Safety and feasability of ureteroscopy for pediatric stone, in children under 5 Years (SFUPA 5): A French multicentric study.

INTRODUCTION: Ureteroscopy (URS) can be proposed as first-line therapy for the management of pelvic stones from 10 to 20 mm and for lower ureteric stones in children. However, little is known about the success and the morbidity of URS in young children. Ureteroscopic treatment may present matters in young children because of the small size of the pediatric kidney and the small size of the collecting system. OBJECTIVE: To assess safety and efficacy of URS for the treatment of urinary stones in children aged of 5 years or less. STUDY DESIGN: After the institutional ethical board approval was obtained, we conducted a retrospective, analytic, multicentric study that included all URS performed between January 2016 and April 2022 in children aged of 5 years or less. In this non-comparative case series, anonymized pooled data were collected from 7 tertiary care centers of pediatric patients. Endpoints were the one-session SFR at 3 months and per and postoperatives complications. Descriptive statistics were applied to describe the cohort. RESULTS: Eighty-three patients were included. For them, 96 procedures were performed at the median age of 3.5 years (IQR: 0.8-5) and median weight of 14 Kg (6.3-23). Median stone size was 13 mm (4-45). There were 65 (67 %) renal stones treated with flexible URS, most of which were in the renal pelvis (30 %) and in the lower calix (33 %). A ureteral access sheath was used in 91 % procedures. Preoperative ureteral stent was placed in 52 (54 %) of patients. None of patients had ureteral dilatation. The single-session SFR was 67.4 % (56.3 and 89.2 % for flexible URS and semi-rigid URS respectively) and children require 1.4 procedures to achieve complete stone clearance. The overall complication rate was 18.7 %, most of them were minor (Clavien I-II). Intraoperative perirenal extravasation (Clavien IIIb) due to forniceal rupture was documented in 6.2 % of cases, related to an increased intrapelvic pressure (IPP) performed in a closed pelvicalyceal system. DISCUSSION: Pediatric urologists should be aware of forniceal rupture based on the presence of extravasation of contrast during endourological procedures especially when they have difficulties to reach lower caliceal stone in small patient. CONCLUSION: URS in patients aged of 5 years or less, is a complex minimally invasive procedure with reasonable efficacy and low morbidity. Intrarenal stones treated by RIRS in young children carries the risk of additional procedures to complete stone clearance.

Circumcision and Risk of Febrile Urinary Tract Infection in Boys with Posterior Urethral Valves: Result of the CIRCUP Randomized Trial.

BACKGROUND: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this. OBJECTIVE: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs. DESIGN, SETTING, AND PARTICIPANTS: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision. INTERVENTION: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method. RESULTS AND LIMITATIONS: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age. CONCLUSIONS: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs. PATIENT SUMMARY: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs.

Management of ureteropelvic junction obstruction in children-a roundtable discussion.

The investigation, management and follow-up of paediatric ureteropelvic junction obstruction is not standardized. The Young Pediatric Urology Committee of the European Society of Pediatric Urology interviewed five experts in the field on various aspects of management and compared this with published literature.

[Appendicovesicostomy (Mitrofanoff procedure) in children: Long-term follow-up and specific complications]. / Dérivations urinaires continentes trans-conduit selon Mitrofanoff chez l'enfant : suivi à long terme et complications spécifiques.

OBJECTIVE: In children, intermittent catheterization by appendicovesicostomy according to Mitrofanoff is an interesting alternative to the urethral approach. Objective of the study was to evaluate the rate of appendicovesicostomy's specific complications. METHOD: From 1997 to 2017, data on children treated and followed for an appendicovesicostomy in an academic institution were collected retrospectively. Rates of surgical complications specifically encountered on appendicovesicostomy, time of onset, frequency, and necessity of surgical reinterventions have been reported. RESULTS: Thirty-four patients were operated on and followed for a median of 6.2 years [0.3-24]. Fifty percent had a complication, occurring after a median of 8 months [2-90], and 38% required at least one surgical revision. If complication occurred, adjustment of medical treatment and intermittent catheterization was effective in 12% of patients, endoscopic or over-fascial surgery was necessary in 17% of cases, and under-fascial revision in 21% of cases. Median time to complication was 4 months [1-90] after creation or revision of appendicovesicostomy. Thirty-height percent of difficult channel catheterization were reported, of which 46% were over or under-fascial stenosis. Inaugural urinary incontinence was 18%, and only 9% if using the appendix. At the end, 97% of appendicovesicostomy were continent. CONCLUSIONS: Appendicovesicostomy is a high risk of complications and postoperative revisions surgery, in order to have a functional continent channel. LEVEL OF EVIDENCE: 4.

One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results.

BACKGROUND: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. OBJECTIVE: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. DESIGN, SETTING, AND PARTICIPANTS: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]. INTERVENTION: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. OUTCOME MEASUREMENTS: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. RESULTS AND LIMITATIONS: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up. COMPLICATIONS: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. CONCLUSION: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy.

Neuroblastoma chemotherapy can be augmented by immunotargeting O-acetyl-GD2 tumor-associated ganglioside.

Despite recent advances in high-risk neuroblastoma therapy, the prognosis for patients remains poor. In addition, many patients suffer from complications related to available therapies that are highly detrimental to their quality of life. New treatment modalities are, thus, urgently needed to further improve the efficacy and reduce the toxicity of existing therapies. Since antibodies specific for O-acetyl GD2 ganglioside display pro-apoptotic activity against neuroblastoma cells, we hypothesized that combination of immunotherapy could enhance tumor efficacy of neuroblastoma chemotherapy. We demonstrate here that combination of anti-O-acetyl GD2 monoclonal antibody 8B6 with topotecan synergistically inhibited neuroblastoma cell proliferation, as shown by the combination index values. Mechanistically, we evidence that mAb 8B6 induced plasma cell membrane lesions, consistent with oncosis. Neuroblastoma tumour cells treated with mAb 8B6 indeed showed an increased uptake of topotecan by the tumor cells and a more profound tumor cell death evidenced by increased caspase-3 activation. We also found that the combination with topotecan plus monoclonal antibody 8B6 showed a more potent anti-tumor efficacy in vivo than either agent alone. Importantly, we used low-doses of topotecan with no noticeable side effect. Our data suggest that chemo-immunotherapy combinations may improve the clinical efficacy and safety profile of current chemotherapeutic modalities of neuroblastoma.

The radical soft-tissue mobilization (Kelly repair) for bladder exstrophy.

UNLABELLED: The radical soft-tissue mobilization (RSTM) technique has been proposed as an alternative for staged reconstruction of bladder exstrophy. In this video, we demonstrate the successive steps of this technique. METHODS: A newborn male with classic bladder exstrophy underwent bladder closure without pelvic osteotomies at birth; he then underwent RSTM at 1 year of age. TECHNIQUE: Mobilization: The RSTM involved simultaneous intra-pelvic and perineal dissection, aiming at full mobilization of potential urethral sphincter striated structures, levatores ani muscles, pudendal pedicles and corpora cavernosa from their attachment on the pubic bones. From the perineal approach, a periosteum incision was performed on the antero-medial aspect of the pubic bones, and the periosteum was peeled from the ischio-pubic ramus, lifting the corpora from the bone and providing control on the pudendal pedicles. The attachments of all striated muscular structures on the lower aspects of the posterior corpora (transverse perinei, bulbo-spongiosus, ischio-cavernosus muscles) were released to allow adequate re-approximation around the reconstructed urethra. Reconstruction: After ureteric reimplantation, a funnel-shaped cervicoplasty was performed and continued by urethroplasty over an 8-Fr catheter. The muscular structures mobilized from the corpora and the bones were wrapped around the neo-urethra, which was transposed ventrally in the scrotal position, creating a temporary hypospadiac urethrostomy. After abdominal wall closure, the corpora were then de-rotated, and secured to the neo-symphisis with non-absorbable sutures, and the penile skin shaft was reconstructed. RESULTS: At the 12-month follow-up, the boy was asymptomatic, still in nappies, with non-dilated upper-tracts on renal ultrasound, and dryness intervals of 90 min. CONCLUSION: Radical soft-tissue mobilization allows a tension-free bladder-neck reconstruction, and may favour development of physiological continence mechanisms independent of the inevitable pubic symphisis re-enlargement. Creation of temporary hypospadias is most often needed to maximize penile length. This technically demanding procedure requires thorough knowledge of detailed anatomy of the bladder exstrophy pelvis, as demonstrated in this video.

Long-term results with the laparoscopic transposition of renal lower pole crossing vessels.

BACKGROUND: For the treatment of ureterovascular pelviureteric junction obstruction (PUJO), transposition of lower pole crossing vessels (LPCV) has been described as an alternative to dismembered pyeloplasty. PURPOSE: To report on the long-term follow-up of children after laparoscopic transposition of LPCV. METHODS: A retrospective analysis of 70 children consecutively treated by laparoscopic transposition of LPCV. Candidate patients were selected on the basis of clinical history, renal ultrasound (US), and pre-operative mercaptoacetyltriglycine (MAG-3) scan. Selection criteria included: presence of LPCV with SFU Grade 1-2 hydronephrosis, impaired drainage on MAG-3 and intraoperative normal pelviureteric junction (PUJ) and ureter peristalsis. Thinned parenchyma, impaired renal function, or history of prenatal hydronephrosis were not considered as exclusion criteria. Children were clinically followed up with US and MAG-3 scan. Success was defined by symptom resolution with improvement in hydronephrosis. RESULTS: Seventy children, aged 8.3 years (range 2.75-16.0), were selected. Procedures were performed through transperitoneal laparoscopy (n = 42) or were robotic-assisted (n = 28). Operative time was 120 min and length of hospital stay was 2 days. The outcome was successful in 67/70 patients (96%), with a median follow-up of 52 months (range 13-114). There were three failures in children who eventually underwent dismembered pyeloplasty for a symptomatic, undiagnosed, intrinsic PUJ obstruction. Two of them had been postnatally followed for a resolving prenatally diagnosed hydronephrosis. Three children became free of symptoms, had improved hydronephrosis, but still showed impaired drainage on MAG-3 and are being closely followed up. DISCUSSION: Although this procedure proves to have long-term efficiency in selected indications, the main challenge is to intraoperatively ascertain the absence of associated intrinsic stenosis. Objective criteria remain difficult to establish, but intraoperative findings, including dependent, funnel-shaped, normal-looking PUJ with decreasing hydronephrosis after pelvis and LPCV mobilisation, and efficient peristalsis across the PUJ under intraoperative diuretic test, represent a low likelihood of associated intrinsic stenosis. Ipsilateral impaired renal function doesn't seem to be associated with an adverse outcome. In contrast, a prenatal history of mild or self-resolving hydronephrosis in a patient later presenting with intermittent dilatation, raises the suspicion of associated intrinsic PUJ obstruction, as it is associated with a higher risk of failure. CONCLUSION: With a long postoperative follow-up, the robotic-assisted or laparoscopic vascular hitch procedure has been successful in treating a selected group of children with obstructive LPCV, and represents a safe and reliable alternative to standard dismembered pyeloplasty in the absence of intrinsic PUJO suspected on prenatal US.

Characteristics of endobronchial primitive tumors in children.

Primary endobronchial tumors are rare in children and they include a broad spectrum of lesions. The aim of this study was to determine the characteristic features, treatments and outcomes of these tumors. We report a retrospective analysis of all patients treated for endobronchial tumor in nine French hospitals between 1990 and 2010 and a comparison of the results with those reported in the medical literature. Twelve tumors were reported: five low grade muco epidermoid carcinomas, two inflammatory myofibroblastic tumors, two hemangiomas, one anaplastic large cell lymphoma, one carcinoid tumor, and one juvenile xanthogranuloma. The mean age of the patients was 7.5 ± 3.5 years. The most common sign revealing the disease was persistent atelectasis or recurrent pneumonia (eight cases). The other revealing signs were a persistent bronchospasm (three cases) and hemoptysis (one case). The clinical presentation, biology, serum tumor markers, and chest X-ray abnormalities were not specific to a particular histological diagnosis. Chest CT scan revealed the presence of an endobronchial tumor in 11 cases. Nine tumors could be diagnosed from a biopsy obtained by video endoscopy. Complete surgical resection was performed in seven patients. Bronchoscopic removal was performed in five cases and was successful in three. There were no deaths. Endobronchial tumors are rare in childhood and their histology is diverse. Chest CT scan and per-endoscopic endobronchial biopsies are required for diagnosis, when possible. Surgical or endoscopic treatment should be discussed by a multidisciplinary team. Despite the multiple etiologies, the prognosis of these tumors is good if diagnosis is early and if resection is complete. Long-term recurrences have been described, so long-term follow-up of these children is recommended.

["Traffic lights" in pediatrics: what do residents beginning their family residency know about them?]. / Les « feux tricolores ¼ en pédiatrie : état des lieux des connaissances en début de 3e cycle de médecine générale.

OBJECTIVES: Pediatric practice is a difficult task requiring physicians to discriminate potentially serious situations among a variety of benign situations. The goal of this study was to assess the knowledge of students beginning their residency in family medicine on these situations. METHODS: One hundred and three students were evaluated on pediatric "traffic lights" using 103 true/false questions. Pediatric and family medicine teachers of the Nantes University Hospital defined "traffic lights" as the fundamentals of pediatrics, misleading situations, and diagnosis and treatment that should not to be missed. Emergency levels were defined by colors, with "red light" corresponding to life-threatening emergencies. RESULTS: Thirty-six percent of the questions (n=103) had a correct response rate below 75%. Thirty-two percent of the questions on emergency situations ("red lights") (n=37) had a response rate below 75%. Fifteen percent of the questions (n=103) had a correct response rate below 50%, half of which were "red light" (e.g., on meningitis, diabetic acidocytosis, or shock). Questions concerning infants (n=24) had significantly fewer correct answers (correct response rate below 50%: 29% versus 10%; P=0.047). All the students answered seven questions correctly. The students' mean score (percentage of good answers) was 76% (±6%). No student had 100% or less than 50% good answers. CONCLUSIONS: Some life-threatening situations or situations concerning infants had not been mastered by most of the students. It is therefore essential to optimize the teaching of pediatrics during the second cycle of medical studies.

[Neonatal surgical antibioprophylaxis: which target? which propositions?]. / Antibioprophylaxie en chirurgie néonatale : quelle est la cible ? quelles propositions ?

Neonatal surgical antibioprophylaxis must take into account the specificities of this life period, as immunological immaturity, microbiotal implantation and antibiotic treatment pharmacokinetics properties. Very few data are available, but it seems that coagulase negative Staphylococcus (CNS) is the most reported bacteria, in intestinal tract at the time of surgery as well as in surgical site infections. Usual vertical transmission bacteria as S. agalactiae or E. coli K1 are rarely documented. The antibioprophylaxis treatment must consider the age and the environmental context of the newborn; first or second generation cephalosporins sometimes in association with vancomycin seem appropriate in this context. It is now urgent to conduct clinical trials in order to validate theses propositions.

[Comparison of two protocols of febrile urinary tract infection management in children]. / Comparaison de deux protocoles de prise en charge des infections urinaires fébriles de l'enfant.

PURPOSE: To compare two first febrile urinary tract infection (UTI) management protocols with regards to the diagnosis of high-grade vesicoureteral reflux (VUR) and cost. METHODS: This study compared two cohorts of children under 16 years of age, admitted for a first episode of febrile UTI. The first group (in 2005) was managed according to previous recommendations (IV treatment and cystography performed for all children under 3 years of age). The second group (in 2006) was managed according to age and procalcitonin level. High-grade VUR frequency, UTI recurrence, hospitalization rate, and cost were compared between the two cohorts. RESULTS: A total of 225 children were included in 2005 and 116 in 2006. High-grade VUR was found in 6.2 and 9.5% of the patients in 2005 and 2006, respectively (P=0.274). There was no statistically significant difference in the UTI recurrence rate between the two cohorts (5.3% in 2005 and 8.6% in 2006; P=0.237). The mean cost of an episode of febrile UTI was not significantly different in 2005 and 2006 (€2235 in 2005, €2256 in 2006; P=0.902), but was lower for children older than 6 months in 2006 (€1292 versus €1882 in 2005; P=0.0042). CONCLUSION: Our management protocol for a first febrile UTI episode in children based on procalcitonin levels seems to be suitable for the diagnosis of high-grade VUR. The hospitalization rate and the mean cost of management for children older than 6 months of age was significantly reduced in 2006. The management guidelines for a first occurrence of febrile UTI in children should be reconsidered.

[Morbidity of foreskin reconstruction in distal hypospadias repair surgery]. / La reconstruction du prépuce dans la chirurgie d'hypospade distal : quelle morbidité ?

UNLABELLED: Hypospadias is almost constantly associated with an abnormality of the prepuce, resulting in incomplete prepuce. In the context of distal hypospadias repair surgery, prepuce reconstruction is an alternative to circumcision. The authors report their experience of this operation and analyze its specific morbidity. PATIENTS AND METHODS: Single-center retrospective study of 316 prepuce reconstructions performed in the context of distal hypospadias surgery between 1996 and 2004. The median age at the time of surgery was 12.1 months. The prepuce reconstruction technique was based on the principles of Righini preputioplasty. Urethroplasty was performed according to the tubularized urethral plate (Duplay-Snodgrass) technique in the majority of children (204/316, 65%). No urethral catheter was left in place in 293 children (93%). The operation was performed as an outpatient procedure in 245 cases (78%). RESULTS: Partial or complete disunion of the reconstructed prepuce was observed in 18 children (6%), mainly early in the authors' experience, and nine of these 18 cases occurred in a more general context of failure of hypospadias repair surgery (urethrocutaneous fistula). Secondary phimosis was observed in 40 cases, 12 months after the operation. Topical corticosteroids (betamethasone 1.0% cream) allowed normal foreskin retraction in 26 out of 30 cases (85%). CONCLUSIONS: Prepuce reconstruction performed in the context of distal hypospadias repair surgery is responsible for a low rate of specific morbidity. If the objective of distal hypospadias surgery is to restore a penis with an appearance as normal as possible, prepuce reconstruction should constitute a key element of the final result.

Antibiotic prophylaxis for the prevention of recurrent urinary tract infection in children with low grade vesicoureteral reflux: results from a prospective randomized study.

PURPOSE: Antibiotic prophylaxis is given to children at risk for urinary tract infection. However, evidence concerning its effectiveness in grade I to III vesicoureteral reflux is lacking. The objective of this study was to determine whether antibiotic prophylaxis reduces the incidence of urinary tract infection in young children with low grade vesicoureteral reflux. MATERIALS AND METHODS: Children 1 month to 3 years old with grade I to III vesicoureteral reflux were assigned randomly to receive daily cotrimoxazole or no treatment, and followed for 18 months. A urinary tract infection constituted an exit criterion. Infection-free survival rates were calculated using the Kaplan-Meier method and compared using the log rank test. RESULTS: A total of 225 children were enrolled in the study. Distribution of gender, age at inclusion and reflux grade were similar between the 2 groups. There was no significant difference in the occurrence of urinary tract infection between the 2 groups (17% vs 26%, p = 0.2). However, a significant association was found between treatment and patient gender (p = 0.017). Prophylaxis significantly reduced urinary tract infection in boys (p = 0.013), most notably in boys with grade III vesicoureteral reflux (p = 0.042). CONCLUSIONS: These data suggest that antibiotic prophylaxis does not reduce the overall incidence of urinary tract infection in children with low grade vesicoureteral reflux. However, such a strategy may prevent further urinary tract infection in boys with grade III reflux.

Long-term outcome of laparoscopic Nissen-Rossetti fundoplication for neurologically impaired and normal children.

BACKGROUND: This study aimed to assess the long-term effects of laparoscopic Nissen-Rossetti fundoplication (LNF) on clinical and pH evaluations of children with gastroesophageal reflux disease (GERD) according to neurologic status. METHODS: The study examined 127 children (73 neurologically impaired and 54 neurologically normal with primary GERD) who consecutively underwent LNF from 1992 to 2003. The follow-up protocol included evaluations at 3, 15, and more than 36 months (long-term evaluation) postoperatively, which consisted of physical examination and 24-h pH monitoring. Recurrences were defined as abnormal pH-metry exhibited by symptomatic children. RESULTS: The long-term follow-up period averaged 5.5 years. Of the 73 neurologically impaired children, 9 (12%) had GERD recurrences, which occurred during the evaluation period and required redo surgery in four cases, including two Bianchi procedures. In the neurologically normal group, one recurrence (2%) occurred 3 months after surgery. The long-term complications in this group included dyspepsia (n = 3), occasional dysphagia (n = 2), gas bloat syndrome (n = 1), and alimentary disorders (n = 2). CONCLUSIONS: For children with primary GERD, LNF is a long-term efficient procedure. For the neurologically impaired children, the results were good, with more than 85% of the children symptom free after 5 years, although repeated evaluations are required to diagnose late recurrences related to evolving dysmotility disorders.

[Pleuropulmonary blastoma: a case of early neonatal diagnosis through antenatal scan screening]. / Pleuropneumoblastome: un cas de découverte néonatale précoce grâce au dépistage échographique prénatal.

We report a case of pleuropulmonary blastoma (PPB) prenatally diagnosed at 32 weeks of gestation. The suspected diagnoses was a congenital cystic adenomatoid malformation of the lung (CCAM). The excision of the malformation was performed through a thoracotomy at 4 months of age. On pathological examination, the presence of focal stromal hypercellularity (cambium layer) in the cyst wall was consistent with both the diagnosis of type IV CCAM or grade I PPB. The presence of nodules of immature cartilage and the positivity of particular immunohistochemical staining (indicate which staining came positive) confirmed the diagnosis of grade I PPB. Pleuropulmonary blastoma is a rare malignant tumour in children, sometimes difficult to differentiate from the type IV CCAM as they share similar circumstances of diagnosis, radiological and macroscopic features. The pathological examination does not always allow to differentiate them clearly, implying the necessity of an extended medical supervision for the children carrying those border lesions. Because of the risks of malignancy and of the risks of septic complications, surgical resection during the first year of life is often advocated. Therefore, prenatal detection is essential to allow adequate early management and complete resection of these lesions.

[Understanding nephroblastoma. Information dedicated to parents and relatives]. / Comprendre le néphroblastome. Information à l'usage des parents et des proches d'un enfant malade.

In response to the evolution of the information-seeking behaviour of patients and concerns from health professionals regarding cancer patient information, the French National Federation of Comprehensive Cancer Centres (FNCLCC) introduced, in 1998, an information and education program dedicated to patients and relatives, the SOR SAVOIR PATIENT program. The methodology of this program adheres to established quality criteria regarding the elaboration of patient information. Cancer patient information developed in this program is based on clinical practice guidelines produced by the FNCLCC and the twenty French regional cancer centres,the National League against Cancer, The National Cancer Institute, the French Hospital Federation, the National Oncology Federation of Regional and University Hospitals, the French Oncology Federation of General Hospitals, many learned societies, as well as an active participation of patients, former patients and caregivers. The handbook SOR SAVOIR PATIENT Understanding nephroblastoma is an adapted version of various scientific publications and international clinical practice guidelines, validated by oncology experts and by the Nephroblastoma Committee of the French Society against Cancers and Leukaemias in children and adolescents (SFCE). It was elaborated with the active participation of parents and other family members. It is meant to provide a basis for the explanation of the disease, to help parents asking questions, and to facilitate discussions with the healthcare team. It is available from the FNCLCC (101, rue de Tolbiac, 75013 PARIS, Tel. (0033)1 76 64 78 00, www.fnclcc.fr). This document was validated at the end of 2005 and published in May 2006. SOR SAVOIR PATIENT guides are systematically updated when new research becomes available. Information leaflets, extracted from the handbook SOR SAVOIR PATIENT Understanding nephroblastoma and published in this edition of the Cancer et Radiothérapie, describe the physiopathology of nephroblastoma, as well as treatments and follow-up. The guide allows parents and relatives to better understand the disease and the treatments proposed. It also offers health professionals a synthetic evidence-based patient information source which facilitates discussions with the patient.