding genes. This study analysed the relationship of 11q loss, clinical phenotype and global transcriptomic profiles in four clinico-genetic subgroups (11q alteration/favourable outcome, n=7; 11q alteration/unfavourable outcome, n=14; no 11q alteration/favourable outcome, n=81; no 11q alteration/unfavourable outcome, n=8; tumours with MYCN amplification and/or 1p loss were excluded). Unsupervised and supervised comparisons of gene expression profiles consistently showed significantly different mRNA patterns between favourable and unfavourable neuroblastomas, both in the subgroups with and without 11q loss. In contrast, favourable tumours with and without 11q loss showed highly similar transcriptomic profiles. Disproportionate downregulation of 11q genes was observed only in unfavourable tumours with 11q loss. The diverging molecular profiles were neither caused by considerable differences in the size of the deleted regions nor by differential methylation patterns of 11q genes. Together, this study shows that neuroblastoma with 11q loss comprises two biological subgroups that differ both in their clinical phenotype and gene expression patterns, indicating that 11q loss is not a primary determinant of neuroblastoma tumour behaviour. |
| Flentje M, Weirich A, Potter R, Ludwig R |
Hepatotoxicity in irradiated nephroblastoma patients during postoperative treatment according to SIOP9/GPOH. |
Radiother Oncol 1994, 31: 222 |
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| Flentje M, Weirich A, Graf N, Potter R, Zimmerman H, Ludwig R |
Abdominal irradiation in unilateral nephroblastoma and its impact on local control and survival. |
Int J Radiat Oncol Biol Phys 1998, 40: 163 |
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| Fletcher O, Easton D, Anderson K, Gilham C, Jay M, Peto J |
Lifetime risks of common cancers among retinoblastoma survivors. [+] |
Journal of the National Cancer Institute 2004, 96: 357 |
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| BACKGROUND: Compared with the general population, carriers of germline mutations in RB1 who survive retinoblastoma (i.e., hereditary retinoblastoma survivors) are at increased risk of early-onset second cancers, particularly sarcomas, brain tumors, and melanoma. However, their risks for the epithelial cancers that commonly occur after age 50 years are not known. METHODS: We used hospital records to identify British retinoblastoma survivors born between 1873 and 1950, a period when few British retinoblastoma patients received high-dose radiotherapy. Cancers and deaths were identified by linkage with national registration records. All statistical tests were two-sided. RESULTS: We could trace the cancer histories of 144 survivors of hereditary retinoblastoma. From age 25 to age 84, there were 58 subsequent cancers, for a cumulative cancer incidence of 68.8% (95% confidence interval [CI] = 48.0% to 87.4%) and a cumulative cancer mortality of 56.3% (95% CI = 40.5% to 73.3%). Only eight of the 58 cancers were of bone or soft tissue, in marked contrast to findings from contemporary studies of American patients treated with external beam radiotherapy, among whom most second tumors are sarcomas. Compared with the general population, hereditary retinoblastoma survivors had higher mortality from lung cancer (standardized mortality ratio [SMR] = 7.01, 95% CI = 3.83 to 11.76), bladder cancer (SMR = 26.31, 95% CI = 8.54 to 61.41), and all other epithelial cancers combined (SMR = 3.29, 95% CI = 1.64 to 5.89). The overall standardized mortality ratio for epithelial cancer was inversely proportional to the approximate square of age (exponent of age = -2.1, 95% CI = -3.6 to -0.7), declining from 11.32 (95% CI = 4.15 to 24.64) at age 25-44 to 2.83 (95% CI = 1.04 to 6.16) at age 65-84. CONCLUSIONS: Survivors of hereditary retinoblastoma who are not exposed to high-dose radiotherapy have a high lifetime risk of developing a late-onset epithelial cancer. Most of the excess cancer risks in hereditary retinoblastoma survivors might be preventable by limiting exposures to DNA damaging agents (radiotherapy, tobacco, and UV light). |
| Flotho C, Valcamonica S, Mach-Pascual S, Schmahl G, Corral L, Ritterbach J, Hasle H, Arico M, Biondi A, Niemeyer C |
RAS mutations and clonality analysis in children with juvenile myelomonocytic leukemia (JMML). |
Leukemia 1999, 13: 32 |
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| Flower KB, Hoppin JA, Lynch CF, Blair A, Knott C, Shore DL, Sandler DP |
Cancer risk and parental pesticide application in children of Agricultural Health Study participants. |
Environ Health Perspect 2004, 112: 631 |
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| Flohr T, Schrauder A, Cazzaniga G, Panzer-Grümayer R, van der Velden V, Fischer S, Stanulla M, Basso G, Niggli FK, Schäfer BW, Sutton R, Koehler R, Zimmermann M, Valsecchi MG, Gadner H, Masera G, Schrappe M, van Dongen JJ, Biondi A, Bartram CR, International BFM Study Group (I-BFM-SG) |
Minimal residual disease-directed risk stratification using real-time quantitative PCR analysis of immunoglobulin and T-cell receptor gene rearrangements in the international multicenter trial AIEOP-BFM ALL 2000 for childhood acute lymphoblastic leukemia. [+] |
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K 2008, 22: 771 |
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| Detection of minimal residual disease (MRD) is the most sensitive method to evaluate treatment response and one of the strongest predictors of outcome in childhood acute lymphoblastic leukemia (ALL). The 10-year update on the I-BFM-SG MRD study 91 demonstrates stable results (event-free survival), that is, standard risk group (MRD-SR) 93%, intermediate risk group (MRD-IR) 74%, and high risk group (MRD-HR) 16%. In multicenter trial AIEOP-BFM ALL 2000, patients were stratified by MRD detection using quantitative PCR after induction (TP1) and consolidation treatment (TP2). From 1 July 2000 to 31 October 2004, PCR target identification was performed in 3341 patients: 2365 (71%) patients had two or more sensitive targets (< or =10(-4)), 671 (20%) patients revealed only one sensitive target, 217 (6%) patients had targets with lower sensitivity, and 88 (3%) patients had no targets. MRD-based risk group assignment was feasible in 2594 (78%) patients: 40% were classified as MRD-SR (two sensitive targets, MRD negativity at both time points), 8% as MRD-HR (MRD > or =10(-3) at TP2), and 52% as MRD-IR. The remaining 823 patients were stratified according to clinical risk features: HR (n=108) and IR (n=715). In conclusion, MRD-PCR-based stratification using stringent criteria is feasible in almost 80% of patients in an international multicenter trial. |
| Foell JL, Max D, Giersberg C, Körholz D, Staege MS |
Sensitivity of Hodgkin's lymphoma cell lines to the cell cycle inhibitor roscovitine. [+] |
Anticancer research 2008, 28: 887 |
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| BACKGROUND: The prognosis of patients with Hodgkin's lymphoma (HL) has improved in recent decades. However, not all patients can be cured and the development of alternative treatment strategies is necessary. MATERIALS AND METHODS: Gene expression in HL cell lines was analyzed using DNA microarrays and both conventional and quantitative reverse transcriptase-polymerase chain reaction. Sensitivity of HL cell lines to the cell cycle inhibitor roscovitine was assessed in vitro. RESULTS: All HL cell lines express high levels of cyclin D2. Treatment of HL cells with roscovitine induced cell death in some cell lines whereas other cell lines were resistant to roscovitine. Roscovitine-sensitive cell lines were characterized by expression of T-cell markers and expressed high levels of the unusual cytokine interleukin-26. CONCLUSION: Roscovitine is a cytotoxic drug for a subpopulation of HL cells and might be an interesting agent for the treatment of patients with HL. |
| Franzius C, Bielack S, Sciuk J, Vollet B, Jürgens H, Schober O |
High-activity samarium-153-EDTMP therapy in unresectable osteosarcoma. |
Nuklearmedizin 1999, 38: 337 |
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| Franzius C, Sciuk J, Brinkschmidt C, Jürgens H, Schober O |
Evaluation of chemotherapy response in primary bone tumors with F-18 FDG positron emission tomography compared with histologically assessed tumor necrosis. |
Clinical Nuclear Medicine 2000, 25: 874 |
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| Franzius C, Sciuk J, Daldrup-Link H, Jürgens H, Schober O |
FDG-PET for detection of osseous metastases from malignant primary bone tumors. |
Eur J Nucl Med 2000, 27: 1305 |
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| Franzius C, Daldrup-Link H, Sciuk J, Rummeny E, Bielack S, Jürgens H, Schober O |
FDG-PET for detection of pulmonary metastases from malignant primary bone tumors. |
Ann Oncol 2001, 12: 479 |
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| Franzius C, Schulte M, Hillmann A, Winkelmann W, Jürgens H, Bockisch A, Schober O |
Klinische Wertigkeit der Positronenemissionstomographie (PET) in der Diagnostik der Knochen- und Weichteiltumore. |
Chirurg 2001, 72: 1071 |
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| Franzius C, Bielack S, Flege S, Sciuk J, Jürgens H, Schober O |
Prognostic significance of (18)F-FDG and (99m)Tc-methylene diphosphonate uptake in primary osteosarcoma. |
J Nucl Med 2002, 43: 1012 |
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| Franzius C, Daldrup-Link H, Wagner-Bohn A, Sciuk J, Heindel W, Jürgens H, Schober O |
FDG-PET for detection of recurrences from malignant primary bone tumors. |
Ann Oncol 2002, 13: 157 |
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| Franzius C, Schuck A, Bielack S |
High-dose samarium-153 ethylene diamine tetramethylene phosphonate. |
J Clin Oncol 2002, 20: 1953 |
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| Franzius C, Hotfilder M, Poremba C, Hermann S, Schafers K, Gabbert HE, Jürgens H, Schober O, Schafers M, Vormoor J |
Successful high-resolution animal positron emission tomography of human Ewing tumours and their metastases in a murine xenograft model. [+] |
European journal of nuclear medicine and molecular imaging 2006, 33: 1432 |
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| PURPOSE: As primary osseous metastasis is the main adverse prognostic factor in patients with Ewing tumours, a NOD/scid mouse model for human Ewing tumour metastases has been established to examine the mechanisms of metastasis. The aim of this study was to evaluate the feasibility of diagnostic molecular imaging by small animal PET in this mouse model. METHODS: Human Ewing tumour cells were transplanted into immune-deficient NOD/scid mice via s.c injection (n=17) or i.v. injection (n=17). The animals (mean weight 23.2 g) were studied 2-7 weeks after transplantation using a submillimetre resolution animal PET scanner. To assess glucose utilisation and bone metabolism, mice were scanned after intravenous injection of 9.6 MBq (mean) 2-[(18)F]fluoro-2-deoxy-D: -glucose (FDG) or 9.4 MBq (mean) [(18)F]fluoride. Whole-body PET images were analysed visually and semi-quantitatively [%ID/g, tumour to non-tumour ratio (T/NT)]. Foci of pathological uptake were identified with respect to the physiological organ uptake in corresponding regions. RESULTS: Subcutaneously transplanted Ewing tumours demonstrated a moderately increased glucose uptake (median %ID/g 2.5; median T/NT 2.2). After i.v. transplantation, the pattern of metastasis was similar to that in patients with metastases in lung, bone and soft tissue. These metastases showed an increased FDG uptake (median %ID/g 3.6; median T/NT 2.7). Osseous metastases were additionally visible on [(18)F]fluoride PET by virtue of decreased [(18)F]fluoride uptake (osteolysis; median %ID/g 8.4; median T/NT 0.59). Metastases were confirmed immunohistologically. CONCLUSION: Diagnostic molecular imaging of Ewing tumours and their small metastases in an in vivo NOD/scid mouse model is feasible using a submillimetre resolution PET scanner. |
| Franzius C, Juergens KU, Vormoor J |
PET/CT with diagnostic CT in the evaluation of childhood sarcoma. |
AJR. American journal of roentgenology 2006, 186: 581; author reply 581 |
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| Franke M, Hardes J, Helmke K, Jundt G, Jürgens H, Kempf-Bielack B, Kevric M, Tunn PU, Werner M, Bielack S |
Solitary skeletal osteosarcoma recurrence. Findings from the Cooperative Osteosarcoma Study Group. [+] |
Pediatr Blood Cancer 2011, 56: 771 |
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| BACKGROUND: Solitary skeletal osteosarcoma (OS) manifestations distant from the site of the primary tumor rarely arise as only sign of disease recurrence.
METHODS: This report reviews 38 patients with high-grade central OS of the limbs or axial skeleton and initial complete surgical remission (CR) who developed first recurrences as solitary osseous lesions distant from the primary tumor. The Cooperative Osteosarcoma Study Group (COSS) database was used to evaluate patient-, tumor-, and treatment-related variables and outcomes.
RESULTS: Thirty-eight patients (27 males and 11 females; 36 limb and 2 axial primaries) developed solitary osseous recurrences a median of 2.1 years (range: 0.5-14.3) from primary diagnosis. Relapses involved axial (24), extremity (10), or craniofacial bones (4). Treatment for recurrence included surgery (28), radiotherapy (10), and chemotherapy (27). After a median follow-up of 1.9 years (range: 0.1-21.2) from first recurrence for all 38 patients and 5.5 years (0.3-21.2) for 16 survivors (10 in continuous second CR), 2- and 5-year overall and event-free survival (EFS) probabilities were 55% and 34% and 34% and 27%, respectively. A long interval to recurrence (>1.5 years) predicted for better overall (P < 0.001) and EFS (P = 0.005). For 21 patients achieving a second CR, 2- and 5-year overall and EFS probabilities were 81% and 61% and 52% and 49%, respectively, while only 1/17 others survived beyond 5 years (P < 0.001). Survivors (14/16) had also received second-line chemotherapy.
CONCLUSION: First solitary skeletal recurrences of OS should be treated with curative intent. Some presumed bone metastases may represent second primary OSs. Pediatr Blood Cancer. © 2010 Wiley-Liss, Inc. |
| Franke M, Hardes J, Helmke K, Jundt G, Jürgens H, Kempf-Bielack B, Kevric M, Tunn PU, Werner M, Bielack S |
Solitary skeletal osteosarcoma recurrence. Findings from the Cooperative Osteosarcoma Study Group. [+] |
Pediatric blood & cancer 2011, 56: 771 |
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| Solitary skeletal osteosarcoma (OS) manifestations distant from the site of the primary tumor rarely arise as only sign of disease recurrence. |
| Freidank C |
Wegweiser für zu Hause. |
Leitung der bundesweiten Arbeitsgruppe pädiatrisch-onkologischer Kinderkrankenschwestern und Kinderkrankenpfleger (GPONG: German Pediatric Oncology Nurses Group) 2002 |
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| Friedman JM, Birch PH |
Type 1 neurofibromatosis: a descriptive analysis of the disorder in 1,728 patients. |
Am J Med Genet 1997, 70: 138 |
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| Friesen C, Fulda S, Debatin K |
Induction of CD95 ligand and apoptosis by doxorubicin is modulated by the redox state in chemosensitive- and drug-resistant tumor cells. |
Cell Death Differ 1999, 6: 471 |
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| Frühwald MC, Krefeld B, Benesch M, Büchner J, Boos J, Ebetsberger G, Graf N, Kortmann R, Nysom K, Rutkowski S, Schneppenheim R, Siebert R, Timmermann B, Warmuth-Matz M, Hasselblatt M |
The European Rhabdoid Registry (EU-RHAB) – A comprehensive approach towards biology and clinical management. [+] |
Neurooncology 2010, 12:ii36 |
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| Rhabdoid tumors mainly affect very young children; below 6 months of age they may be the most common intracranial malignancies. In 2007 we initiated the European Rhabdoid Registry (EU-RHAB) in cooperation with the SIOP subcommittee on brain tumors to increase our knowledge of rhabdoid tumors and to ultimately improve outcome. EU-RHAB has collected data on epidemiology, molecular genetics, pathology and treatment of
35 patients with atypical teratoid rhabdoid tumors (AT/RT). Median age at diagnosis was 22 months. All cases were confirmed by reference histopathology (in general by INI1 staining). Only one case showed no INI1-loss. Molecular genetic analyses of SMARCB1/hSNF5/INI1 were performed in 24 cases (germ line mutations n¼ 3). In only 11 of 29 patients initial pathology revealed the correct diagnosis. 2/35 received
no therapy, 32 were treated according to a consensus therapy. Follow-up is short (0 to 29 months). Patients treated according to the consensus demonstrate a 1-year-OS of 71.4%. 11 received some form of radiotherapy, all of these are alive. 10 patients are free of disease .12 months. The median age at diagnosis of the 9 patients who died within
0.5–12 months was 9 months. Patients with a germ line mutation and those who did not receive any therapy were among these. CONCLUSION: We present first data from the European Rhabdoid Registry (EU-RHAB). The prospective registration of children with rhabdoid tumors is a prerequisite for the development of innovative strategies
that will then be implemented into phase I/II clinical trials. Supported by
Horizont/Weseke and the Deutsche Kinderkrebsstiftung |
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| Fröhlich B, Ahrens S, Burdach S, Klingebiel T, Ladenstein R, Paulussen M, Zoubek A, Jürgens H |
High-dosage chemotherapy in primary metastasized and relapsed Ewing's sarcoma. (EI)CESS. |
Klin Pädiatr 1999, 211: 284 |
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| Fuchs N, Winkler K |
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| Fuchs J, Wenderoth M, von Schweinitz D, Haindl J, Leuschner I |
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| Fuchs N, Bielack S, Epler D, Bieling P, Delling G, Körholz D, Graf N, Heise U, Jürgens H, Kotz R, Salzer-Kuntschik M, Weinel P, Werner M, Winkler K |
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| Fuchs J, Bode U, von Schweinitz D, Weinel P, Erttmann R, Harms D, Mildenberger H |
Analysis of treatment efficiency of carboplatin and etoposide in combination with radical surgery in advanced and recurrent childhood hepatoblastoma. |
Klin Pädiatr 1999, 211: 305 |
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| Fuchs J, Rydzynski J, von Schweinitz D, Bode U, Hecker H, Weinel P, Burger D, Harms D, Erttmann R, Oldhafer K, Mildenberger H |
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| Fulda S, Honer M, Menke-Moellers I, Berthold F |
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| Fulda S, Susin S, Kroemer G, Debatin K |
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| Fulda S, Jeremias I, Pietsch T, Debatin K |
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| Fulda S, Jeremias I, Steiner H, Pietsch T, Debatin K |
Betulinic acid. |
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| Fulda S, Lutz W, Schwab M, Debatin K |
MycN sensitizes neuroblastoma cells for drug-induced apoptosis. |
Oncogene 1999, 18: 1479 |
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| Fulda S, Meyer E, Debatin K |
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Cancer Res 2000, 60: 3947 |
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| Furlan I, Batz C, Flotho C, Mohr B, Lübbert M, Suttorp M, Niemeyer CM |
Intriguing response to azacitidine in a patient with juvenile myelomonocytic leukemia and monosomy 7. |
Blood 2009, 113: 2867 |
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| Furtwängler R, Nourkami N, Alkassar M, von Schweinitz D, Schenk JP, Rübe C, Siemer S, Leuschner I, Graf N |
Update on relapses in unilateral nephroblastoma registered in 3 consecutive SIOP/GPOH studies - a report from the GPOH-nephroblastoma study group. [+] |
Klin padiatr 2011, 223: 113 |
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| INTRODUCTION:
Treatment and stratification of progressive/relapsed unilateral nephroblastoma (PD) has significantly evolved over the last 20 years. Early PD (≤ 6 months), initial high risk histology, local stage III, multiple site PD and stage IV have been implemented as high risk classification factors and novel drugs have been introduced.
PATIENTS AND METHODS:
We analysed all 251 patients having had a unilateral nephroblastoma (Stage I-IV) and progressive disease who had been treated according to SIOP9/GPO (n = 77), SIOP93-1/GPOH (n = 93) and SIOP2001/GPOH (n = 81) initially.
RESULTS:
3y-overall survival (OS) increased from 43% to 61% and 59% respectively (both p<0.01). 3y-OS for localized stage I-III rose from 43% to 65% and 68% respectively while only little improvement can be seen for initial stage IV patients with 43%, 53% and 44% respectively. Multivariate analysis confirmed high risk histology, local stage III, shorter time to PD, combined relapse as independent risk factors. 26 patients had received high-dose chemotherapy showing 64% 3y-OS compared to 54% for all non-transplanted (p=0.11).
CONCLUSION:
Structuring the treatment of progressive nephroblastoma as well as introducing new drugs have improved the outcome significantly. However improvement is depending on the specific risk profile. Very high risk tumours are often resistant to conventional treatment, hence an international uniform treatment concept is needed to achieve conclusive results in this small group. |
| Futaki M, Yamashita T, Yagasaki H, Toda T, Yabe M, Kato S, Asano S, Nakahata T |
The IVS4 + 4 A to T mutation of the fanconi anemia gene FANCC is not associated with a severe phenotype in Japanese patients. [+] |
Blood 2000, 95: 1493 |
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| Fanconi anemia (FA) is an autosomal recessive disease characterized by congenital anomalies, aplastic anemia, and a susceptibility to leukemia. There are at least 8 complementation groups (A through H). Extensive analyses of the FA group C gene FANCC in Western countries revealed that 10% to 15% of FA patients have mutations of this gene. The most common mutation is IVS4 + 4 A to T (IVS4), a splice mutation in intron 4, which has been found only in patients of Ashkenazi Jewish ancestry. When we screened 29 Japanese patients (20 unrelated patients and 4 families) using polymerase chain reaction-single strand conformation polymorphism, we found 8 unrelated patients homozygous for IVS4. This is apparently the first non-Ashkenazi-Jewish population for whom this mutation has been detected. The Ashkenazi Jewish patients homozygous for IVS4 have a severe phenotype, in comparison with other FA patients. Our analyses of Japanese patients indicate no significant difference between IVS4 homozygotes and other patients with regard to severity of a clinical phenotype. Thus, ethnic background may have a significant effect on a clinical phenotype in FA patients carrying the same mutation. (Blood. 2000;95:1493-1498) |
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