Background. High-risk medulloblastoma is defined by the presence of metastatic disease and/or incomplete resection and/or unfavorable histopathology and/or tumors with MYC amplification. We aimed to assess the 3-year progression-free survival (PFS) and define the molecular characteristics associated with PFS in patients aged 5-19 years with newly diagnosed high-risk medulloblastoma treated according to the phase II trial PNET HR+5. Methods. All children received postoperative induction chemotherapy (etoposide and carboplatin), followed by 2 high-dose thiotepa courses (600 mg/m(2)) with hematological stem cell support. At the latest 45 days after the last stem cell rescue, patients received risk-adapted craniospinal radiation therapy. Maintenance treatment with temozolomide was planned to start between 1-3 months after the end of radiotherapy. The primary endpoint was PFS. Outcome and safety analyses were per protocol (all patients who received at least one dose of induction chemotherapy). Results. Fifty-one patients (median age, 8 y; range, 5-19) were enrolled. The median follow-up was 7.1 years (range: 3.4-9.0). The 3 and 5-year PFS with their 95% confidence intervals (95% CI) were 78% (65-88) and 76% (63-86), and the 3 and 5-year OS were 84% (72-92) and 76% (63-86), respectively. Medulloblastoma subtype was a statistically significant prognostic factor (P-value = 0.039) with large-cell/anaplastic being of worse prognosis, as well as a molecular subgroup (P-value = 0.012) with sonic hedgehog (SHH) and group 3 being of worse prognosis than wingless (WNT) and group 4. Therapy was well tolerated. Conclusions. This treatment based on high-dose chemotherapy and conventional radiotherapy resulted in a high survival rate in children with newly diagnosed high-risk medulloblastoma.

Abstract PURPOSE To assess the 3-year EFS rate of children younger than 5 years of age with high-risk medulloblastoma (MB) treated according to the prospective multicenter trial HR MB-5. PATIENTS AND METHODS After surgery, all children received 2 cycles of Etoposide- Carboplatine. If partial (PR) or complete response (CR) was achieved after induction chemotherapy, children received 2 courses of thiotepa (600mg/m²) with stem cell rescue. For patients in CR after high-dose chemotherapy, they received one course of Cyclophosphamide – Busilvex with stem cell rescue (Phase I part). The others patients (not in PR after induction or in CR after thiotepa) were treated with 2 cycles of Temozolomide-Irinotecan followed by age-adapted craniospinal irradiation and maintenance treatment. RESULTS 28 children (2 to 4 years; median: 3.0 years) were enrolled. Group 3 MB were most common (57%). The response rate to Etoposide-Carboplatine was 60.7%. Among 20 patients treated with Thiotepa, 13 children were in CR and received Cyclophosphamide – Busilvex without radiotherapy. Out of them, 9 patients (45%) are alive in CR without craniospinal irradiation (median follow-up 5 years). Among 15 patients treated with radiotherapy, 8 patients are alive (median follow-up 3.8 years). The study was prematurely stopped for an excess of events. The median follow-up was 4 years (range 1.5 - 6.1). The 3-year EFS and OS were 42.3% [25.9 - 60.6] and 71.3% [52.7 - 84.7], respectively. CONCLUSIONS This risk-adapted strategy did not improve EFS in young children with high-risk MB. However, the study shows that good responders to chemotherapy can be cured without recourse to irradiation.

BACKGROUND High-risk medulloblastoma are defined by the presence of metastatic disease and/or incomplete resection and/or unfavorable histopathology and/or tumors with MYC amplification. We aimed to assess the 3-year progression-free survival (PFS) and define the molecular characteristics associated with PFS in patients aged 5 to 19 years with newly diagnosed high-risk medulloblastoma treated according to the phase 2 trial PNET HR+5. METHODS All children received postoperative induction chemotherapy (etoposide and carboplatin), followed by 2 high-dose thiotepa courses (600 mg/m 2) with hematological stem cell support. At the latest 45 days after the last stem cell rescue, patients received risk-adapted craniospinal radiation therapy. Maintenance treatment with temozolomide was planned to start between 1-3 months after the end of radiotherapy. The primary endpoint was PFS. Outcome and safety analyses were per protocol (all patients who received at least one dose of induction chemotherapy). RESULTS Fifty-one patients (median age, 8 years; range, 5-19) were enrolled. The median follow-up was 7.1 years (range: 3.4-9.0). The 3 and 5-year PFS with their 95% confidence intervals (95%CI) were 78% (65-88) and 76% (63-86), and the 3 and 5-year OS were 84% (72-92) and 76% (63-86), respectively. Medulloblastoma subtype was a statistically significant prognostic factor (p-value=0.039) with large-cell/anaplastic being of worse prognosis, as well as molecular subgroup (p-value=0.012) with SHH and group 3 being of worse prognosis than WNT and group 4. Therapy was well tolerated. CONCLUSIONS This treatment based on high-dose chemotherapy and conventional radiotherapy resulted in a high survival rate in children with newly diagnosed high-risk medulloblastoma.

Purpose: To identify the incidence of patients with perihippocampal metastases to assess the risk of brain relapse when sparing the hippocampal area. Medulloblastoma (MB) represents 20% of pediatric brain tumors. For high-risk MB patients, the 3- to 5-year event-free survival rate has recently improved from 50% to >76%. Many survivors, however, experience neurocognitive side effects. Several retrospective studies of patients receiving whole brain irradiation (WBI) have suggested a relationship between the radiation dose to the hippocampus and neurocognitive decline. The hippocampal avoidance-WBI (HA-WBI) approach could partially reduce neurocognitive impairment in children treated for high-risk MB. Methods and Materials: From 2008 to 2011, 51 patients with high-risk MB were treated according to the French trial primitive neuroectodermal tumor HR+5. Hippocampal contouring was manually generated on 3-dimensional magnetic resonance images according to the Radiation Therapy Oncology Group 0933 atlas. The distribution of metastases was assessed relative to the hippocampus: 0 to 5 mm for the first perihippocampal area and 5 to 15 mm for the rest of the perihippocampal area. Results: The median patient age was 8.79 years (33% female). After a follow-up of 2.4 years, 43 patients were alive; 28 had had brain metastasis at diagnosis and 2 at relapse, with 16% in the first perihippocampal area and 43% in the rest of the perihippocampal area. Of the 18 patients without brain metastases at diagnosis, including M1 patients, none developed secondary lesions within the first or the rest of the perihippocampal area, after receiving 36 Gy. No clinical or biological factor was significantly associated with the development of perihippocampal metastases. Conclusions: Our results suggest the HA-WBI strategy should be evaluated for the subgroup of high-risk MB patients without metastatic disease. (C) 2017 Elsevier Inc. All rights reserved.

La connaissance de l’anatomie et de la physiologie ovarienne est un prérequis essentiel pour comprendre la pathologie fonctionnelle de l’ovaire. Un dysfonctionnement d’un ou plusieurs follicules ou du stroma ovarien génère de multiples anomalies habituellement réversibles chez les femmes en période d’activité génitale. La pathologie folliculaire peut être mono folliculaire, générant kystes folliculaires simples ou hémorragiques. Elle est aussi fréquemment pluri folliculaire, à l’origine de dystrophies ovariennes macro polykystiques ou micro polykystiques, alors associées à des perturbations métaboliques ou endocriniennes. L’atteinte du stroma est plus rare, à l’origine d’hyperplasie stromale, d’œdème ovarien massif ou de fibromatose ovarienne d’aspect pseudo-tumoral. L’échographie endovaginale est l’examen de première intention pour l’exploration de l’ensemble de ces affections. Lorsqu’un doute persiste sur la nature fonctionnelle de ces diverses anomalies, l’IRM est un complément utile pour compléter leur caractérisation. La grossesse est responsable de diverses modifications fonctionnelles posant des problèmes diagnostiques différentiels avec des tumeurs organiques ne devant pas conduire à une chirurgie délétère. Le syndrome d’hyperstimulation est une forme iatrogène d’hyperreactio luteinalis lié à la prise en charge de l’infertilité par les inducteurs de l’ovulation.Knowledge of ovarian anatomy and physiology is an essential prerequisite to understand tumor-like lesions of the ovary. Dysfunction of the follicular or ovarian stroma generates multiple reversible abnormalities in women of childbearing age. Follicular disorders can be related to only one follicle, generating simple or hemorrhagic follicular cysts. Follicular disorders related to numerous ovarian follicles create polycystic ovarian diseases that are frequently associated with clinical and biological disturbances. Involvement of the ovarian stroma is less frequent and is responsible for stromal hyperplasia, massive ovarian edema or ovarian fibromatosis. Transvaginal ultrasound is the first-line examination for the evaluation of all these disorders. In the presence of sonographically indeterminate lesions, magnetic resonance imaging is useful to characterize these disorders. Pregnancy is associated with various modifications giving rise to tumor-like lesions that must not lead to inappropriate surgical management. The hyperstimulation syndrome which is related to induction of the ovulation during medical assisted procreation is similar to hyperreactio luteinalis.

Neurofibromatosis type 2 (NF2) is a heritable syndrome characterized by multifocal proliferation of neural crest-derived cells. It has long been regarded as an adolescent- and adult-onset disease. We report here on a case of a 6-year-old girl with infantile-onset clinical signs. The girl, who had a history of amblyopia and congenital retinal hamartoma, presented with rough dimness of visual acuity. Cerebral magnetic resonance imaging found a left voluminous fronto-temporal tumor including the chiasma and optical nerves. Vestibular and cervical nerve schwannomas were also found. She underwent a first neurosurgical partial excision and histopathology revealed meningioma. Postoperative radiotherapy was necessary due to a secondary increase of the tumor size. Subsequent molecular testing revealed a NF2 gene abnormality. NF2 can become evident in infancy but clinical early symptomatology is often different: ocular symptoms and neurological problems are common. There is no consensus on the treatment of tumors involving the central and peripheral nervous system, abstention being usual. In case of severe signs, surgery and radiotherapy can be proposed. The diagnosis of a hamartoma must lead to multidisciplinary follow-up.

Définir le retour veineux pulmonaire anormal (RVPA), en décrire les différentes formes anatomiques et connaître leurs corrélations clinico-radiologiques. Savoir identifier et faire une description précise d'un RVPA sur un angio-scanner. Illustration du cas d'un nourrisson présentant une hypoxie sévère d'étiologie rare : un RVPA total bloqué de forme supra-cardiaque droite. Analyse rétrospective d'angio-scanners et revue de la littérature illustrant les principales formes de RVPA et leurs présentations clinico-radiologiques. Le RVPA est une cardiopathie congénitale rare : connexion anormale du système veineux pulmonaire dans la circulation systémique. Les tableaux cliniques sont variés : des formes asymptomatiques de certains RVPA partiels à la décompensation hypoxique brutale d'un RVPA total bloqué nécessitant une chirurgie en urgence. L'angio-scanner est une technique d'imagerie fiable qui confirme le diagnostic de RVPA, parfois suspecté en échographie, en fait une description anatomique rigoureuse, parfois essentielle en préopératoire (trajet, position du canal collecteur, sténose, compression). L'angio-scanner permet un bilan exhaustif des RVPA. Dans les formes totales bloquées, il permet de guider le geste chirurgical à réaliser en urgence.

Intérêt de l’échographie dans le diagnostic et le suivi des infections cervicales profondes. Etude rétrospective de 37 enfants âgés de 1 à 12 ans (moyenne 4,2 ans), consultant en urgence pour un torticolis fébrile, parfois un refus du décubitus dorsal, une dysphagie ou une odynophagie. Tous les enfants ont bénéficié d’une échographie première, d’une imagerie en coupes, scanner (n=30) et/ou IRM (n=10) avec injection puis d’un suivi échographique. L’échographie a suspecté ou mis en évidence l’atteinte rétro ou latéro-pharyngée dans tous les cas. Le scanner ou l’IRM ont permis d’orienter la décision thérapeutique en : évaluant la topographie précise de la lésion, sa taille, différenciant un abcès rétro ou latéro-pharyngé (n=32) d’une cellulite rétro-pharyngée (n=10), recherchant d’éventuelles complications. La majorité des patients ont été traités médicalement, sans drainage, avec un suivi échographique (taille de la collection, complication vasculaire). L’évolution a toujours été favorable. outre sonintérêt dans le diagnostic des infections cervicales profondes, l’échographie réalisée par un opérateur expérimenté permet surtout un suivi fiable sous traitement.

10007 Background: To assess the 3-year progression-free survival (PFS) rate of patients with newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET) between 5-20 years treated according to the prospective multicenter trial PNET HR+5. Methods: Children received as postoperative induction chemotherapy two cycles of etoposide (500mg /m²) - carboplatine (800mg/m²), followed by two courses of thiotepa (600mg/m² per course) with autologous stem cell rescue. Risk-adapted conventional radiotherapy (RT) was delivered around day 45 after second transplantation. Craniospinal RT dose was 36 Gy for patients with metastatic disease or with unfavourable histology (anaplastic MB, large cell MB, MB with myc amplification) followed by a tumor bed boost of 18 Gy. Patients with localized sPNET received focal RT at the dose of 54 Gy. Maintenance treatment with 6 cycles of temozolomide was planned to start between 1-3 months after the end of RT. Results: From January 2009 to February 2012, 64 patients (MB=51; sPNET=13) between 5 and 19 years (median age, 9 years) were enrolled. Five patients didn’t received RT due to progressive disease. Maintenance treatment was administered in 44 patients. The median follow-up was 32 months (range, 16-54 months). The 3-year PFS and overall survival (OS) were 80% (95% CI: 68-88%) and 85% (95% CI: 74-92%), respectively. The 3-year PFS was 79% (95% CI: 65-88%) for children with MB and 85% (95% CI: 58-96%) for those with sPNET. No major unexpected toxicities and no treatment-related deaths were reported. Conclusions: This treatment based on high-dose chemotherapy and conventional RT resulted in a high overall survival rate in children and adolescent with newly diagnosed high-risk cerebral PNET. Clinical trial information: NCT00936156.

Sagittal and axial corrections of the three-dimensional deformity characteristic of scoliosis remain challenging.

Purpose Sagittal spine and pelvic alignment of adolescent idiopathic scoliosis (AIS) is poorly described in the literature. It generally reports the sagittal alignment with regard to the type of curve and never correlated to the thoracic kyphosis. The objective of this study is to investigate the relationship between thoracic kyphosis, lumbar lordosis and sagittal pelvic parameters in thoracic AIS. Methods Spinal and pelvic sagittal parameters were evaluated on lateral radiographs of 86 patients with thoracic AIS; patients were separated into hypokyphosis group ( n = 42) and normokyphosis group ( n = 44). Results were statistically analyzed. The lumbar lordosis was lower in the hypokyphosis group, due to the low proximal lordosis. The thoracic kyphosis was not correlated with any pelvic parameters but with the proximal lordosis. The pelvic incidence was correlated with sacral slope, pelvic tilt, lumbar lordosis and highly correlated with distal lumbar lordosis in the two groups. There was a significant linear regression between thoracic kyphosis and proximal lordosis and between pelvic incidence and distal lordosis. Conclusions We can consider that the proximal part of the lordosis depends on the thoracic kyphosis and the distal part depends on the pelvic incidence. The hypokyphosis in AIS is independent of the pelvic parameters and could be described as a structural parameter, characteristic of the scoliotic deformity.

BACKGROUND:Recurrent cholesteatoma after surgical excision occurs frequently in children. Until recently, a surgical second look was mandatory and considered as standard reference. MRI including a delayed T1 sequence after gadolinium injection and diffusion-weighted imaging (DWI) has proved its efficiency but has been evaluated mainly in adults.OBJECTIVE:Our purpose was to evaluate the accuracy of DWI to diagnose recurrence of cholesteatoma in children.MATERIALS AND METHODS:We evaluated prospectively with MRI 20 ears in 18 children who had had surgery for cholesteatoma. We compared DWI and delayed T1-weighted images following gadolinium administration with intraoperative or follow-up findings. We calculated the sensitivity and specificity of each sequence for the diagnosis of recurrent cholesteatoma.RESULTS:Sensitivity to diagnose recurrent cholesteatoma was 87% for both DWI and delayed post-gadolinium sequences, specificity was 71% and 83%, respectively. Adding both sequences, the sensitivity was 87%, the specificity 100%. There was one false negative probably due to small size recurrence.CONCLUSION:In our series, DWI was reliable to diagnose recurrent cholesteatoma in children and allows avoiding surgery when negative. However, because small recurrences less than 5 mm may be missed, follow-up must be prolonged (5 years).

During childhood, between neonatal period with its maternal hormonal impregnation and adult age with beginning of menstruation period, female genitalia modify morphologically and functionally. A precise knowledge of normal appearances and evolution is mandatory before studying various pathological aspects. Pathological aspects are not frequent and different from those encountered from adolescence. We will distinguish two periods: neonatal period and childhood. Nowadays, anomalies encountered during neonatal period are often diagnosed or suggested before birth with prenatal diagnosis (ovarian cyst, malformations or sexual differentiation anomalies). During childhood, gynecologic diseases are mainly related to precocious puberty or abdominal acute or chronic pain, tumors are rare. Ultrasound is the main imaging exam, sufficient in many cases. MRI or CT may be necessary in particular situations. (C) 2013 Elsevier Masson SAS. All rights reserved.

A peripherally inserted central catheter (PICC) is a central venous access mostly used in France in the adult population, whereas it is only rarely used in the pediatric population. The main objective of this study was to analyze a cohort of children treated with PICCs inserted under radiological guidance. We conducted a single-center study in the Radiology department of Nice University Hospital and the Lenval Foundation Children's Hospital. During a 43-month period between November 2008 and June 2012, a total of 67 catheter placement attempts were performed in 57 pediatric patients aged from 7 months to 18 years. We achieved 95.5% technical success with a median procedure duration of 17min. Only 6% of the PICC placements required light intravenous sedation; all the others were performed using a combination of local anesthesia, EMLA cream, and equimolar mixture of oxygen and nitrous oxide (EMONO). Subjective scale analysis of pain during catheter insertion showed a median score of 2.1. Catheter life ranged from 1 to 210 days (median, 38.3 days) with the treatment fully completed in 75% of the cases. The overall complication rate was 18.7% (4.9 per 1000 catheter-days), largely dominated by mechanical complications (9.4%) such as accidental removal (6.2%) or catheter obstruction (3.1%). Infectious complications occurred in 7.8% of the patients. The duration of catheterization and the use of tape to secure the catheter significantly affected the occurrence of complications. Peripheral insertion of central catheters was highly feasible in infants and children. It is a simple, safe, and effective alternative to intravenous central devices in the pediatric population. The occurrence of complications, typically mechanical, must be reduced and prevented by strict management of this type of central line by the nursing team.

STUDY DESIGN:Retrospective analysis of a consecutive cohort of 62 adolescent patients treated by posterior spinal fusion (PSF) with a minimum follow-up of 2 years.OBJECTIVE:To present sagittal and coronal results of a specific method of reduction: the simultaneous translation on 2 rods (ST2R).SUMMARY OF BACKGROUND DATA:The long-term outcome of surgically treated scoliosis is dependent not only on the coronal correction but also on restoration of sagittal curves. Recent publications confirm the moderate correction of thoracic hypokyphosis by posterior instrumentation with hooks or pedicle screws.METHODS:Radiographic parameters were measured preoperatively and at 6 weeks, 1 year, and last follow-up (between 2 and 7.4 years) in a consecutive cohort of 62 patients with adolescent idiopathic scoliosis (AIS) treated by PSF. All operative procedures were performed by the same surgeon using stable anchorages such as screws or self-stabilizing claws. The screws and claws included a polyaxial-threaded extension, which was fixed to the rod with connecting clamps. Reduction of the deformity was obtained by gradual and alternate tightening of the nuts on all threaded extensions on both rods, which allowed the vertebrae to gradually approach the rods while the translation maneuver was performed.RESULTS:In the coronal plane, the average main curve was reduced from 51° to 16° and maintained 70% of correction at last follow-up. In the sagittal plane, for patients with hypokyphosis (27 cases < 20°), the average kyphosis angle was significantly improved from 9° to 29° and maintained during follow-up (32°) with a mean gain of 23° (P < 0.0001). Only 1 patient reported hypokyphosis (18°) at last follow-up. For patients with normal kyphosis, the average gain was 8°.CONCLUSION:In a large consecutive cohort, reduction of scoliosis by ST2R is a simple method that allows 70% of correction in the coronal plane, equivalent to screw instrumentations, and a restoration of normal thoracic kyphosis.

RADIOLOGIE ET IMAGERIE MEDICALE : Genito-urinaire - Gyneco-obstetricale - Mammaire - 34-590-A-11