Godzinski J.,Marciniak Hospital |
Godzinski J.,Wroclaw Medical University
Journal of Indian Association of Pediatric Surgeons | Year: 2014
This paper attempts to briefly describe the International Society of Paediatric Oncology (SIOP) policy of treatment of nephroblastoma since first study (SIOP 1) launched in 1971 until today. It focuses on the advantages of the preoperative chemotherapy and the stratification of patients induced this way. Marked efficacy of the pretreatment opened the way for less aggressive surgical management also in case of the "so-called" regular unilateral cases: Nephron-sparing surgery and minimal invasive techniques will probably find its place in this field of pediatric surgical oncology; however, very careful selection of cases must be the priority.
G-CSF therapy and catheter-related Gram-positive sepsis increase serum IL-2 receptor α level and may falsely suggest a relapse in children with soft tissue sarcomas unless serum beta2-microglobulin, lactate dehydrogenase and C-reactive protein levels are determined concomitantly
Bien E.,Medical University of Gdansk |
Balcerska A.,Medical University of Gdansk |
Niedzwiecki M.,Medical University of Gdansk |
Krawczyk M.,Medical University of Gdansk |
And 3 more authors.
Cytokine | Year: 2011
Many components of oncologic treatment increase serum sIL-2Rα level, which may falsely suggest a relapse. We tried to establish whether granulocyte colony stimulating factor (G-CSF) and central vein catheter (CVC)-related sepsis increase serum sIL-2Rα level to values on relapse of childhood soft tissue sarcomas (STS) and how to distinguish real relapse from a "false" one. Serum sIL-2Rα, B2-M, LDH, CRP and ESR levels and rates of markers' elevated values were determined prospectively in 18 STS children: pre-treatmently (ST1), in complete remission (CR; ST2), in CR during G-CSF therapy (ST3), in CR during CVC-related sepsis (ST4), on relapse (ST5) and after treatment (ST6) and once in 50 healthy pediatric controls. It appeared that pre-treatment serum sIL-2Rα, LDH, CRP and ESR but not B2-M declined significantly with remission (ST2) achievement. At ST5 sIL-2Rα, B2-M, LDH and CRP increased from ST2 to ST1 values. SIL-2Rα levels at ST3 and ST4 rose significantly in all patients from ST2 to ST1 and ST5 values. At ST3 also serum LDH and B2-M increased to values at ST1 and ST5 and exceeded significantly those at ST2 and ST4. At ST4 CRP but not B2-M and LDH, rose significantly in most patients to values at ST1 and ST5. Thus, serum sIL-2Rα monitoring in pediatric STS reflects well response to chemotherapy unless samples are collected during G-CSF therapy or CVC-related sepsis. Determination of serum B2-M, LDH and CRP together with sIL-2Rα may help to distinguish between "real" relapse and "false" sIL-2Rα increase due to G-CSF administration or CVC-related sepsis. © 2011 Elsevier Ltd.
Godzinski J.,Marciniak Hospital |
Godzinski J.,Wroclaw Medical University |
Graf N.,Homburg University |
Audry G.,University Pierre and Marie Curie
European Journal of Pediatric Surgery | Year: 2014
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. © 2014 Georg Thieme Verlag KG Stuttgart, New York.
Graf N.,Saarland University |
Van Tinteren H.,Netherlands Cancer Institute |
Bergeron C.,Institute dhemato oncologie pediatrique |
Pein F.,InstitutGustave Roussy |
And 7 more authors.
European Journal of Cancer | Year: 2012
Purpose: To determine the prognosis of children with stage II and III of low or intermediate risk histology (SIOP classification) in unilateral localised Wilms tumour (WT) after neoadjuvant chemotherapy according to the trial and study of the International Society of Paediatric Oncology, SIOP 93-01. Patients and methods: Patients with unilateral localised WT and stage II or III with low (LR) or intermediate risk (IR) histology between 6 months and 18 years of age, were selected from the total sample of patients registered in the SIOP 93-01 study between June 1993 and December 2001. All patients received 4 weeks of actinomycin-D/vincristine before surgery. Postoperative chemotherapy consisted of actinomycin-D, vincristine and epirubicin/doxorubicin for 27 weeks. Flank or whole abdomen irradiation was given for stage III. Event-free survival (EFS) and overall survival (OS) were analysed for various subgroups. Results: Of 1476 registered patients 594 (40%) met the inclusion criteria for this analysis. Four hundred and two (67%) had stage II disease and 563 (95%) had intermediate risk histology. Median tumour volume was 439 ml at diagnosis and 163 ml after preoperative chemotherapy. With a median follow-up of 8 years, 5-year EFS was 90% (95% confidence interval [95% CI]: 87-92%) and OS 95% (95% CI: 93-97%). Patients with stage III, blastemal type histology and a large volume at surgery had a worse outcome. Conclusion: Treatment for stage II and III LR or IR WT is successful in a neoadjuvant setting as advised by the SIOP. Stage, tumour volume and blastemal type histology are the most important prognostic factors. © 2012 Elsevier Ltd. All rights reserved.
Armeanu-Ebinger S.,University of Tubingen |
Bonin M.,University of Tubingen |
Habig K.,University of Tubingen |
Poremba C.,University Hospital |
And 6 more authors.
International Journal of Oncology | Year: 2011
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™ 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.