Sainte-Foy-lès-Lyon, France
Sainte-Foy-lès-Lyon, France

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Zacharoulis S.,Royal Marsden Hospital | Ashley S.,Royal Marsden Hospital | Moreno L.,Royal Marsden Hospital | Gentet J.-C.,Hopital de la Timone | And 2 more authors.
Child's Nervous System | Year: 2010

Introduction More than a third of children with ependymoma experience relapse, and despite multimodal treatment, less than 25% of them can then achieve long-term survival. Prognostic factors for patients who relapse have not been extensively analyzed. Patients and methods We retrospectively analyzed 82 patients from four pediatric oncology European institutions in order to identify prognostic factors and influence of treatment modalities in relapsed ependymoma. Results First relapse occurred after a median of 19 months (1 month-16 years). Five-year progression-free survival and overall survival of the cohort were 17% and 27.6%, respectively. Survival was statistically significantly higher for patients achieving gross total resection. No survival benefit was seen for children receiving chemotherapy whereas patients who were amenable to some form of reirradiation had a better outcome. Objective responses were found in more than 25% of patients receiving oral etoposide, temozolomide, or vincristine/etoposide/cyclophosphamide regimens. Multivariate analysis confirmed that patients with mixed relapses, no surgery at relapse, and receiving chemotherapy did worse (hazard ratio=3.6, 3.3, and 1.7, respectively, all p<0.05). Discussion Relapsed ependymoma carries a very poor prognosis with an indolent chronic course, leading to death in approximately 90% of the patients. Complete surgical resection whenever possible should be encouraged. Radiation therapy of the relapsed lesions can provide some minor benefit whereas chemotherapy despite the occasional responses provides no benefit in the final outcome which is dismal. Efforts have to be orchestrated internationally to enroll these patients on clinical trials using biology-based therapies. © Springer-Verlag 2009.


PubMed | Lyon University Hospital Center, Nantes University Hospital Center, University Paris - Sud, Montpellier University Hospital Center and 3 more.
Type: Journal Article | Journal: Bulletin du cancer | Year: 2015

Sex-cord stromal tumors (SCT) are rare pediatric tumors accounting for less than 5% of gonadal tumors in children and adolescents. They differ from those diagnosed in adults by their presentation, histology, evolution and treatment modalities. Testicular SCT occur mostly in infants less than 6 months. Testicular swelling is often the only symptom, but signs of hormonal secretion with gynecomastia may be present. Juvenile granulosa SCT is the main histologic subtype. Sertoli SCTs are much less frequent while Leydig tumors occurred in older children and adolescents. Prognosis is excellent after inguinal orchiectomy. Testis sparing surgery could be performed but indications and modalities have to be strongly defined. Ovarian SCT are diagnosed in older children and adolescents and present with abdominal symptoms and/or signs of hormonal secretion: estrogenic manifestations (isosexual pseudoprecocity, menometrorrhagia) or virilization (hirsutism, amenorrhea). Main histologic subtype is juvenile granulosa (rarely Sertoli-Leydig). If oophorectomy (or salpingo-oophorectomy) may be curative for localized disease, adjuvant cisplatin-containing chemotherapy is mandatory in case of tumor rupture or peritoneal dissemination to prevent recurrences. Because of the rarity of these pediatric tumors, concerted multidisciplinary cares are required to best adapt therapeutic strategy before any surgical intervention.


Calaminus G.,University Childrens Hospital | Kortmann R.,University of Leipzig | Worch J.,University Childrens Hospital | Nicholson J.C.,Addenbrookes Hospital | And 7 more authors.
Neuro-Oncology | Year: 2013

BackgroundWe conducted a nonrandomized international study for intracranial germinoma that compared chemotherapy followed by local radiotherapy with reduced-dose craniospinal irradiation (CSI) alone, to determine whether the combined treatment regimen produced equivalent outcome and avoided irradiation beyond the primary tumor site(s).MethodsPatients with localized germinoma received either CSI or 2 courses of carboplatin and etoposide alternating with etoposide and ifosfamide, followed by local radiotherapy. Metastatic patients received CSI with focal boosts to primary tumor and metastatic sites, with the option to be preceded with chemotherapy.ResultsPatients with localized germinoma (n = 190) received either CSI alone (n = 125) or combined therapy (n = 65), demonstrating no differences in 5-year event-free or overall survival, but a difference in progression-free survival (0.97 ± 0.02 vs 0.88 ± 0.04; P =. 04). Seven of 65 patients receiving combined treatment experienced relapse (6 with ventricular recurrence outside the primary radiotherapy field), and only 4 of 125 patients treated with CSI alone experienced relapse (all at the primary tumor site). Metastatic patients (n = 45) had 0.98 ± 0.023 event-free and overall survival.ConclusionsLocalized germinoma can be treated with reduced dose CSI alone or with chemotherapy and reduced-field radiotherapy. The pattern of relapse suggests inclusion of ventricles in the radiation field. Reduced-dose craniospinal radiation alone is effective in metastatic disease. © 2013 The Author(s).


Benesch M.,Medical University of Graz | Frappaz D.,Institute dHemato Oncologie Pediatrique | Massimino M.,Instituto Nazionale
Child's Nervous System | Year: 2012

Background Spinal cord ependymomas are very rare among children and adolescents. Due to their rarity, our current knowledge of these tumors is based on case reports and few retrospective case series. Methods The present review summarizes the currently available literature on childhood spinal cord ependymomas. Results Although overall survival rates are favorable, relapse incidence is high, particularly in myxopapillary ependymomas. Since long-term follow-up data are provided in a limited number of studies only, the true relapse incidence is unknown. Maximal safe radical surgery is the backbone of treatment for children with spinal cord ependymomas, but the impact of adjuvant treatment on progression and survival is still unclear. Presently, the decision to initiate nonsurgical treatment depends primarily on the WHO grade of the tumor and the extent of resection. In terms of the known side effects, early radiotherapy should be avoided in children with WHO grade II spinal cord ependymomas irrespective of the extent of resection but is indicated in anaplastic spinal cord ependymomas both after complete and incomplete resection. The high relapse incidence in myxopapillary ependymomas argue for the use of early radiotherapy, but its definitive impact on progression has to be proven in larger series. Close surveillance is important due to the high recurrence rate in all patients with spinal cord ependymomas. Conclusion Prospective collection of both clinical and molecular data from a greater number of patients with spinal cord ependymomas within an international collaboration is the prerequisite to establish standardized management guidelines for these rare CNS tumors. © Springer-Verlag 2012.


PubMed | French Scientific and Technical Center for Building, Center Oscar Lambret, Nantes University Hospital Center, Institute dhemato oncologie pediatrique and 3 more.
Type: Journal Article | Journal: Bulletin du cancer | Year: 2016

Therapeutic education (TE) is a practice developed over 40years at an international level to give people with chronic illness the skills necessary to help them better manage their disease. The lengthening survival time of cancer patients as well as changes in the patient-caregiver relationship have contributed to the development of TE in oncologic diseases. Every year in France, about 1900adolescents and young adults (15-25years old) are diagnosed with cancer which is the second leading cause of death in this age group. The observed survival rates for these patients are lower when compared with childrens. Some of the hypotheses put forward to explain this difference include a lack of constancy in care and a non-following treatment, as failure to adhere to therapies is common in this age group. Go-AJA, an interdisciplinary national organization established in 2012, aims to improve the quality of care and treatment results for AYA living with cancer. Therapeutic education for AYA in oncology is an active working group of Go-AJA and intends to draw recommendations and to improve adapted communication on different education topics. Elaboration and preparation of TE programs by skilled multidisciplinary teams engaged in interactive educational actions is the first and most crucial step.The TE Go-AJA working group has federated pediatric and adult oncologists, nurses, psychologists, TE professionals, and resource patients, thanks to the commitment of professionals from the 8 national teams supported by the National Cancer Institute. Physical meetings and conference calls were organized from 2012 to 2015 to construct TE tools and programs for AYA with cancer.A competence referential was built and adapted to AYA population with cancer, after focused groups organized in 2main oncology centers with on-therapy sarcoma patients and members of the multidisciplinary TE working group. Tools were validated and adapted to adolescents or young adults with cancer, to help in the 4stages of TE: the educational diagnosis allowing the caregiver to better understand the patient in his life journey with the disease; the therapeutic alliance allowing to agree with the patient on his/her priorities; the implementation which is an action step: information, awareness, learning and psychosocial support. The final step called assessment allows the caregiver to take stock on the changes and difficulties with the patient. TE for AYA with cancer included individual and/or group sessions to improve self-care skills: knowledge about the disease (group sessions what is cancer? with use of microscopes to visualize sarcoma cells, and guided tours in a tumor research laboratory), and details about the treatment and its consequences (workshops about management of febrile neutropenia). Moreover, TE aimed to enrich the field of coping skills, in particular to improve the coordination and experience of cares between the different complex and varied network of care (group and/or individual sessions focused on physical rehabilitation, and adapted school/professional orientation).Regardless of the care system, care workers dedicated to AYA with cancer should use TE-specific actions to reinforce treatment participation and therapeutic relationships. This active multidisciplinary TE working group dedicated to AYA with cancer elaborated TE programs by skilled multidisciplinary teams engaged in interactive educational actions. After this work of a national TE organization, more studies using methodological tools are still required to evaluate the impact of such implemented programs on the treatment results and the quality of life.


PubMed | Center Hospitalier Lyon Sud, Institute dHemato Oncologie Pediatrique, Tissue and Cells Bank, Histocompatibility Laboratory and Registre France Greffe de Moelle
Type: Journal Article | Journal: Human immunology | Year: 2016

In the absence of an HLA matched familial donor, a search for an unrelated donor or cord blood unit is initiated through worldwide registries. Although a first look-up on available HLA information of donors in the book at BMDW (Bone Marrow Donor Worldwide) can provide a good estimation of the number of compatible donors, the variety of resolution typing levels requires confirmatory typing (CT) which are expensive and time consuming. In order to help recipient centers in their work. The French donor registry (France Greffe de Moelle/Agence de la Biomedecine) has recently developed a software program called EasyMatch that uses haplotype frequencies to compute the likelihood of phenotypic match in donors according to various typing resolution levels. The goal of our study is to report a single monocentric user-experience with EasyMatch, demonstrating that its routine use reduced the cost and the delay of the donor search in our center, allowing the definition of a new strategy to search compatible unrelated donors. The strategy was first established on a retrospective cohort of 217 recipients (185 adults and 32 children=before score) and then validated on a prospective cohort of 171 recipients (160 adults and 11 children=after score). For all patients, we calculated the delay between the registration day and the donor identification day, and the number of CT requested to the donor centre. Considering both groups, we could observe a significant decrease of the number of CT from 8 to 2 (p<0,001), and a significant decrease of the median delay to identify a suitable donor from 43 to 31days (p<0.0001). EasyMatch estimates the number of potentially identical donors, but doesnt foresee availability of the donors. It provides us an easy tracking of mismatches, an estimation of the number of potential donors, the selection of population following ethnic origin of patients and a high prediction when probability is high or low. It affords a new approach of donor search in our daily work and improves the efficiency in the great challenge of the compatible donor identification.


PubMed | Institute dhemato oncologie pediatrique and Institute Claudius Regaud
Type: Journal Article | Journal: Bulletin du cancer | Year: 2016

Management of extracranial germ cell tumors (TG) is emblematic of the adolescents and young adults problematic. Beyond persisting differences in care between adult and pediatric oncologists, it emphasizes the need for effective collaboration between both teams. If the therapy is primarily guided today by adult standards, pediatricians bring expertise in long-term follow-up, justified by the increasing description of late side effects in this young population, with highly curable disease since cisplatins emergence.


PubMed | Institute dhemato oncologie pediatrique, Center Leon Berard and University of Lyon
Type: Journal Article | Journal: Bulletin du cancer | Year: 2016

General anesthesia (GA) is often needed for radiotherapy (RT) in young children. This study aimed to evaluate the place of the rituals and/or hypnosis in pediatric in a reference center in pediatric radiation oncology in Rhne-Alpes Auvergne.This observational study retrospectively collected data on AG in children<5years treated by RT in Leon-Berard regional center, Lyon, France between 2003and 2014. Two-time periods, before and after 2008have been compared, the second one introducing accompaniment methods such as hypnosis systematically. Explanatory analyses of AG were performed using logistic regression.One hundred and thirty-two children benefited from RT in that period and were included (70patients until 2008, 62after 2008). Fifty-three percent were irradiated under GA. There was significant reduction (P<0.1) in the use of GA after 2008. The use of GA was not significantly associated with the RT techniques. The patients more likely to undergo RT without GA were the oldest and the patients treated for abdominal lesions (P<0.01).The study confirms that rituals and hypnosis can be used instead of GA in about half of patients under 5years, even also with high-technicity RT requiring optimal immobilization.


PubMed | Institute dhemato oncologie pediatrique and University of Lyon
Type: | Journal: World neurosurgery | Year: 2016

Primary leptomeningeal melanomatosis (PLM) is a rare and aggressive form of nonmetastatic invasion of leptomeninges by malignant melanocytic cells. Clinical presentation includes nonspecific meningism with various forms of cerebrospinal fluid circulation or absorption disorders leading to hydrocephalus.A 5-year-old child with PLM without neurocutaneous melanosis presented with cystic enlargement of the brainstem surrounding cisterns and hydrocephalus requiring occipitoaxial decompression and endoscopic cystostomy. Initial cerebrospinal fluid cytology screening and frontal meningeal and brain biopsy specimens showed negative results. The diagnosis of unpigmented (amelanotic) malignant melanocytic cells was made after a biopsy specimen of the bulbar leptomeninges was obtained. Despite continuous management of hydrocephalus and chemotherapy, the disease progressed, and the patient died 11 months after diagnosis.To the best of our knowledge, this is the first report of an amelanotic form of PLM without association of neurocutaneous melanosis in a child. This case report illustrates the difficulty of diagnosis in the absence of cutaneous lesions and lack of melanin.


Moos D.,Center Hospitalier Of Valence | Droitcourt C.,Center Hospitalier Of Valence | Rancherevince D.,Center Leon Berard | Marec Berard P.,Institute dHemato Oncologie Pediatrique | Skowron F.,Center Hospitalier Of Valence
Pediatric Dermatology | Year: 2012

We report a large infiltrating atypical granular cell tumor in a child with Noonan syndrome. Even though granular cell tumors are rare in childhood, five cases have been reported in children with Noonan syndrome. This study compares these different cases and explores the possibility of activation of the granular cell by the Ras pathway. © 2012 Wiley Periodicals, Inc.

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