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  • 1
    Electronic Resource
    Electronic Resource
    Allelic loss of the short arm of chromosome 4 in neuroblastoma suggests a novel tumour suppressor gene locus (1996)
    Springer
    Human genetics 〈Berlin〉 97 (1996), S. 834-837 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Neuroblastoma is a childhood neural crest tumour, genetically characterized by frequent deletions of the short arm of chromosome 1 and amplification of N-myc. Here we report the first evidence for a neuroblastoma tumour suppressor locus on 4pter. Cytogenetically we demonstrated rearrangements of 4p in 7 out of 26 evaluable tumours (27%). Subsequent analysis of loss of heterozygosity (LOH) by Southern blotting revealed allelic loss of 4p in 16/82 (19.5%) informative neuroblastomas. Taken together cytogenetic and Southern blot analyses showed loss of 4p in 20/86 neuroblastomas analysed (23%). The common deleted region was bordered by the probe D4S 123 and encompassed the distal 34 cM of 4p. We found no evidence for genomic imprinting of the 4p locus as the 4p alleles lost in the tumours were of random maternal and paternal origin. LOH4p was found at all disease stages and in every age group. Furthermore LOH4p was present both in cases with and without LOHIp and amplification of N-myc.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02346199
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  • 2
    Electronic Resource
    Electronic Resource
    Allelic loss of the short arm of chromosome 4 in neuroblastoma suggests a novel tumour suppressor gene locus (1996)
    Springer
    Human genetics 〈Berlin〉 97 (1996), S. 834-837 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Neuroblastoma is a childhood neural crest tumour, genetically characterized by frequent deletions of the short arm of chromosome 1 and amplification of N-myc. Here we report the first evidence for a neuroblastoma tumour suppressor locus on 4pter. Cytogenetically we demonstrated rearrangements of 4p in 7 out of 26 evaluable tumours (27%). Subsequent analysis of loss of heterozygosity (LOH) by Southern blotting revealed allelic loss of 4p in 16/82 (19.5%) informative neuroblastomas. Taken together cytogenetic and Southern blot analyses showed loss of 4p in 20/86 neuroblastomas analysed (23%). The common deleted region was bordered by the probe D4S123 and encompassed the distal 34 cM of 4p. We found no evidence for genomic imprinting of the 4p locus as the 4p alleles lost in the tumours were of random maternal and paternal origin. LOH4p was found at all disease stages and in every age group. Furthermore LOH4p was present both in cases with and without LOH1p and amplification of N-myc.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050146
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  • 3
    Electronic Resource
    Electronic Resource
    Reconstructive surgery as part of the AMORE protocol in the treatment of pediatric head and neck soft tissue sarcoma (2000)
    Springer
    European journal of plastic surgery 23 (2000), S. 168-173 
    Details
    ISSN: 1435-0130
    Keywords: Key words Soft tissue sarcoma ; Rhabdomyosarcoma ; AMORE protocol ; Reconstructive surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  For advanced stage residual or recurrent paediatric soft tissue sarcomas in the head and neck area, the AMORE protocol was developed in our hospital. It consists of Ablative surgery, afterloading brachytherapy, using a MOulage technique and REconstructive surgery in 1 week. The reconstructive surgical component of this treatment regimen was reviewed. Between January 1993 and November 1999, 18 children with soft tissue sarcomas in the head and neck area were treated according to the AMORE protocol. The median follow-up was 31.5 months (range 2 months to 6.5 years). For reconstruction, a free vascularized muscle transfer was performed in 12 patients and a pedicled muscle transposition in six patients. The surgical reconstruction was successful in all but one patient and in 16 of 18 patients, healing was uneventful. Since the introduction of this multidisciplinary approach in the treatment of paediatric soft tissue sarcomas in the head and neck region, the results have been promising. The immediate reconstruction of an irradiated, contaminated wound bed with uncompromised, well-vascularized muscle tissue has, in general, resulted in excellent wound healing. The treatment was undertaken in a limited amount of time and resulted in an acceptable morbidity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002380050242
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  • 4
    Electronic Resource
    Electronic Resource
    Treatment of rhabdomyosarcoma and other malignant mesenchymal tumours of childhood with ifosfamide + vincristine + dactinomycin (IVA) as front-line therapy (1989)
    Springer
    Cancer chemotherapy and pharmacology 24 (1989), S. S30 
    Details
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00253236
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  • 5
    Electronic Resource
    Electronic Resource
    The role of ifosfamide in paediatric soft tissue sarcomas (1986)
    Springer
    Cancer chemotherapy and pharmacology 18 (1986), S. S23 
    Details
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Early clinical trials in adults showed favourable results of ifosfamide (IF) in several tumours. In a previous study we used IF and vincristine (VCR) and observed 6 complete responses (CR) among 25 previously heavily treated children. Especially patients with rhabdomyosarcoma (RMS) responded well, with 4 partial responses (PR) and 2 CR among 6 patients. This and the fact that the combination of VCR, actinomycin D (ACD) and cyclophosphamide (CYT) still give good results in RMS patients led us to replace CYT with IF in this combination. This IVA protocol consists of IF 3000 mg/m2 i.v. in 1 h on days 1 and 2, VCR 1.5 mg/m2 by i.v. push on day 1, and ACD 900 μg/m2 by i.v. push on days 1 and 2. The course is repeated at 28-day intervals. VCR 1.5 mg/m2 is given on day 14. We used this protocol als induction therapy in 18 newly diagnosed RMS patients. The primary sites were: abdomen (4 patients), bladder and prostate (3), head and neck (9: orbita 5), extremity (1), chest wall (1). At diagnosis, 9 patients had stage I, 4 stage II and 5 stage IV disease. Except for one mixed mesodermal type, all were of the embryonal type. The patients age at diagnosis varied from 2 to 16 years. After three courses, in some patients surgery was performed if radical tumour extirpation seemed possible. The first evaluation took place after three courses. At this point one patient had no response, one had a reduction in tumour mass of 〈50% (partial response PR), 9 patients had 〉50% tumour-reduction (good partial response [GPR]), and 7 were in complete remission (CR). In 2 of these 7 (CR) no tumour was found on histopathological examination of the specimen. In patients with GPR or CR therapy was continued for 6 months and then stopped. Thirteen patients have been disease-free for 1–20 months from the date of CR. Four patients relapsed 4, 6, 11 and 11 months after CR. There was one therapy-related death. Except for this patient no major toxicity was encountered. These results indicate that by replacing CYT with IF, remission induction can be improved with a major contribution to survival and to enhanced quality of life in these patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00647445
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  • 6
    Electronic Resource
    Electronic Resource
    Closing remarks (1989)
    Springer
    Cancer chemotherapy and pharmacology 24 (1989), S. S51 
    Details
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00253242
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  • 7
    Electronic Resource
    Electronic Resource
    Peripheral primitive neuroectodermal tumour and extra-osseous Ewing's sarcoma; a histological, immunohistochemical and DNA flow cytometric study (1995)
    Springer
    Virchows Archiv 425 (1995), S. 611-616 
    Details
    ISSN: 1432-2307
    Keywords: Peripheral primitive neuroectodermal tumour ; Extra-osseous Ewing's sarcoma ; Schmidt classification scheme ; DNA flow cytometry ; Mitotic activity index
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Although peripheral primitive neuroectodermal tumour (pPNET) and extra-osseous Ewing's sarcoma (EES) are thought to be closely related neoplasms, their clinical behaviour differs considerably. To determine the clinical relevance of the Schmidt classification scheme for differentiating pPNET and EES, 20 tumour specimens of poorly differentiated round cell tumours were evaluated. In addition, the diagnostic value of several neural markers and the prognostic value of quantitative morphological variables (DNA ploidy, S-phase fraction, and the mitotic activity) were assessed. Homer-Wright rosettes were present in 9 tumours. Neuron specific enolase (NSE) was expressed in 11 tumours, 8 of which expressed a second neural marker (CD57, S100, or neurofilament). According to the Schmidt classification, 11 pPNET and 5 EES were distinguished. HBA-71 was exclusively expressed in pPNET and EES. The remaining tumours were classified as sarcoma not otherwise specified (n=2), rhabdomyosarcoma (n=1), and desmoplastic tumour with divergent differentiation (n=1). EES611 patients fared significantly better than the pPNET patients (100% versus 42% 5-year survival). Neither DNA ploidy nor S-phase fraction assessed in 12 evaluative histograms (9 pPNET and 3 EES), nor mitotic activity yielded information of additional prognostic value. On the basis of this study and the Schmidt classification scheme, it can be concluded that if the diagnosis of EES and pPNET is based on light microscopy (Homer-Wright rosettes) and/or immunohistochemistry (at least two neural markers, i.e. NSE, S-100, CD57, and neurofilament), the classification provides important clinical information. Furthermore, positivity for HBA-71 is helpful in differentiating pPNET and EES from all other small round cell tumours.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00199351
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  • 8
    Electronic Resource
    Electronic Resource
    Selective decontamination of the digestive tract in oncological paediatric patients (1982)
    Springer
    Antonie van Leeuwenhoek 48 (1982), S. 500-501 
    Details
    ISSN: 1572-9699
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00448433
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  • 9
    Electronic Resource
    Electronic Resource
    A phase II study of cisplatin, ifosfamide and doxorubicin in operable primary, axial skeletal and metastatic osteosarcoma (1999)
    Springer
    Annals of oncology 10 (1999), S. 1211-1218 
    Details
    ISSN: 1569-8041
    Keywords: chemotherapy ; osteosarcoma ; relative dose intensity ; survival ; tumour response
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: Despite advances in the treatment of primary limb osteosarcoma, the outcome of patients with primary metastatic and axial skeletal disease remains poor. The European Osteosarcoma Intergroup have assessed a combination chemotherapy regimen consisting of ifosfamide (IFOS) 3 g/m2/d1–2, doxorubicin (DOX) 25 mg/m2/d1–3 i.v. bolus and cisplatin (CDDP) 100 mg/m2/d1. Patients and methods: One hundred nine previously untreated patients with primary osteosarcoma were registered. Eligibility was confirmed in 103. At presentation, 45 eligible patients had metastatic disease, 15 axial skeletal primary tumours and 43 non-metastatic limb tumours. Results: The major toxicities were myelosuppression (90%, grade 3 or 4) and nausea and vomiting (74%, grade 3 or 4). Overall mean relative dose intensity (RDI) was 80% (88% CDDP, 75% IFOS, 81% DOX). Clinical response as measured by reduction in tumour volume occurred in 36% (95% confidence interval (95% CI): 27%–47%) of primary tumours. Response of pulmonary metastases to chemotherapy was seen in 33% (95% CI: 19%–49%). Good histological response (≥90% necrosis of the tumour) occurred in 33% (95% CI: 22%–45%) of resected tumours. Five-year survival was 62% in limb-non-metastatic, 41% in axial skeletal and 16% in limb metastatic patients. Conclusions: This regimen is active in osteosarcoma but does not appear to be more active than the two-drug CDDP–DOX regimen currently recommended by EOI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1008361612767
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  • 10
    Electronic Resource
    Electronic Resource
    Formiminoglutamic/hydantoinpropionic aciduria in three patients with different tumours (1994)
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 642-643 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00711613
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