15 results on '"Perwein, T"'
Search Results
2. MR Imaging and Clinical Characteristics of Diffuse Glioneuronal Tumor with Oligodendroglioma-like Features and Nuclear Clusters
- Author
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Neurochirurgie, Brain, Cancer, MS Radiologie, Circulatory Health, Benesch, M, Perwein, T, Apfaltrer, G, Langer, T, Neumann, A, Brecht, I B, Schuhmann, M U, Cario, H, Frühwald, M C, Vollert, K, van Buiren, M, Deng, M Y, Seitz, A, Haberler, C, Mynarek, M, Kramm, C, Sahm, F, Robe, P A, Dankbaar, J W, Hoff, K V, Warmuth-Metz, M, Bison, B, Neurochirurgie, Brain, Cancer, MS Radiologie, Circulatory Health, Benesch, M, Perwein, T, Apfaltrer, G, Langer, T, Neumann, A, Brecht, I B, Schuhmann, M U, Cario, H, Frühwald, M C, Vollert, K, van Buiren, M, Deng, M Y, Seitz, A, Haberler, C, Mynarek, M, Kramm, C, Sahm, F, Robe, P A, Dankbaar, J W, Hoff, K V, Warmuth-Metz, M, and Bison, B
- Published
- 2022
3. Imatinib-induced long-term remission in a relapsed RCSD1-ABL1-positive acute lymphoblastic leukemia
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Perwein, T., primary, Strehl, S., additional, Konig, M., additional, Lackner, H., additional, Panzer-Grumayer, R., additional, Mann, G., additional, Attarbaschi, A., additional, Urban, E.-C., additional, and Haas, O. A., additional
- Published
- 2016
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4. Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile.
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Nussbaumer G, Benesch M, Grabovska Y, Mackay A, Castel D, Grill J, Alonso MM, Antonelli M, Bailey S, Baugh JN, Biassoni V, Blattner-Johnson M, Broniscer A, Carai A, Colafati GS, Colditz N, Corbacioglu S, Crampsie S, Entz-Werle N, Eyrich M, Friker LL, Frühwald MC, Garrè ML, Gerber NU, Giangaspero F, Gil-da-Costa MJ, Graf N, Hargrave D, Hauser P, Herrlinger U, Hoffmann M, Hulleman E, Izquierdo E, Jacobs S, Karremann M, Kattamis A, Kebudi R, Kortmann RD, Kwiecien R, Massimino M, Mastronuzzi A, Miele E, Morana G, Noack CM, Pentikainen V, Perwein T, Pfister SM, Pietsch T, Roka K, Rossi S, Rutkowski S, Schiavello E, Seidel C, Štěrba J, Sturm D, Sumerauer D, Tacke A, Temelso S, Valentini C, van Vuurden D, Varlet P, Veldhuijzen van Zanten SEM, Vinci M, von Bueren AO, Warmuth-Metz M, Wesseling P, Wiese M, Wolff JEA, Zamecnik J, Morales La Madrid A, Bison B, Gielen GH, Jones DTW, Jones C, and Kramm CM
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- Humans, Child, Male, Female, Adolescent, Retrospective Studies, Prognosis, Child, Preschool, Phenotype, Survival Rate, DNA Methylation, Infant, Biomarkers, Tumor genetics, Mutation, Follow-Up Studies, Neoplasm Grading, Neoplasms, Neuroepithelial pathology, Neoplasms, Neuroepithelial genetics, Brain Neoplasms genetics, Brain Neoplasms pathology, Glioma genetics, Glioma pathology
- Abstract
Background: The term gliomatosis cerebri (GC), a radiology-defined highly infiltrating diffuse glioma, has been abandoned since molecular GC-associated features could not be established., Methods: We conducted a multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical and (epi-)genetic characterization., Results: Median overall survival (OS) was 15.5 months (interquartile range, 10.9-27.7) with a 2-year survival rate of 28%. Histopathological grading correlated significantly with median OS: CNS WHO grade II: 47.8 months (25.2-55.7); grade III: 15.9 months (11.4-26.3); grade IV: 10.4 months (8.8-14.4). By DNA methylation profiling (n = 49), most tumors were classified as pediatric-type diffuse high-grade glioma (pedHGG), H3-/IDH-wild-type (n = 31/49, 63.3%) with enriched subclasses pedHGG_RTK2 (n = 19), pedHGG_A/B (n = 6), and pedHGG_MYCN (n = 5), but only one pedHGG_RTK1 case. Within the pedHGG, H3-/IDH-wild-type subgroup, recurrent alterations in EGFR (n = 10) and BCOR (n = 9) were identified. Additionally, we observed structural aberrations in chromosome 6 in 16/49 tumors (32.7%) across tumor types. In the pedHGG, H3-/IDH-wild-type subgroup TP53 alterations had a significant negative effect on OS., Conclusions: Contrary to previous studies, our representative pediatric GC study provides evidence that GC has a strong predilection to arise on the background of specific molecular features (especially pedHGG_RTK2, pedHGG_A/B, EGFR and BCOR mutations, chromosome 6 rearrangements)., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.)
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- 2024
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5. Sustained Survival Benefit in Recurrent Medulloblastoma by a Metronomic Antiangiogenic Regimen: A Nonrandomized Controlled Trial.
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Peyrl A, Chocholous M, Sabel M, Lassaletta A, Sterba J, Leblond P, Nysom K, Torsvik I, Chi SN, Perwein T, Jones N, Holm S, Nyman P, Mörse H, Öberg A, Weiler-Wichtl L, Leiss U, Haberler C, Schmook MT, Mayr L, Dieckmann K, Kool M, Gojo J, Azizi AA, André N, Kieran M, and Slavc I
- Subjects
- Humans, Male, Child, Child, Preschool, Adolescent, Female, Etoposide, Quality of Life, Administration, Metronomic, Antineoplastic Combined Chemotherapy Protocols adverse effects, Medulloblastoma drug therapy, Medulloblastoma etiology, Brain Neoplasms drug therapy, Cerebellar Neoplasms drug therapy, Cerebellar Neoplasms etiology
- Abstract
Importance: Medulloblastoma recurrence in patients who have previously received irradiation has a dismal prognosis and lacks a standard salvage regimen., Objective: To evaluate the response rate of pediatric patients with medulloblastoma recurrence using an antiangiogenic metronomic combinatorial approach (Medulloblastoma European Multitarget Metronomic Anti-Angiogenic Trial [MEMMAT])., Design, Setting, and Participants: This phase 2, investigator-initiated, multicenter nonrandomized controlled trial assessed 40 patients with relapsed or refractory medulloblastoma without a ventriculoperitoneal shunt who were younger than 20 years at original diagnosis. Patients were enrolled between April 1, 2014, and March 31, 2021., Interventions: Treatment consisted of daily oral thalidomide, fenofibrate, celecoxib, and alternating 21-day cycles of low-dose (metronomic) oral etoposide and cyclophosphamide, supplemented by intravenous bevacizumab and intraventricular therapy consisting of alternating etoposide and cytarabine., Main Outcomes and Measures: The primary end point was response after 6 months of antiangiogenic metronomic therapy. Secondary end points included progression-free survival (PFS), overall survival (OS), and quality of life. Adverse events were monitored to assess safety., Results: Of the 40 patients (median [range] age at treatment start, 10 [4-17] years; 25 [62.5%] male) prospectively enrolled, 23 (57.5%) achieved disease control after 6 months of treatment, with a response detected in 18 patients (45.0%). Median OS was 25.5 months (range, 10.9-40.0 months), and median PFS was 8.5 months (range, 1.7-15.4 months). Mean (SD) PFS at both 3 and 5 years was 24.6% (7.9%), while mean (SD) OS at 3 and 5 years was 43.6% (8.5%) and 22.6% (8.8%), respectively. No significant differences in PFS or OS were evident based on molecular subgroup analysis or the number of prior recurrences. In patients demonstrating a response, mean (SD) overall 5-year PFS was 49.7% (14.3%), and for patients who remained progression free for the first 12 months of treatment, mean (SD) 5-year PFS was 66.7% (16.1%). Treatment was generally well tolerated. Grade 3 to 4 treatment-related adverse events included myelosuppression, infections, seizures, and headaches. One heavily pretreated patient with a third recurrence died of secondary acute myeloid leukemia., Conclusions and Relevance: This feasible and well-tolerated MEMMAT combination regimen demonstrated promising activity in patients with previously irradiated recurrent medulloblastoma. Given these results, this predominantly oral, well-tolerated, and outpatient treatment warrants further evaluation., Trial Registration: ClinicalTrials.gov Identifier: NCT01356290.
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- 2023
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6. Boswellic acid formulations are not suitable for treatment of pediatric high-grade glioma due to tumor promoting potential.
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Wiese M, Pohlmeier B, Kubiak K, El-Khouly FE, Sitte M, Carcaboso AM, Baugh JN, Perwein T, Nussbaumer G, Karremann M, Gielen GH, Salinas G, and Kramm CM
- Abstract
Background and Aim: Pediatric high-grade gliomas (pedHGG) comprise a very poor prognosis. Thus, parents of affected children are increasingly resorting to complementary and alternative medicine (CAM), among those Boswellia extracts. However, nothing is known about the therapeutic effectiveness of their active substances, Boswellic acids (BA) in pedHGG. Thus, we aimed to investigate if the three main Boswellic acids (BA) present in Boswellia plants, alpha-boswellic acid (α-BA), beta-boswellic acid (β-BA) and 3-acetyl-11-keto-beta-boswellic acid (AKBA) hold any promising potential for treatment of affected pedHGG patients., Experimental Procedure: Histone 3 (H3)-wildtype and H3.3K27M-mutant pedHGG cell lines were treated with BA, either alone or in combination with radio-chemotherapy with temozolomide. Cell viability, stemness properties, apoptosis, in ovo tumor growth and the transcriptome was investigated upon BA treatment., Results and Conclusion: Interestingly, α-BA and β-BA treatment promoted certain tumor properties in both pedHGG cells. AKBA treatment reduced cell viability and colony growth accompanied by induction of slight anti-inflammatory effects especially in H3.3K27M-mutant pedHGG cells. However, no effects on apoptosis and in ovo tumor growth were found. In conclusion, besides positive anti-tumor effects of AKBA, tumor promoting effects were observed upon treatment with α-BA and β-BA. Thus, only pure AKBA formulations may be used to exploit any potential positive effects in pedHGG patients. In conclusion, the use of commercially available supplements with a mixture of different BA cannot be recommended due to detrimental effects of certain BA whereas pure AKBA formulations might hold some potential as therapeutic supplement for treatment of pedHGG patients., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 Center for Food and Biomolecules, National Taiwan University. Production and hosting by Elsevier Taiwan LLC.)
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- 2023
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7. How I treat recurrent pediatric high-grade glioma (pHGG): a Europe-wide survey study.
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Perwein T, Giese B, Nussbaumer G, von Bueren AO, van Buiren M, Benesch M, and Kramm CM
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- Adolescent, Humans, Child, Proto-Oncogene Proteins B-raf, Temozolomide therapeutic use, Bevacizumab therapeutic use, Chronic Disease, Brain Neoplasms therapy, Brain Neoplasms drug therapy, Glioma therapy, Glioma drug therapy
- Abstract
Purpose: As there is no standard of care treatment for recurrent/progressing pediatric high-grade gliomas (pHGG), we aimed to gain an overview of different treatment strategies., Methods: In a web-based questionnaire, members of the SIOPE-BTG and the GPOH were surveyed on therapeutic options in four case scenarios (children/adolescents with recurrent/progressing HGG)., Results: 139 clinicians with experience in pediatric neuro-oncology from 22 European countries participated in the survey. Most respondents preferred further oncological treatment in three out of four cases and chose palliative care in one case with marked symptoms. Depending on the case, 8-92% would initiate a re-resection (preferably hemispheric pHGG), combined with molecular diagnostics. Throughout all case scenarios, 55-77% recommended (re-)irradiation, preferably local radiotherapy > 20 Gy. Most respondents would participate in clinical trials and use targeted therapy (79-99%), depending on molecular genetic findings (BRAF alterations: BRAF/MEK inhibitor, 64-88%; EGFR overexpression: anti-EGFR treatment, 46%; CDKN2A deletion: CDK inhibitor, 18%; SMARCB1 deletion: EZH2 inhibitor, 12%). 31-72% would administer chemotherapy (CCNU, 17%; PCV, 8%; temozolomide, 19%; oral etoposide/trofosfamide, 8%), and 20-69% proposed immunotherapy (checkpoint inhibitors, 30%; tumor vaccines, 16%). Depending on the individual case, respondents would also include bevacizumab (6-18%), HDAC inhibitors (4-15%), tumor-treating fields (1-26%), and intraventricular chemotherapy (4-24%)., Conclusion: In each case, experts would combine conventional multimodal treatment concepts, including re-irradiation, with targeted therapy based on molecular genetic findings. International cooperative trials combining a (chemo-)therapy backbone with targeted therapy approaches for defined subgroups may help to gain valid clinical data and improve treatment in pediatric patients with recurrent/progressing HGG., (© 2023. The Author(s).)
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- 2023
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8. MR Imaging and Clinical Characteristics of Diffuse Glioneuronal Tumor with Oligodendroglioma-like Features and Nuclear Clusters.
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Benesch M, Perwein T, Apfaltrer G, Langer T, Neumann A, Brecht IB, Schuhmann MU, Cario H, Frühwald MC, Vollert K, van Buiren M, Deng MY, Seitz A, Haberler C, Mynarek M, Kramm C, Sahm F, Robe PA, Dankbaar JW, Hoff KV, Warmuth-Metz M, and Bison B
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- Humans, Child, Magnetic Resonance Imaging, Oligodendroglioma diagnostic imaging, Oligodendroglioma surgery, Glioma pathology, Central Nervous System Neoplasms pathology, Neoplasms, Neuroepithelial, Brain Neoplasms diagnostic imaging, Brain Neoplasms therapy
- Abstract
Background and Purpose: Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) is a new, molecularly defined glioneuronal CNS tumor type. The objective of the present study was to describe MR imaging and clinical characteristics of patients with DGONC., Materials and Methods: Preoperative MR images of 9 patients with DGONC (median age at diagnosis, 9.9 years; range, 4.2-21.8 years) were reviewed., Results: All tumors were located superficially in the frontal/temporal lobes and sharply delineated, displaying little mass effect. Near the circle of Willis, the tumors encompassed the arteries. All except one demonstrated characteristics of low-to-intermediate aggressiveness with high-to-intermediate T2WI and ADC signals and bone remodeling. Most tumors ( n = 7) showed a homogeneous ground-glass aspect on T2-weighted and FLAIR images. On the basis of the original histopathologic diagnosis, 6 patients received postsurgical chemo-/radiotherapy, 2 were irradiated after surgery, and 1 patient underwent tumor resection only. At a median follow-up of 61 months (range, 10-154 months), 6 patients were alive in a first complete remission and 2 with stable disease 10 and 21 months after diagnosis. The only patient with progressive disease was lost to follow-up. Five-year overall and event-free survival was 100% and 86±13%, respectively., Conclusions: This case series presents radiomorphologic characteristics highly predictive of DGONC that contrast with the typical aspects of the original histopathologic diagnoses. This presentation underlines the definition of DGONC as a separate entity, from a clinical perspective. Complete resection may be favorable for long-term disease control in patients with DGONC. The efficacy of nonsurgical treatment modalities should be evaluated in larger series., (© 2022 by American Journal of Neuroradiology.)
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- 2022
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9. Immunological recovery following HLA-matched CD3+ TCR αß+/CD19+ depleted hematopoietic stem cell transplantation in children.
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Sperl D, Lang P, Benesch M, Bainschab A, Urban C, Wilfing R, Feuchtinger T, Döring M, Seitz C, Strenger V, Lackner H, Seidel MG, Perwein T, Handgretinger R, Sipurzynski S, Rosskopf K, and Schwinger W
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- Antigens, CD19, Child, Humans, Lymphocyte Depletion, Receptors, Antigen, T-Cell, alpha-beta, Transplantation Conditioning methods, Graft vs Host Disease, Hematopoietic Stem Cell Transplantation methods
- Abstract
Background: Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative option for children with both malignant and nonmalignant diseases. T-cell depletion techniques may result in reduced transplant-related mortality compared with unmanipulated grafts due to a lower incidence of GvHD., Methods: Immune recovery and outcome were analyzed in a cohort of 23 patients with malignant and nonmalignant diseases who received CD3+TCRαβ+ T- and B-cell-depleted allografts from matched donors after reduced-intensity or myeloablative conditioning. The median number of CD34+, CD3+TCRαβ+, and CD19+B-cells infused was 12.7 × 10
6 /kg, 16.8 × 103 /kg, and 96 × 103 /kg bodyweight., Results: With a median follow-up of 36 (range 1-73) months, overall survival and disease-free survival at 3 years were 65.2% and 60.8%. Eight patients died, six due to the underlying disease and two of extended visceral cGvHD. Immune reconstitution, disease-free, and overall survivals were similar compared with a historical cohort of 23 patients transplanted with matched unmanipulated bone marrow. A significant lower rate of higher grade (III-IV) aGvHD was observed in the manipulated HSCT group (8.7% vs. 26%; p = 0.001), whereas the incidence of cGvHD was equal., Conclusions: Our data suggest that this graft manipulation strategy could be a safe and effective alternative to conventional HSCT techniques in matched donors., (© 2022 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)- Published
- 2022
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10. Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates.
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Gielen GH, Baugh JN, van Vuurden DG, Veldhuijzen van Zanten SEM, Hargrave D, Massimino M, Biassoni V, Morales la Madrid A, Karremann M, Wiese M, Thomale U, Janssens GO, von Bueren AO, Perwein T, Nussbaumer G, Hoving EW, Niehusmann P, Gessi M, Kwiecien R, Bailey S, Pietsch T, Andreiuolo F, and Kramm CM
- Abstract
Background: The WHO Classification of Tumors of the Central Nervous System has undergone major restructuring. Molecularly defined diagnostic criteria were introduced in 2016 (revised 4th edition) and expanded in 2021 (5th edition) to incorporate further essential diagnostic molecular parameters. We investigated potential differences between specialists in perception of these molecularly defined subtypes for pediatric high-grade gliomas (pedHGG)., Methods: We designed a 22-question survey studying the impact of the revised 4th edition of the WHO classification on pedHGG. Data were collected and statistically analyzed to examine the spectrum of viewpoints and possible differences between neuro-oncologists and neuropathologists., Results: 465 participants from 53 countries were included; 187 pediatric neuro-oncologists (40%), 160 neuropathologists (34%), and 118 additional experts (26%). Neuro-oncologists reported issues with the introduction of molecularly defined tumor types, as well as the abolishment or renaming of established tumor entities, while neuropathologists did not to the same extent. Both groups indicated less relevant or insufficient diagnostic definitions were available in 2016. Reported issues were classified and assessed in the 2021 WHO classification and a substantial improvement was perceived. However, issues of high clinical relevance remain to be addressed, including the definition of clinical phenotypes for diffuse intrinsic pontine glioma and gliomatosis cerebri., Conclusions: Within the WHO classification of pediatric brain tumors, such as pedHGG, rapid changes in molecular characterization have been introduced. This study highlights the ongoing need for cross talk between pathologist and oncologist to advance the classification of pedHGG subtypes and ensure biological relevance and clinical impact., (© The Author(s) 2022. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)
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- 2022
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11. Transitioning to molecular diagnostics in pediatric high-grade glioma: experiences with the 2016 WHO classification of CNS tumors.
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Baugh JN, Gielen GH, van Vuurden DG, Veldhuijzen van Zanten SEM, Hargrave D, Massimino M, Biassoni V, Morales la Madrid A, Karremann M, Wiese M, Thomale U, Janssens GO, von Bueren AO, Perwein T, Hoving EW, Pietsch T, Andreiuolo F, and Kramm CM
- Abstract
Background: Pediatric neuro-oncology was profoundly changed in the wake of the 2016 revision of the WHO Classification of Tumors of the Central Nervous System. Practitioners were challenged to quickly adapt to a system of tumor classification redefined by molecular diagnostics., Methods: We designed a 22-question survey studying the impact of the revised WHO classification on pediatric high-grade glioma. The survey collected basic demographics, general attitudes, issues encountered, and opinions on pediatric subtypes. Participant answers were analyzed along socioeconomic lines utilizing the human development index (HDI) of the United Nations and membership in the group of seven (G7) world economic forum., Results: Four hundred and sixty-five participants from 53 countries were included, 187 pediatric neurooncologists (40%), 160 neuropathologists (34%), and 118 other experts (26%). When asked about pediatric high-grade glioma entities, participants from very high development countries preferred treating a patient based on genetic findings. Participants from high and medium development countries indicated using traditional histology and tumor location as mainstays for therapeutic decisions. Non-G7 countries tended to regard the introduction of molecularly characterized tumor entities as a problem for daily routine due to lack of resources., Conclusions: Our findings demonstrate an overall greater reliance and favorability to molecular diagnostics among very high development countries. A disparity in resources and access to molecular diagnostics has left some centers unable to classify pediatric high-grade glioma per the WHO classification. The forthcoming edition should strain to abate disparities in molecular diagnostic availability and work toward universal adaptation., (© The Author(s) 2021. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)
- Published
- 2021
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12. High frequency of disease progression in pediatric spinal cord low-grade glioma (LGG): management strategies and results from the German LGG study group.
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Perwein T, Benesch M, Kandels D, Pietsch T, Schmidt R, Quehenberger F, Bison B, Warmuth-Metz M, Timmermann B, Krauss J, Thomale UW, Kortmann RD, Driever PH, and Gnekow AK
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- Child, Disease Progression, Humans, Prospective Studies, Spinal Cord, Brain Neoplasms genetics, Brain Neoplasms therapy, Glioma genetics, Glioma therapy
- Abstract
Background: Knowledge on management of pediatric spinal cord low-grade glioma (LGG) is scarce., Methods: We analyzed clinical datasets of 128 pediatric patients with spinal LGG followed within the prospective multicenter trials HIT-LGG 1996 (n = 36), SIOP-LGG 2004 (n = 56), and the subsequent LGG-Interim registry (n = 36)., Results: Spinal LGG, predominantly pilocytic astrocytomas (76%), harbored KIAA1549-BRAF fusion in 14/35 patients (40%) and FGFR1-TACC1 fusion in 3/26 patients (12%), as well as BRAFV600E mutation in 2/66 patients (3%). 10-year overall survival (OS) and event-free survival (EFS) was 93% ± 2% and 38% ± 5%, respectively. Disseminated disease (n = 16) was associated with inferior OS and EFS, while age ≥11 years and total resection were favorable factors for EFS. We observed 117 patients following total (n = 24) or subtotal/partial resection (n = 74), biopsy (n = 16), or radiologic diagnosis only (n = 3). Eleven patients were treated first with chemotherapy (n = 9) or irradiation (n = 2). Up to 20.8 years after diagnosis/initial intervention, 73/128 patients experienced one (n = 43) or up to six (n = 30) radiological/clinical disease progressions. Tumor resections were repeated in 36 patients (range, 2-6) and 47 patients required nonsurgical treatment (chemotherapy, n = 20; radiotherapy, n = 10; multiple treatment lines, n = 17). Long-term disease control for a median of 6.5 (range, 0.02-20) years was achieved in 73/77 patients following one (n = 57) or repeated (n = 16) resections, and in 35/47 patients after nonsurgical treatment., Conclusions: The majority of patients experienced disease progression, even after years. Multiple interventions were required for more than a third, yet multimodal treatment enabled long-term disease control. Molecular testing may reveal therapeutic targets., (© The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
- Published
- 2021
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13. Management of children and adolescents with gray zone lymphoma: A case series.
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Perwein T, Lackner H, Ebetsberger-Dachs G, Beham-Schmid C, Zach K, Tamesberger M, Simonitsch-Klupp I, Lüftinger R, Dworzak M, Mann G, Benesch M, and Attarbaschi A
- Subjects
- Adolescent, Austria, Autografts, Female, Follow-Up Studies, Humans, Lymphoma, Large B-Cell, Diffuse diagnosis, Male, Mediastinal Neoplasms diagnosis, Retrospective Studies, Chemoradiotherapy, Lymphoma, Large B-Cell, Diffuse therapy, Mediastinal Neoplasms therapy, Stem Cell Transplantation
- Abstract
Background: Data on management of gray zone lymphoma (GZL) in children and adolescents are scarce., Procedure: This retrospective study assessed clinical characteristics and outcome in six Austrian patients with GZL less than 18 years of age (male-to-female ratio: 1:1; median age: 15.8 years)., Results: Two patients each had a classical Hodgkin lymphoma (cHL)-like and composite GZL subtype, and one patient each had a large B-cell non-Hodgkin lymphoma (LBCL)-like and sequential GZL subtype. All had advanced disease with mediastinal and extranodal involvement. Five patients received an LBCL- and one patient a cHL-directed polychemotherapy ± radiotherapy. Out of the former patients, three survived, including two who relapsed and underwent high-dose chemotherapy with autologous stem cell rescue. The latter patient survived., Conclusions: GZL remains a diagnostic and therapeutic challenge, necessitating the development of novel treatment concepts performed in a prospective setting., (© 2020 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals, Inc.)
- Published
- 2020
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14. Giant Pelvic Cyst in 16-Year-Old Boy With Bloody Diarrhea: Atypical Presentation of Colonic Adenocarcinoma.
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Kohlmaier B, Perwein T, Egger M, Zöhrer E, Sorantin E, Till H, Urban C, and Jahnel J
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- Adenocarcinoma complications, Adenocarcinoma surgery, Adolescent, Colectomy methods, Colonic Neoplasms complications, Colonic Neoplasms surgery, Cysts pathology, Cysts surgery, Humans, Intestinal Perforation complications, Intestinal Perforation surgery, Laparotomy methods, Magnetic Resonance Imaging, Male, Adenocarcinoma diagnosis, Colonic Neoplasms diagnosis, Diarrhea etiology, Gastrointestinal Hemorrhage etiology
- Published
- 2017
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15. Survival and late effects in children with stage 4 neuroblastoma.
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Perwein T, Lackner H, Sovinz P, Benesch M, Schmidt S, Schwinger W, and Urban C
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- Antineoplastic Agents therapeutic use, Child, Child, Preschool, Disease-Free Survival, Female, Humans, Incidence, Infant, Kaplan-Meier Estimate, Male, Neoplasm Staging, Neoplasms, Second Primary epidemiology, Neuroblastoma pathology, Neuroblastoma therapy, Radiotherapy adverse effects, Retrospective Studies, Survivors, Neuroblastoma complications, Neuroblastoma mortality
- Abstract
Background: Treatment of metastatic neuroblastoma (NB) demands aggressive oncological therapy, which may cause long-term sequelae in survivors. The aim of this retrospective single center study is to give an overview of survival in children with stage 4 NB and to describe the spectrum of late effects seen in survivors., Procedure: Medical records of 31 patients with stage 4 NB treated between 1984 and 2009, who were included in a follow-up programme, were reviewed for information on tumor, treatment and late effects., Results: Five-year overall survival was 54.3 ± 9% and 5-year event-free survival was 44.9 ± 9%. Patients diagnosed after 1996 had a significantly better survival rate than those diagnosed before (74 ± 11.2% vs. 33.3 ± 12.2%, P = 0.011). In 15 of the 16 survivors (93.8%), numerous late effects were detected. The most common long-term sequelae were renal changes in 10 patients (62.5%) and endocrine disturbances in 9 patients (56.3%), including hypothyroidism with need of substitution in 50%, GH deficiency in 37.5% and hypogonadism in 12.5%. Sensorineural hearing loss occurred in 37.5% of survivors. Further observed late effects were hepatobiliary changes (31.3%), musculoskeletal problems, and pulmonary abnormalities (each 25%), as well as neurologic changes (18.8%), dental defects (12.5%), and unilateral blindness (6.3%). Second neoplasms appeared in 3 patients, 1 of whom died of hepatocellular carcinoma following infection with hepatitis B., Conclusions: More than 50% of children with stage 4 NB may survive. The high incidence of severe long-term sequelae underlines the importance of careful follow-up in order to detect and treat late effects early enough., (Copyright © 2011 Wiley-Liss, Inc.)
- Published
- 2011
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