Purpose. - Interstitial brachytherapy is indicated as part of a conservative strategy for children with bladder and/or prostate rhabdomyosarcoma (RMS), providing high local control probability with acceptable functional results. Vaginal and/or rectal complications were however reported, due to the close proximity to the implanted volume. We investigated the dosimetric impact of a vaginal spacer in terms of rectal and vaginal doses. Methods and patients. - Medical records of 12 consecutive female patients with bladder neck RMS, median age 32 months (range: 1.3-6 years), were reviewed. Five patients were treated prior to 2017 without a vaginal spacer and seven patients treated after 2017 had their brachytherapy delivered with a vaginal spacer placed at time of implant. Results. - Minimal doses delivered to the most exposed 2 cm3, 1 cm3, and 0.5 cm3 of the rectum were all statistically significantly lower among patients treated with a vaginal spacer, as compared to those treated without a spacer. Median rectal D2cm3 was 22 GyEQD2 versus 38 GyEQD2 (P = 0.02), D1 cm3 was 29 GyEQD2 versus 51 GyEQD2 (P = 0.013), and D0.5cm3 was 32 GyEQD2 versus 61 GyEQD2 (P = 0.017), with and without the vaginal spacer, respectively. The posterior vaginal wall D0.5cm3 dose was also significantly decreased, with median D0.5cm3 of 92 GyEQD2 versus 54 GyEQD2 (P< 0.0001), with and without the spacer, respectively. Acute tolerance was excellent in all patients, with no need for replanning and no acute complication. Conclusions. - The use of vaginal spacers in brachytherapy of female pediatric patients with bladder neck RMS resulted in significantly decreased doses to the rectum and the posterior vaginal wall. Though the clinical impact of such dose reduction remains undemonstrated, routine utilization of a vaginal spacer could be a method to decrease long-term morbidity in these patients. (c) 2021 Societe francaise de radiotherapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS:Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS:One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION:Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.

Purpose: We analysed the cohort of paediatric patients with metastatic nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) treated in the BERNIE protocol, i.e. open-label, multicentre, randomised phase II study evaluating the role of bevacizumab (B020924/ITCC-006; ClinicalTrials.gov: NCT00643565). Methods: Eligible patients were randomised 1:1 to add or not add bevacizumab to nine courses of intensive multi-drug chemotherapy, followed by 12-month maintenance chemotherapy (plus surgery and radiotherapy). The primary end-point was event-free survival (EFS); secondary objectives were objective response rate (ORR) and overall survival (OS). Results: From 2008 and 2013, 49 NRSTS patients (out of 154 cases) were treated, 26 in the standard arm and 23 in the bevacizumab arm. ORR was seen in 10 out of 36 evaluable cases (27.7%), i.e. 4/18 standard arm cases and 6/18 bevacizumab arm cases. Two-year EFS was 27.3% (95% confidence interval [CI] 13.9-42.5) for all NRSTS patients, i.e. 34.9% (95% CI 14.6-56.2) for bevacizumab arm and 22.9% (95% CI 7.1-43.9) for standard arm (p value = 0.19). Three-year OS (median follow-up 48.6 months) was 35.2%, with no differences in the two arms. Time to event and time to death were 16.3 and 17.2 months for bevacizumab arm and 2.1 and 7.6 months for standard arm, respectively. Patients not receiving any local treatment on primary disease had a worse outcome as compared to others. Treatment results were better for patients receiving surgical resection and worse for those who did not receive any specific treatment. Conclusion: The addition of the anti-angiogenic agent to the standard chemotherapy did not show statistically significant improvement in survival in metastatic NRSTS. (C) 2020 Elsevier Ltd. All rights reserved.

OBJECTIVES:To evaluate the impact of local therapies on the outcome of patients with liver-bile duct rhabdomyosarcoma (LBDRMS). METHODS:Data of 30 patients included in the EpSSG-RMS 2005 study were analyzed. RESULTS:The median age at diagnosis was 3 years (11 months-8 years). All patients had non-alveolar histology. Fifteen patients had a tumor > 5 cm and six had enlarged regional lymph nodes on imaging. Eight patients (27%) had primary surgery (1 R0). Six of them received external beam radiotherapy (EBRT). All are in first complete remission (CR1) except one (R1, EBRT+ , local relapse, death). Six patients (20%) received EBRT without surgery: one had local relapse and died. Sixteen patients (53%) underwent delayed surgery, with 12 achieving R0 margins, which were higher than those in the primary surgery group (P = 0.003). Three patients with R0 margins received EBRT; one had a metastatic relapse and died. Nine patients with R0 resection did not receive EBRT, three relapsed locally (two deaths). Four R1 patients received additional EBRT without relapses. Local relapse occurred in two among 19 patients with EBRT and three among 11 without EBRT (P = 0.326). At a median follow-up of 61 months (48-84 months), five patients died; all had a tumor size > 5 cm (P = 0.01). The five-year overall survival was 85% (95% CI, 65-94), and event-free survival was 76% (95% CI, 54-89). CONCLUSION:This analysis did not show any significant difference in outcome between irradiated and nonirradiated patients. Local relapse in LBDRMS is related to initial tumor size and is often fatal.

Las masas ováricas, tanto si son orgánicas (tumores benignos o malignos) como funcionales, se diagnostican a cualquier edad durante la infancia o la adolescencia, casi siempre por dolor abdominal o por la palpación de una masa abdominal. La ecografía pélvica suprapúbica con la vejiga llena es el examen de referencia para la exploración de los ovarios, completada con la resonancia magnética abdominopélvica, que permite cartografiar la masa. La determinación de los marcadores tumorales es esencial para el diagnóstico de los tumores ováricos y debe realizarse antes de la cirugía de exéresis. La cirugía es esencial en el tratamiento de la mayoría de las masas ováricas. Es importante poder anticipar la naturaleza probablemente benigna o seguramente maligna de la masa para proponer el tratamiento adecuado. En caso de masa ovárica tumoral que no presente ningún signo clínico, radiológico o biológico sospechoso de malignidad, el acto adecuado es una tumorectomía con conservación del parénquima ovárico, con el objetivo de preservar la fertilidad. A la inversa, debe realizarse una ooforectomía o una anexectomía en caso de un tumor maligno para no comprometer el pronóstico de la paciente. La quimioterapia completa el tratamiento en caso de ciertos tumores malignos.

La préservation de la fertilité des enfants atteints de cancer est un enjeu majeur de leur prise en charge. Les techniques varient en fonction du sexe et de l’âge du patient. Chez le garçon, la protection du testicule de la radiothérapie se fait par blindage de la région testiculaire/pelvienne ou bien par transposition testiculaire. En cas de chimiothérapie, elle varie en fonction de l’âge de l’enfant. Chez le garçon pubère, elle se fait comme chez l’adulte par conservation de sperme. Chez le garçon non pubère, la préservation de la fertilité implique la cryoconservation de pulpe testiculaire pour prélever des spermatogonies souches qui seront réintroduites à l’âge adulte selon 3 techniques : l’injection directe de cellules souches en suspension dans les tubes séminifères ou dans le rete testis, la greffe de fragments de pulpe testiculaire et la maturation in vitro des spermatogonies jusqu’à l’obtention de cellules haploïdes pouvant être utilisées en ICSI. La fertilité des filles traitées par radiothérapie peut être préservée par transposition des ovaires en dehors du champ d’irradiation. En cas de chimiothérapie, la cryoconservation de tissu ovarien est pratiquée depuis 1995 chez la fille pré et post pubère et comporte le prélèvement d’un ovaire et sa congélation pour conservation en vue d’une éventuelle autogreffe ultérieure. Il faudra, comme chez le garçon, une maturation in vivo ou bien in vitro des ovocytes immatures. La préservation de tissu gonadique doit être proposée en cas de traitement à fort potentiel stérilisant même si la maturation des cellules germinales immatures est encore incertaine chez l’enfant et du domaine de la recherche.

Treatment modalities of children with malignancies are chosen in order to achieve high cures rates but also to minimize the risk of definitive sequelae.Radical surgical approaches have been now replaced by conservative treatments involving radiotherapy as part of a multimodal strategy.In this context, brachytherapy has been used in few specialized centers as part of the treatment of patients with rhabdomyosarcoma (RMS), based on the dosimetric advantages of this technique and in an attempt to avoid the long-term side effects of external beam radiotherapy.The management of bladder prostate RMS is an appropriate example of how do surgery and brachytherapy complete each other.In selected bladder prostate RMS, it has been shown that a multimodal strategy based on a conservative surgery combined with brachytherapy of the prostate and bladder/neck was effective for ensuring high cures rates at the expanse of acceptable morbidity rates.The surgery consists of a partial cystectomy and/or partial prostatectomy preserving the muscular layer of the trigone and the urethra.Implantation of plastic tubes is conducted perioperatively through a transperineal approach, with four single leader plastic tubes encompassing the prostate and bladder neck.A ureteral reimplantation and testicular transposition are performed to decrease the risk of ureteral stenosis and to preserve fertility, respectively.Brachytherapy and surgery can be also clearly complementary in the management of gynecological RMS, and the choice of the optimal treatment relies on an estimate of the best therapeutic ratio, to spare fertility.In soft tissues sarcoma surgically treated, the perioperative placement of plastic tubes gives the possibility to have an accurate determination of the volume to be irradiated in case of positive surgical margin.Brachytherapy can also complete with external irradiation.Indeed, brachytherapy is a very appropriate technique for delivering a boost to the primary site when there is regional lymph node extension, or in case brachytherapy should not be given as only treatment because of tumor bulk or because of a too high risk of complication (e.g.: anal RMS).Altogether, these examples illustrate that brachytherapy should be still considered as the best irradiation modality in these selected patients, and that neither surgery nor external irradiation should be seen as competitive, but rather as complementary to give the highest probability of cure with acceptable morbidity.