Introduction:Preoperative chemotherapy is recommended for children with Wilms tumour with intravascular extension. Extended chemotherapy may improve resectability, but increase tumour adherence to vascular endothelium, precluding complete resection. To evaluate the optimal length of preoperative treatment, we report a two-part review comprising systematic review of the literature and investigation of patients treated in the International Society of Paediatric Oncology (SIOP) WT 2001 trial. Methods: Studies were identified using Medline and Embase databases from 1996 to present. English language titles reporting management of intravascular Wilms tumour were analysed. Patients with Wilms tumour and thrombus were identified from the SIOP WT 2001 trial. Overall survival (OS) and event-free survival (EFS), tumour regression, completeness of resection and cavectomy were investigated. Results: The search retrieved 43 articles documenting 498 children. Note that 72% of the patients received neoadjuvant chemotherapy: 101 received standard course (4-6 weeks, standard course neoadjuvant chemotherapy [StC]) and 62 extended course (> 6 weeks, extended course neoadjuvant chemotherapy [EC]). There was no significant difference between the groups in terms of thrombus regression or completeness of resection. EFS was greater in the StC group (78 vs 54%; P = .04). Of 4511 patients registered in the SIOP WT 2001 trial, 166 had thrombus. Note that 97% of the patients received neoadjuvant chemotherapy: 63 StC and 67 EC. There was no significant difference between the groups with regard to tumour regression, complete resection, or cavectomy. Survival was significantly higher in those receiving StC than EC (OS: 95% vs 82%, P = .025; EFS: 88% vs 72%, P = .047). Conclusion:There is no evidence that prolonged courses of neoadjuvant chemotherapy beyond the recommended protocols confer any additional benefit in treating intravascular extension of Wilms tumour.

Abstract. expression profiling of tumor tissue allows a systematic search for targeted therapies and offers relevant prognostic information. Molecular studies on rhabdomyosarcoma (RMS) revealed a more differentiated classification than the histological subgrouping into embryonal (rMe) and alveolar (RMA) rhabdomyosarcoma, and reflected the chromosomal aberrations found in rMS. We addressed biological processes like cell migration and emerging drug resistance by expression profiling to identify mechanisms of metastasic invasion and differential response to chemotherapy in rMS. gene expression analysis was performed in 19 rMS samples using the affymetrix u133 Plus2 array. Validation of target genes was performed by qrt-Pcr. Data were analyzed using Pathway analysis software. involvement of these genes in invasion processes was evaluated in knock-down experiments using specific interference rna and Matrigel tM invasion assay. in rMa tissues 211 of 534 genes were overexpressed, in rMe tissues 323 genes were overexpressed. Pathway analysis software identified a group of genes involved in cell growth, morphology and motility. in patients with distant metastases especially transcription factors such as foXf1 and lMo4 showed a high expression, which were described as determinants of tumor cell migration. Down-regulation of these factors inhibited the invasion of rMS cells >10-fold. Microarray technology is a powerful method not only to classify rMS samples, but also to identify major regulatory processes. functional evaluation of lMo4 and foXf1 identified targets of a molecular network for preventing metastatic invasion in rMS.

This article describes the current status of surgical approach to Wilms tumor. Combined multimodal treatment including classical open nephrectomy is still the most recommended and offers excellent survivals. Patients suffering from Wilms tumor as potentially very long-term survivors also need great care of quality of their further life. Nephron-sparing surgery seems an important step in this regard; however, selection of patients is necessary to avoid failures and decreasing survival rate. A new method of describing this kind of procedure developed within the SIOP Renal Tumours Study Group (RTSG) offers an opportunity for further comparisons and assessment. Minimally invasive nephrectomy is not recommended in the treatment of Wilms tumor; however, in experienced hands and correctly selected (rare) cases, minimally invasive nephrectomy may offer the same outcome as the classical open approach. Lymph nodes sampling, essential for reliable staging, appeared rarely correct in children operated by this technique. Any competition with partial nephrectomy should be avoided in favor of the nephron-sparing approach. A small proportion of patients still create surgical difficulties and this is in fact the target group for further surgical reviews. Their prognosis seems at least in part surgeon-dependent. Few clinical factors available preoperatively (tumor side, age, and tumor volume) were detected as influencing potential risk of surgical failures. Once identified, this needs increased attention from the surgical point of view.

The purpose of the study was to present a modern classification and discuss the treatment of midfacial fractures in children. From the beginning of 1 January 1998 to 31 October 2000, 147 children were treated for different craniofacial fractures. Among them 28 patients (19 %) had extensive midfacial fractures complicated by impaired vision and/or CNS dysfunction and were treated surgically. The fractures were divided into: zygomatico-orbital (1 pt) and zygomatico-orbito-maxillary (5 pts), isolated orbital wall fractures (14 pts) and naso-orbital dislocations (2 pts), upper facial portion dislocations (2 pts) and fronto-naso-orbital (2 pts) or cranio-orbital fractures (2 pts). Clinical examination revealed mainly dysfunction of facial morphology and aesthetics (enophthalmos, telecanthus), CSF leakage, impairment of vision and restricted eyeball movements. Ophthalmological, neurological and radiological (Waters view, CT, NMR) examinations were performed in all cases. The treatment consisted of bone repositioning, reconstruction with autologous bone grafts and three-dimensional stabilisation using titanium plates. Twenty-three pts were operated on before and 5 pts after 7th day after trauma. The restoration of normal facial morphology was achieved in 26 patients. In 2 cases it was impossible because of the destruction of soft tissue and an extremely extensive bony trauma. Better reposition of bony fragments, prompt healing and better final results were achieved in patients operated before day 7 after trauma. Achieving good results in midfacial fractures requires prompt and adequate treatment and a multidisciplinary (paediatric, maxillofacial, plastic surgeons, neurosurgeon, ophthalmologist) team.

No abstract