Publications (8)84.97 Total impact
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Article: Prognostic stratification for children with hepatoblastoma: the SIOPEL experience.
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ABSTRACT: To identify factors relevant to long-term outcome in newly diagnosed hepatoblastoma, and define subgroups for clinical research on tailoring treatment to the individual patient. Between 1995 and 2006 the SIOPEL group conducted two clinical trials which established risk-adapted therapy for hepatoblastoma patients. Patients were stratified into high-risk (AFP < 100 ng/mL and/or PRETEXT IV and/or vascular invasion and/or extra-hepatic intra-abdominal disease (V+/P+/E+) and/or metastases) and standard-risk (all others). The hierarchy of these factors plus multifocality, PRETEXT III, AFP > 1,200,000 ng/mL, patient age, platelet count and histology were further explored. The outcome measure was event-free survival (EFS). In 541 patients, reduced EFS correlated significantly with AFP < 100 ng/ml (hazard ratio [HR] 4.09, 95% confidence interval 2.16-7.75), AFP ≥ 1.2 × 10(6)ng/mL (2.48, 1.47-4.17), metastatic disease (3.02, 2.05-4.44), PRETEXT IV (2.15, 1.19-3.87), multifocality (1.59, 1.01-2.50), age > 5 years (2.76, 1.68-4.53); borderline with small cell undifferentiated (SCU) histology (2.29, 95% confidence interval 0.91-5.77); but not with PRETEXT III, age 30-60 months, platelet count or V+/P+/E+. By using the significant factors and SCU to stratify the population, we have identified three distinct prognostic groups: PRETEXT I/II/III, and no other factors, have 3 year EFS of 90%, PRETEXT IV and/or multifocal tumour and/or age> 5 years and/or AFP > 1.2 × 10(6) have 3 year EFS of 71% and SCU and/or AFP < 100 ng/mL and/or metastatic have a 3year EFS of 49%. Prognostic stratification for clinical research on newly diagnosed hepatoblastoma should take into consideration PRETEXT, metastatic disease, AFP, multifocality, age and SCU histology.European journal of cancer (Oxford, England: 1990) 01/2012; 48(10):1543-9. · 4.12 Impact Factor -
Article: Successful treatment of childhood high-risk hepatoblastoma with dose-intensive multiagent chemotherapy and surgery: final results of the SIOPEL-3HR study.
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ABSTRACT: The primary objective was to determine the efficacy of a newly designed preoperative chemotherapy regimen in an attempt to improve the cure rate of children with high-risk hepatoblastoma. High risk was defined as follows: tumor in all liver sections (ie, Pretreatment Extension IV [PRETEXT-IV]), or vascular invasion (portal vein [P+], three hepatic veins [V+]), or intra-abdominal extrahepatic extension (E+), or metastatic disease, or alpha-fetoprotein less than 100 ng/mL at diagnosis. Patients were treated with alternating cycles of cisplatin and carboplatin plus doxorubicin (preoperatively, n = 7; postoperatively, n = 3) and delayed tumor resection. Of the 151 patients (150 evaluable for response) 118 (78.7%) achieved a partial response to chemotherapy. Complete resection of the liver tumor could be achieved in 115 patients (76.2%) either by partial hepatectomy (55.6%) or by liver transplantation (20.6%). In 106 children (70.2%), complete resection of all tumor lesions (including metastases) was achieved. Among the patients with initial lung metastases, 52.2% achieved complete remission of the lung lesions with chemotherapy alone. In half of the patients with initial PRETEXT-IV tumor as the only high-risk feature, the tumor could be completely resected with partial hepatectomy. Event-free (EFS) and overall survival (OS) estimates at 3 years were 65% (95% CI, 57% to 73%) and 69% (95% CI, 62% to 77%) for the whole group. EFS and OS for all patients with PRETEXT-IV tumor were 68% and 69%, respectively, and they were 56% and 62%, respectively, for patients with metastasis. The applied treatment rendered a great proportion of tumors resectable, and, in comparison with previously published results, led to an improved survival in patients with high-risk hepatoblastoma.Journal of Clinical Oncology 05/2010; 28(15):2584-90. · 18.37 Impact Factor -
Article: Laser-guided repair of complex bile duct strictures.
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ABSTRACT: The repair of bile duct strictures (BDS) requires identification of healthy bile duct proximal to the stenosis. Identification may be difficult in complex bile duct injuries after cholecystectomy or partial liver resection. We describe a technique to identify the prestenotic bile duct using the sentinel light of a laser fiber passed through the catheter after percutaneous transhepatic biliary drainage (PTD). Seven patients were seen with hepatic duct or segmental BDS after (extended) right hemihepatectomy (4), cholecystectomy (2) or previous hepaticojejunostomy (1). All patients underwent preoperative PTD for imaging of stricture site and drainage. During operation for repair, a laser fiber (0.2-0.6 mm) connected to a (low-power) red light-emitting diode laser was passed through the PTD catheter into the proximal end of the stricture. In 6 patients, the prestenotic bile duct was identified and exposed with the aid of the sentinel light of the laser fiber. Roux-en-Y hepaticojejunostomy was carried out in all patients. Postoperative cholangiographies showed complete restoration of biliary continuity. In one patient, positioning of the laser fiber failed because of angulation of the PTD catheter. Laser-guided identification of prestenotic bile duct facilitates exposure of the stricture site without unnecessary and usually difficult dissection in complex postoperative BDS.Digestive surgery 11/2009; 26(5):358-63. · 1.37 Impact Factor -
Article: Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma.
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ABSTRACT: Preoperative cisplatin alone may be as effective as cisplatin plus doxorubicin in standard-risk hepatoblastoma (a tumor involving three or fewer sectors of the liver that is associated with an alpha-fetoprotein level of >100 ng per milliliter). Children with standard-risk hepatoblastoma who were younger than 16 years of age were eligible for inclusion in the study. After they received one cycle of cisplatin (80 mg per square meter of body-surface area per 24 hours), we randomly assigned patients to receive cisplatin (every 14 days) or cisplatin plus doxorubicin administered in three preoperative cycles and two postoperative cycles. The primary outcome was the rate of complete resection, and the trial was powered to test the noninferiority of cisplatin alone (<10% difference in the rate of complete resection). Between June 1998 and December 2006, 126 patients were randomly assigned to receive cisplatin and 129 were randomly assigned to receive cisplatin plus doxorubicin. The rate of complete resection was 95% in the cisplatin-alone group and 93% in the cisplatin-doxorubicin group in the intention-to-treat analysis (difference, 1.4%; 95% confidence interval [CI], -4.1 to 7.0); these rates were 99% and 95%, respectively, in the per-protocol analysis. Three-year event-free survival and overall survival were, respectively, 83% (95% CI, 77 to 90) and 95% (95% CI, 91 to 99) in the cisplatin group, and 85% (95% CI, 79 to 92) and 93% (95% CI, 88 to 98) in the cisplatin-doxorubicin group (median follow-up, 46 months). Acute grade 3 or 4 adverse events were more frequent with combination therapy (74.4% vs. 20.6%). As compared with cisplatin plus doxorubicin, cisplatin monotherapy achieved similar rates of complete resection and survival among children with standard-risk hepatoblastoma. Doxorubicin can be safely omitted from the treatment of standard-risk hepatoblastoma. (ClinicalTrials.gov number, NCT00003912.)New England Journal of Medicine 10/2009; 361(17):1662-70. · 53.30 Impact Factor -
Article: Has the liver and other visceral organs migrated to its normal position in children with giant omphalocele? A follow-up study with ultrasonography.
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ABSTRACT: This study evaluates whether, on the long run, in patients born with a giant omphalocele, the liver and other solid organs reach their normal position, shape, and size. Seventeen former patients with a giant omphalocele, treated between 1970 and 2004, were included. Physical examination was supplemented with ultrasonography for ventral hernia and precise description of the liver, spleen, and kidneys. The findings were compared with 17 controls matched for age, gender, and body mass index. We found an abnormal position of the liver, spleen, left kidney, and right kidney in eight, six, five, and four patients, respectively. An unprotected liver was present in all 17 patients and in 11 controls, the difference being statistically significant (p = 0.04). In ten of the 11 patients with an incisional hernia, the liver was located underneath the abdominal defect. CONCLUSION: In all former patients with a giant omphalocele, an abnormal position of the liver and in the majority of them, an incisional hernia was also found. The liver and sometimes also the spleen and the kidneys do not migrate to their normal position. Exact documentation and good information are important for both the patient and their caretakers in order to avoid liver trauma.European Journal of Pediatrics 09/2009; 169(5):563-7. · 1.88 Impact Factor -
Article: Risk factors for abdominal wound dehiscence in children: a case-control study.
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ABSTRACT: In the limited literature concerning abdominal wound dehiscence after laparotomy in children, reported incidences range between 0.2-1.2% with associated mortality rates of 8-45%. The goal of this retrospective case-control study was to identify major risk factors for abdominal wound dehiscence in the pediatric population. Patients younger than aged 18 years who developed abdominal wound dehiscence in three pediatric surgical centers during the period 1985-2005 were identified. For each patient with abdominal wound dehiscence, four controls were selected by systematic random sampling. Patients with (a history of) open abdomen treatment or abdominal wound dehiscence were excluded as control subjects. Putative relevant patient-related, operation-related, and postoperative variables for both cases and control subjects were evaluated in univariate analyses and subsequently entered in multivariate stepwise logistic regression models to identify major independent predictors of abdominal wound dehiscence. A total number of 63 patients with abdominal wound dehiscence and 252 control subjects were analyzed. Mean presentation of abdominal wound dehiscence was at postoperative day 5 (range, 1-15) and overall mortality was 11%. Hospital stay was significantly longer (p < 0.001) in the case group (median, 42 vs. 10 days). Major independent risk factors for abdominal wound dehiscence were younger than aged 1 year, wound infection, median incision, and emergency surgery. Incisional hernia was reported in 12% of the patients with abdominal wound dehiscence versus 3% in the control group (p = 0.001). Abdominal wound dehiscence is a serious complication with high morbidity and mortality. Median incisions should be avoided whenever possible.World Journal of Surgery 05/2009; 33(7):1509-13. · 2.36 Impact Factor -
Article: Respiratory symptoms in a boy revealing Carney triad.
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ABSTRACT: The association of gastrointestinal stromal cell tumor (GIST), paraganglioma, and pulmonary chondroma is known as the Carney triad, occurring predominantly in young adult females. We present the case of a 14-year-old male with respiratory symptoms resulting in the diagnosis Carney triad.Pediatric Blood & Cancer 03/2008; 50(2):399-401. · 1.89 Impact Factor -
Article: 2005 PRETEXT: a revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group.
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ABSTRACT: Over the last 15 years, various oncology groups throughout the world have used the PRETEXT system for staging malignant primary liver tumours of childhood. This paper, written by members of the radiology and surgery committees of the International Childhood Liver Tumor Strategy Group (SIOPEL), presents various clarifications and revisions to the original PRETEXT system.Pediatric Radiology 03/2007; 37(2):123-32; quiz 249-50. · 1.67 Impact Factor
Top co-authors
Top Journals
Institutions
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2012
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International Breast Cancer Study Group
Bern, BE, Switzerland -
Radboud Universiteit Nijmegen
- Medical Centre
Nijmegen, Provincie Gelderland, Netherlands
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2010
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Universiteit van Amsterdam
Amsterdam, North Holland, Netherlands
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2007
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Great Ormond Street Hospital NHS
- Department of Radiology
London, ENG, United Kingdom
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