Fournier-Charriere E.,Unite douleur et soins palliatifs de ladulte et de lenfant |
Marec-Berard P.,Institute dhemato oncologie pediatrique |
Schmitt C.,Unite douleur |
Delmon P.,Consultation douleur pediatrique |
And 2 more authors.
Archives de Pediatrie
Neuropathic pain exists in children and its incidence is often underestimated due to the lack of knowledge on the existence and the diagnosis of this pain. Although the semiological characteristics can be compared to those of the adult (allodynia, hypoesthesia, burning and stabbing sensations), their etiology often differs, and pain treatments are more limited because of a lack of pharmacological data and the absence of clinical studies. Therapeutic management is sometimes insufficient and requires better knowledge of this entity. Based on the June 2009 recommendations of the French Agency for Food and Drug Safety (Afssaps) (drug therapy in acute and chronic pain in children), this article presents a review of the data available in the literature on the subject, taking into account expert opinion and proposing clinical recommendations of good practice for the recognition and the treatment of neuropathic pain in children. © 2011. Source
Smets A.M.J.B.,University of Amsterdam |
Tinteren H.V.,Netherlands Cancer Institute |
Bergeron C.,Institute dhemato oncologie pediatrique |
Camargo B.D.,Instituto Nacional Of Cancer |
And 2 more authors.
European Journal of Cancer
Background: The SIOP 2001 nephroblastoma study hypothesised that patients with 'CT-only' pulmonary nodules would have the same outcome as patients with localised disease of same stage and histology. Patients: Unilateral Wilms' tumour (WT) patients, who had chest CT scans at diagnosis showing any sized pulmonary nodules undetected on chest X-ray, between November 2001 and November 2009, were selected from the SIOP 2001 database. Results: Among 2532 WT patients, 103 unilateral nephroblastoma patients with CT-only lung lesions were found. Thirty-seven patients received preoperative treatment according to the localised-disease protocol, and 66 according to the metastatic-disease protocol. The 3-year event-free survival (EFS) was 70% (95% CI: 55-89%) and 77% (95% CI: 66-89%), respectively. Corresponding 3-year overall survival (OS) was 89% (95% CI: 77-100%) and 85% (95% CI: 75-96%), respectively (p-value not significant). EFS and OS of all 2071 patients with true localised disease were 87% (95% CI: 86-89%) and 96% (95% CI: 94-97%), respectively. Patients with metastatic disease (n = 358) had 3-year EFS and OS estimates of 68% (95% CI: 63-74%) and 77% (95% CI: 72-82%), respectively. Conclusions: EFS and OS of patients with CT-only lung lesions were inferior to that of true localised-disease patients and superior to that of patients with metastatic disease. However, no significant difference was found in EFS and OS between CT-only patients treated for localised or metastatic disease. The clinician's preference to treat patients with CT-only pulmonary nodules as metastatic disease is not evidence-based. Chest CT at diagnosis does not improve outcome but presents paediatric oncologists with a difficult dilemma. © 2011 Elsevier Ltd. All rights reserved. Source
Wilde J.C.H.,The Surgical Center |
Aronson D.C.,The Surgical Center |
Sznajder B.,Netherlands Cancer Institute |
Van Tinteren H.,Netherlands Cancer Institute |
And 14 more authors.
Pediatric Blood and Cancer
Background: Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors. Aim: Inventory of the current SIOP NSS-experience. Procedures: 2,800 patients with a unilateral, localized or metastatic and an unequivocal surgical technique recorded were included. All had neo-adjuvant chemotherapy and delayed surgery. In 91 (3%) NSS was performed and in 2709 TN. Data was retrieved from the SIOP WT 2001 database. Results: NSS group contained 65% stage I tumours and the TN group 48%. Tumor volume (at diagnosis and surgery) was significantly smaller in the NSS group. Within stage III, after NSS, 7/12 (58%) had positive margins (M+), 5 with tumor negative lymph nodes (LN-). After TN, 355/712 (55%) had M+, 182 were LN- Treatment of M+ in the NSS group resulted in two conversions to TN (one combined with radiotherapy), three patients had radiotherapy only and in two patients local therapy, if given, was not recorded. After NSS, four recurrences occurred. For localized disease the 5-year overall (OS) and event free survival (EFS) in NSS group was 100 and 94.8 (95% CI:89.9-99.9), respectively, while OS and EFS in the TN group were 94.4 (95% CI: 93.2-95.5, log-rank test P=0.06) and 86.5 (95% CI:85.0-88.1, log-rank test P=0.06), respectively. Conclusions: NSS was only performed in 3% of patients with uWT. Despite excellent survival with few relapses, the gain of nephrons needs to be weighed against the risk to induce stage III with intensified therapy. © 2014 Wiley Periodicals, Inc. Source
Mestrallet G.,University Claude Bernard Lyon 1 |
Bertholet-Thomas A.,University Claude Bernard Lyon 1 |
Ranchin B.,University Claude Bernard Lyon 1 |
Bouvier R.,Groupement Hospitalier Est |
And 2 more authors.
FS is an inherited disease characterized by male pseudohermaphroditism and glomerular involvement leading to end-stage renal disease during adolescence or early adulthood (J Pediatr 1964:64:740). The FS phenotype in 46,XY patients consists of female external genitalia, gonadal dysgenesis, high risk of gonadoblastoma, and development of renal failure in the second decade of life. FS is caused by heterozygous mutation in intron 9 of the WT1 leading to a change in splicing that results in loss of three amino acids (+KTS isoform), thus disrupting the normal ratio of the +KTS/-KTS isoforms that is critical for proper gonadal and renal development (Nat Genet 1997:17:467; Hum Mol Genet 1998:7:709). We report on a patient followed for FS revealed by acute peritoneal syndrome because of ovarian dysgerminoma. Therapeutic options had led to an unusual course with recurrent neoplastic disease after renal transplantation. © 2010 John Wiley & Sons A/S. Source
Zacharoulis S.,Royal Marsden Hospital |
Ashley S.,Biostatistics |
Moreno L.,Royal Marsden Hospital |
Gentet J.-C.,Hopital de la Timone |
And 2 more authors.
Child's Nervous System
Introduction More than a third of children with ependymoma experience relapse, and despite multimodal treatment, less than 25% of them can then achieve long-term survival. Prognostic factors for patients who relapse have not been extensively analyzed. Patients and methods We retrospectively analyzed 82 patients from four pediatric oncology European institutions in order to identify prognostic factors and influence of treatment modalities in relapsed ependymoma. Results First relapse occurred after a median of 19 months (1 month-16 years). Five-year progression-free survival and overall survival of the cohort were 17% and 27.6%, respectively. Survival was statistically significantly higher for patients achieving gross total resection. No survival benefit was seen for children receiving chemotherapy whereas patients who were amenable to some form of reirradiation had a better outcome. Objective responses were found in more than 25% of patients receiving oral etoposide, temozolomide, or vincristine/etoposide/cyclophosphamide regimens. Multivariate analysis confirmed that patients with mixed relapses, no surgery at relapse, and receiving chemotherapy did worse (hazard ratio=3.6, 3.3, and 1.7, respectively, all p<0.05). Discussion Relapsed ependymoma carries a very poor prognosis with an indolent chronic course, leading to death in approximately 90% of the patients. Complete surgical resection whenever possible should be encouraged. Radiation therapy of the relapsed lesions can provide some minor benefit whereas chemotherapy despite the occasional responses provides no benefit in the final outcome which is dismal. Efforts have to be orchestrated internationally to enroll these patients on clinical trials using biology-based therapies. © Springer-Verlag 2009. Source