I tumori del sistema nervoso periferico (SNP) comprendono dei tumori benigni essenzialmente rappresentati dagli schwannomi e dai neurofibromi, e dei tumori maligni raggruppati sotto il termine di tumori maligni della guaina dei nervi periferici (malignant peripheral nerve sheath tumours [MPNST]). Questi tumori sono sporadici o si sviluppano nell'ambito di una facomatosi. La neurofibromatosi di tipo 1 (NF1) si caratterizza per la presenza di neurofibromi e di eventuali tumori maligni delle guaine dei nervi periferici. Gli schwannomi multipli sono un criterio diagnostico della neurofibromatosi di tipo 2 (NF2), oramai definita schwannomatosi legata al gene NF2. Gli schwannomi o neurinomi sono tumori ben delimitati che formano una massa eccentrica nel tragitto di un nervo. In istologia, la proliferazione è costituita da cellule di Schwann organizzate in fasci compatti (zone A di Antoni) o in zone più lasse ed edematose (zone B di Antoni). I nuclei delle cellule possono allinearsi e formare palizzate che delimitano spazi che corrispondono ai noduli di Vérocay, numerosi nelle zone A di Antoni. Le cellule tumorali esprimono le proteine S100 e Sox10 in immunoistochimica. I neurofibromi sono tumori mal delimitati spesso cutanei. Quando si sviluppano nel percorso di un nervo, determinano un aumento di volume fusiforme. La proliferazione tumorale è costituita da cellule di Schwann, da cellule perineurali e da fibroblasti organizzati su un fondo mixoide in cui sono visibili gli assoni. Il trattamento degli schwannomi e dei neurofibromi consiste in un'exeresi chirurgica. I tumori maligni delle guaine dei nervi periferici si sviluppano in oltre il 50% dei casi in un contesto di NF1. Macroscopicamente, si tratta di tumori a crescita rapida che determinano di frequente rimaneggiamenti necroticoemorragici e un marcato potere di invasione locoregionale e, in seguito, generale. L'istologia mostra una proliferazione di cellule fusiformi con un'alternanza di zone compatte e di zone più lasse. Le cellule mostrano atipie citonucleari ed esprimono la proteina S100 e Sox10 in modo più eterogeneo e più debole rispetto ai tumori benigni. Nel 15% dei casi di forma convenzionale, esistono aspetti morfologici inconsueti con una differenziazione divergente, per esempio muscolare striata nel tumore di Triton o talvolta ghiandolare. Sono stati descritti anche due sottotipi anatomoclinici e molecolari: gli MPNST epitelioidi e melanotici. Gli MPNST sono generalmente tumori a alto grado di malignità e la loro prognosi è riservata. Più recentemente, sono stati descritti tumori di prognosi incerta (ANNUBP - atypical neurofibromatous neoplasm of uncertain biological potential) e MPNST a basso grado di malignità che insorgono in un contesto di NF1. Il trattamento rimane essenzialmente chirurgico. In alcuni casi, è discussa una chemioterapia in una riunione di concertazione multidisciplinare.
OBJECTIVE:Exploratory laparoscopy plays a critical role in assessing resectability in suspected advanced-stage ovarian cancer. Performed early, it guides treatment toward either complete cytoreductive surgery or neoadjuvant chemotherapy. With the implementation of B5 accreditation for expert centers, a French national certification system designating specialized oncologic referral centers, peripheral hospitals are becoming key actors in diagnosis and staging. A well-conducted laparoscopy avoids delays and repeat procedures, thereby improving patient outcomes. This study presents a 10-step laparoscopic protocol that is anatomically ordered, simple, and reproducible, facilitating adoption across both expert and partner centers. SETTING:Women undergoing initial assessment for suspected advanced-stage ovarian cancer prior to therapeutic decision-making. PARTICIPANTS:Patients with suspected advanced-stage ovarian cancer referred for diagnostic laparoscopy before determination of the initial treatment strategy. INTERVENTIONS:This video outlines a standardized exploratory sequence using a strict midline laparoscopic approach (single supraumbilical trocar or two aligned trocars). Each of the 12 regions of the Peritoneal Cancer Index (PCI) is documented photographically. Resectability is assessed using three validated tools: Sugarbaker's PCI, the Fagotti score, and the Makar classification. CONCLUSION:The 10 steps include: trocar placement; peritoneal cytology with ascites quantification; inspection of the upper abdomen (right and left diaphragmatic domes, liver, spleen); evaluation of the omentum and transverse colon; exploration of the small bowel and mesentery (Trendelenburg); assessment of both iliac fossae and the pelvis; followed by photographic documentation and biopsies. Key elements include early peritoneal cytology and at least four to six wide (10-mm) biopsies obtained using cold scissors. Epiploic biopsy is prioritized for histologic and molecular analysis. Biopsies near hollow organs (bowel, bladder) are avoided to reduce procedural complications. The standardized 10-step sequence is summarized in Annexe 1 to facilitate reproducibility and clinical adoption. This reproducible and structured 10-step approach strengthens the role of partner centers in advanced ovarian cancer care. It improves diagnostic quality, harmonizes staging practices, and supports timely, expert-level treatment planning. Widespread adoption may reduce disparities and promote equitable care. VIDEO ABSTRACT.
INTRODUCTION:The submental flap, first described by Martin and Baudet in 1990, was originally developed for the reconstruction of defects involving the lower two-thirds of the face. Owing to its reliable vascular pedicle and anatomical proximity, its indications have progressively expanded to include complex soft-tissue losses of the hemiface, particularly at the oral commissure, where both aesthetic and functional demands are high. METHODS:We report the case of a 70-year-old patient presenting with a right cheek and labial commissure defect following an animal bite, associated with loss of the modiolus and orbicularis oris muscle sling. Reconstruction was performed in two stages: (1) restoration of the orbicularis continuity and coverage of the defect using an insular submental flap pedicled on the right submental artery, and (2) secondary commissuroplasty four months later to restore labial continence and facial symmetry. RESULTS:The first stage achieved complete coverage of the composite defect and reestablishment of muscular continuity. Following commissuroplasty, the patient recovered satisfactory labial motility, oral opening, and functional continence. Subsequent staged debulking procedures refined the aesthetic outcome while maintaining flap trophicity and hair-bearing skin texture. DISCUSSION:This case highlights the reliability and versatility of the submental flap in commissural reconstruction. Its favorable skin characteristics, robust vascularization, and wide arc of rotation make it an effective alternative to heterolabial flaps, allowing a "like-with-like" reconstruction that achieves both durable functional recovery and harmonious aesthetic integration.
OBJECTIVE:To evaluate the neonatal prognosis of preterm infants born before 29 weeks of gestation based on their mode of conception (Medically Assisted Reproduction vs spontaneous pregnancy). DESIGN:A retrospective case-control study was conducted in the neonatal intensive care unit of the Hôpital de la Conception in Marseille. SUBJECTS:All preterm newborns under 29 weeks of gestation hospitalized between 2014 and 2020 were included. Newborns conceived through Medically Assisted Reproduction were compared to a control group of newborns from spontaneous pregnancies, matched at a ratio of 1 case to 3 controls. EXPOSURE:Medically assisted reproduction. MAIN OUTCOME MEASURES:The primary outcome was a composite criterion including the number of deaths, bronchopulmonary dysplasia at 36 weeks of gestation, severe intraventricular hemorrhage (grade 3 or higher), severe retinopathy of prematurity (grade 3), and/or necrotizing enterocolitis (grade 2 or higher). Secondary outcomes included Apgar score, cesarean section rate, and average age at discharge from neonatal intensive care. RESULTS:Among the 616 hospitalized newborns, 18.8 % were conceived through Medically Assisted Reproduction (n = 112). A total of 448 patients were included, with 112 cases and 336 controls. Among the cases, 21 were conceived via intrauterine insemination, 14 through ovulation stimulation with clomiphene, 54 through In Vitro Fertilization, and 23 through IntraCytoplasmic Sperm Injection. Multiple pregnancies were more common in the Medically Assisted Reproduction group (61.6 %) compared to spontaneous conception (30.6 %). No significant difference was observed in the neonatal prognosis between newborns conceived via Medically Assisted Reproduction and those from spontaneous pregnancies (74.1 % vs 77.1 %, p = 0.52). The cesarean section rate was significantly higher among mothers who underwent Medically Assisted Reproduction (83 % vs 59.2%, p < 0.05). CONCLUSION:This study suggests that newborns under 29 weeks of gestation conceived through Medically Assisted Reproduction have a neonatal prognosis comparable to those from spontaneous pregnancies, although cesarean section rates are higher among mothers who used Medically Assisted Reproduction. TRIAL REGISTRATION:None.
Objectifs: La qualité de la prise en charge médicamenteuse est un processus complexe et particulièrement à risque de survenue d’erreurs médicamenteuses. L’intérêt de rappeler les bonnes pratiques aux professionnels de santé apparait essentiel pour l’amélioration de la qualité et la sécurité des soins. L’utilisation d’une formation sous forme d’escape game a été retenue. Ce travail décrit la conception et la mise en œuvre d’un escape game original et pédagogique centré sur cette thématique.Méthodes: La conception de l’escape game « Les mystères de la chambre 651 » a nécessité la mise en place d’un groupe de travail multidisciplinaire. Il intègre trois activités de la prise en charge médicamenteuse : prescription, stockage / rangement, préparation / administration des médicaments. Les connaissances ont été évaluées avant et après la formation par un quiz de 10 questions et une enquête de satisfaction a été réalisée à l’issue de chaque session.Résultats: Une version initiale du jeu a été élaborée par le groupe de travail puis une phase fondamentale de « tests/modifications » a permis d’aboutir à la version finale. Sur une année, près de 40 sessions ont été réalisées, permettant la formation de 258 professionnels, principalement des infirmiers diplômés d’État. La satisfaction globale des participants était supérieure à 98,1 %. La moyenne globale du quiz d’évaluation est passée de 7,51/10 avant formation à 8,38/10 (p < 0,001) après formation.Conclusion: Cette formation constitue un outil efficace et apprécié dans la formation continue des professionnels de santé pour le rappel des bonnes pratiques de la prise en charge médicamenteuse en unité de soins.
