H. Martelli

Brachytherapy Combined With Surgery for Conservative Treatment of Children With Bladder Neck and/or Prostate Rhabdomyosarcoma.

PURPOSEnTo report the results of a conservative strategy based on partial surgery combined with brachytherapy in a prospective cohort of children with bladder-prostate rhabdomyosarcoma (BP RMS).nnnMETHODS AND MATERIALSnWe prospectively documented the outcome of children treated in our department between 1991 and 2015 for BP RMS and undergoing a multimodal approach combining conservative surgery (partial cystectomy and/or partial prostatectomy) and perioperative interstitial low-dose-rate or pulse-dose-rate brachytherapy. Before brachytherapy, children had received chemotherapy with modalities depending on their risk group of treatment.nnnRESULTSnA total of 100 patients were identified, with a median age of 28xa0months (range, 5.6 months-14 years). According to the Intergroup Rhabdomyosarcoma Study (IRS) group, 84 were IRS-III, and 12 were IRS-IV tumors. Four patients were treated at relapse. The median number of chemotherapy cycles before local therapy was 6 (range, 4-13). After surgery, 63 patients had a macroscopic tumor residuum. Five patients underwent a brachytherapy boost before pelvic external beam radiation therapy because of nodal involvement, and 95 had exclusive brachytherapy. Median follow-up was 64xa0months (range, 6 months-24.5xa0years). Five-year disease-free and overall survival rates were 84% (95% confidence interval 80%-88%) and 91% (95% confidence interval 87%-95%), respectively. At last follow-up most survivors presented with only mild to moderate genitourinary sequelae and a normal diurnal urinary continence. Five patients required a secondary total cystectomy: 3 for a nonfunctional bladder and 2 for relapse.nnnCONCLUSIONnBrachytherapy is effective as part of a conservative strategy for BP RMS, with a relatively low delayed toxicity as compared with previously published studies using external beam radiation therapy. Longer follow-up is required to ensure that the functional results are maintained over time.

Pulsed-dose rate brachytherapy for pediatric bladder prostate rhabdomyosarcoma: Compliance and early clinical results

OBJECTIVEnNo data are available on the feasibility of pulsed dose rate (PDR) brachytherapy in very young children. Our experience of PDR brachytherapy for bladder prostate (BP) rhabdomyosarcoma (RMS) is reported, with focus on compliance and dosimetric parameters.nnnMATERIALS AND METHODSnTreatment parameters and outcome of consecutive BP RMS patients treated in our center were examined. Treatment was carried out as part of a multimodal conservative radio-surgical treatment. Four single leader plastic tubes were implanted under perioperative guidance, encompassing the prostate/bladder neck. Tubes were systematically sutured to the bladder wall. Treatment planning was based on a computed tomography and followed Paris system rules. Continuous hourly pulses of 0.42Gy were delivered, to a total dose of 60Gy.nnnRESULTSn32 patients were treated, median age 2.6years (10months-12years). Median duration of each pulse was 8.5min (3.8-17.4min). The minimal dose in the most irradiated 1cm3 of rectum was 44.7Gyα/β=3 (range: 4.1-77.3Gyα/β=3). Brachytherapy was conducted without significant compliance concern in all but two patients, 24 and 25months-old, who pulled out plastic tubes before treatment completion. Another child needed re-planning because of slight tube displacement. With median follow-up of 14.4months (15days-75months), all patients were free from any relapse after local treatment. Four grade 3 acute urinary complications were encountered. All long-term side effects were grade 2 or less.nnnCONCLUSIONnPDR brachytherapy is feasible in very young patients, and associated with acceptable acute toxicity rates. Longer follow-up is required to ensure that local control and long-term side effects profile are comparable to the results of low dose rate brachytherapy.

Preservation of future fertility in pediatric patients with cancer

The cure rate for childhood and adolescent patients with cancer has currently reached almost 80% and protecting future fertility and thereby promoting quality of life have become a major challenge in the care of these patients (Bioethics Law, 2004). Age, sex and associated treatments influence the risk of future subfertility. Certain chemotherapies (particularly alkylating agents) and radiotherapy fields that include the gonads or hypothalamopituitary axis may negatively impact the future fertility of patients. Evaluation of the gonadotoxic potential of therapeutic measures and the utilization of appropriate methods to preserve fertility require the combined efforts of a multidisciplinary team that includes pediatric oncologists, radiotherapists, surgeons, reproductive physicians and biologists and psychologists. Techniques for fertility preservation vary depending on the age of the child and range from surgical transposition of the gonads for pelvic radiotherapy to cryopreservation of the ovary or testicle in case of sterilizing chemotherapy. While scientists still do not yet fully understand the maturation of immature germ cells, these children will be seeking the assistance of Medically Assisted Procreation (MAP) in 20-30 years. In the meanwhile, it is to be hoped that many more advances will be achieved in the utilization of harvested germinal tissue.

SP-0005: Role of brachytherapy in the management of paediatric tumours

ESTRO 35 2016 _____________________________________________________________________________________________________ the expense of radiation-induced late toxicity. Progressive cell depletion and inflammation are the leading mechanisms of acute toxicity which is observed during or shortly after treatment. The pathogenetic pathways of late toxicity, developing 90 days or later after the onset of radiotherapy, are more complex and involve processes such as vascular sclerosis and fibrosis. Since many patients have become longterm survivors, awareness and recognition of radiationrelated toxicity has gained in importance and increased efforts are made for its prevention and management. Technical innovations contribute to a reduction in radiotherapy-associated toxicity. The steep dose gradients of highly-conformal radiotherapy techniques allow for an accurate dose delivery with optimal sparing of the normal tissues. Several studies have demonstrated the dosimetrical benefit of intensity-modulated radiotherapy (IMRT) and volumetric modulated arc radiotherapy (VMAT) compared to conventional radiotherapy techniques. It has been shown that the dosimetrical benefit of IMRT translated into a clinically significant reduction in lower gastrointestinal toxicity compared with three-field conventional radiotherapy. In the near future MRI-linacs and proton therapy are likely to broaden the therapeutic window further. Prone positioning on a bellyboard reduces small bowel toxicity by pushing away the small bowel loops from the high dose region. Imageguided radiotherapy allows for an accurate definition, localization and monitoring of tumor position, size and shape before and during treatment and may help to reduce set-up margins. Small randomized controlled trials have shown that the administration of several agents might have a beneficial effect for the prevention of acute (e.g. intrarectal amifostine, oral sulfasalazine and balsalazide) and/or lateonset radiation-induced toxicity (intrarectal beclomethasone and oral probiotics). Once severe toxicity develops, total replacement of the diet with elemental formula may be appropriate. Probiotics influence the bacterial microflora and seem promising in reducing the incidence and severity of radiation-induced diarrhea. Currently there is insufficient evidence for cytoprotective and anti-inflammatory drugs in the management of radiation-induced toxicity. Future challenges lie in the prediction of treatment-related toxicity, which might be a promising step towards an individualized risk-adapted treatment.

Invaginación intestinal aguda en lactantes y niños

La invaginacion intestinal aguda es la causa mas frecuente de oclusion intestinal en lactantes y ninos menores de 2xa0anos. Se define como la penetracion de un segmento de intestino en la luz del segmento inmediatamente adyacente por un mecanismo de inversion en dedo de guante. Se relaciona con un problema del peristaltismo intestinal cuya etiologia aun no se conoce por completo. La incidencia global de la invaginacion varia segun la zona geografica y el nivel sanitario. En mas del 60% de los casos, se produce en menores de 1xa0ano. En el 90% de los casos, es idiopatica. El 10% de las invaginaciones intestinales agudas son secundarias a una causa local, una enfermedad general o un contexto particular. La triada clinica clasica consiste en la asociacion de dolor abdominal, vomitos y rectorragia, pero solo se observa en un tercio de los pacientes. La clasificacion de Brighton, definida para permitir la recopilacion estandarizada de los datos sobre la invaginacion, puede tener una utilidad clinica practica. La ecografia abdominal es la exploracion clave que permite confirmar el diagnostico. La invaginacion intestinal aguda es una urgencia terapeutica. Puede reducirse sin cirugia ejerciendo una contrapresion en el intestino distal mediante un enema retrogrado neumatico o hidrostatico (si no existen contraindicaciones) o reducirse manualmente por el cirujano. El tratamiento no quirurgico es eficaz en el 90% de los casos. El tratamiento quirurgico (a cielo abierto o por via laparoscopica) se efectua cuando existen contraindicaciones para el enema o si este fracasa, asi como en algunas circunstancias especiales. El pronostico de esta afeccion es excelente, pero requiere una colaboracion estrecha entre los equipos pediatrico, quirurgico, radiologico y anestesico.

SFCP CO-73 - Tumeurs rhabdoïdes du foie : étude observationnelle de 5 cas pédiatriques

Objectifs Les tumeurs rhabdoides hepatiques (TRH) sont rares et agressives, survenant essentiellement chez les enfants de moins de 1 an. L’objectif de cette etude est de decrire les traitements recus et la survie de patients avec TRH. Patients et methode Il s’agit d’une etude unicentrique retrospective de 5 patients traites pour TRH entre le 1 er janvier 2007 et le 31 decembre 2013. Les variables etudiees ont ete l’âge au diagnostic, la presence de metastases, le traitement recu et la survie globale. Resultats L’âge median au diagnostic etait de 6 mois (4–23). Dans tous les cas, le diagnostic a ete histologique avec absence d’expression du gene INI1. Aucun n’enfant n’etait metastatique au diagnostic. Tous les patients ont eu de la chimiotherapie et de la chirurgie. Un seul a eu de la radiotherapie. Les 3 patients (60%) vivants ont tous recu de la doxorubicine et ont eu une chirurgie moins de 3 mois apres le diagnostic. Le recul median est de 9 mois (9–80). Conclusions La survie serait amelioree par une chimiotherapie intensive contenant de la doxorubicine et une chirurgie precoce.

SFCP CO-51 - Prise en charge unicentrique de 60 hernies de coupole diaphragmatique

Objectifs Etudier la correlation entre l’image antenatale et le devenir clinique des patients ayant une hernie de coupole diaphragmatique et analyser les resultats a court et moyen terme. Materiels et methodes Etude retrospective de 60 enfants de 2010 a 2013. Nous avons etudie le LHR prenatal, l’utilisation d’un plug tracheal, la technique chirurgicale, la duree d’hospitalisation, les complications et la survie globale. Resultats Le diagnostic antenatal a ete fait chez 49 enfants. Selon le LHR, 21 (43%) formes favorables, 15 (30%) intermediaires et 13 (27%) severes. Seize enfants ont eu un plug. La chirurgie a ete realisee chez 46 (77%) enfants, 13 ont eu un patch. La duree d’hospitalisation mediane etait de 25 jours (7–132). Trois patients sont rentres a domicile avec une nutrition enterale et 2 avec une oxygenotherapie temporaires. Sept patients ont eu une occlusion sur bride et 3 une recidive. Aucun geste de gastrostomie ou anti-reflux n’a ete pratique. Vingt (33%) enfants sont decedes: 9 (45%) formes severes, 8 (40%) intermediaires et 3 (15%) legeres. Le recul median est de 23,5 mois (4–47). Conclusions Le LHR semble etre un bon facteur pronostic. Meme si la mortalite reste severe, les complications associees a une faible morbidite permettent une qualite de vie satisfaisante.

SFCP CO-74 - Chirurgie des métastases hépatiques des néphroblastomes : expérience monocentrique

Introduction Les metastases hepatiques de nephroblastome (MHN) sont rares et leur prise en charge est specialisee. Materiel et methodes Etude monocentrique retrospective (1993–2010) incluant les patients (pts) ayant une chirurgie hepatique pour MHN. Resultats Dix pts ont ete identifies : 4 pts avec metastases synchrones, 6 pts metachrones (delai moyen de rechute de 23 mois [1–123]). Toutes touchaient le lobe hepatique droit. Elles etaient multiples 4 fois et unique 6 fois. Une atteinte pulmonaire etait associee chez 5 pts (dont les 4 synchrones). Localement, dans 40% des cas le stade de la tumeur etait I, II dans 40%, III dans 20%. Elle etait a droite chez 6 pts. Le geste chirurgical etait une chirurgie reglee pour 6 pts et une resection atypique pour 4. Un patient a necessite la pose d’un packing. Il n’y a pas eu de mortalite peroperatoire. La resection a ete microscopiquement complete pour 8 patients. Une chirurgie pulmonaire a ete faite chez 4 pts. 3 patients ont presente une rechute hepatique avec un delai moyen de 5 mois. Parmi eux, 2 sont decedes. La survie globale est de 80% dans cette serie. Conclusion Le pronostic des enfants ayant une resection complete des MHN persistant apres chimiotherapie est bon. Une prise en charge chirurgicale agressive est necessaire.