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Background: Advanced practice nursing was introduced in France in 2018, in response to health needs. The first advanced practice nurses were graduated since 2019 and were trained in one among four medical areas including oncology and onco-hematology. The purpose of this article is to make an early assessment of the development of the profession of oncology Advanced Practice Nurse in France., Method: An exploratory study was conducted. A sample of 44 onco-hematology IPA graduated in 2019 and 2020 was recruited from June 2021 to end of July 2021. The 44 participants completed a questionnaire, by phone interviews or self-administered., Results and Conclusion: The distribution of the 44 participants concerns 12 of the 13 regions of metropolitan France. This profession shows an employability for 86% of the first graduates. These professionals practice in health care institutions and rather in oncology, 71% in the framework of an organizational protocol established with the oncologist. They appear to be well accepted by patients and oncology teams. Further studies on performance and quality indicators will make it possible to evaluate the added value of the oncology Advanced Practice Nurses in the cancer patient's pathway., (Copyright © 2022 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Objectives: Intramedullary gliomas are rare tumors accounting for less than 4% of all primary central nervous system tumors. The aims of this retrospective multicenter study were to assess their natural outcome as well as management., Methods and Materials: We studied 332 patients from 1984 to 2011. Histopathological examination revealed 72% ependymomas (94% were low grade tumors), 24% astrocytomas (29% were high grade tumors), 2.4% mixed gliomas and 1.7% oligodendrogliomas., Results: The mean age at diagnosis was 42.4 years for ependymomas, with male predominance, versus 39.6 years for astrocytomas. Pain was the most common initial presentation. In 20% of cases, astrocytomas were biopsied alone, but more than 80% of ependymomas had surgical resection. Radiotherapy and chemotherapy were reserved for malignant tumors, especially if they were ependymomas. The 5-year survival rate was 76.8% for astrocytomas and 94.5% for ependymomas. Histology, functional status prior to surgery, and tumor grade are among the prognostic factors., Conclusion: Our study showed that surgical treatment of gliomas is well codified, at least for ependymomas, but adjuvant treatment continues to play a marginal role in the management even in astrocytomas, which are infiltrative tumors., (Copyright © 2017. Published by Elsevier Masson SAS.)

Malignant gliomas are the most frequent primary brain tumors in adults. Temozolomide is an oral alkylating cytotoxic agent of second generation, used in the treatment of high-grade gliomas. It is indicated in newly diagnosed glioblastoma multiform as well as in recurrent or progressive malignant gliomas, such as glioblastoma multiform or anaplastic astrocytoma. However, temozolomide is also used, off label, in other clinical situations and the main objective of this study was to establish recommendations and guidelines for relevant prescriptions of temozolomide in primary brain tumors and brain metastasis in adults. The literature review was analysed by experts who determined the evidence level (A to E) according to the scale of recommendations adopted by the "Haute Autorité de santé--HAS--(French National Authority for Health)". For high-grade and low-grade gliomas, based on the level of evidence from the literature, the use of temozolomide can be justified, with a B2 score attributed to these indications. In contrast, for the others indications, the use of temozolomide appeared to be more controversial or even not recommended (score C to E). Regarding the dosing schedule and administration scheme, as well as the co-administration with other anticancer drugs, a C score was attributed for the off label situations.

Unlabelled: The recurrence and progression of treated intracranial meningiomas highlights the problem of the type of follow-up that should be used and whether early complementary treatment is indicated. The aim of this study was to evaluate different biochemical markers involved in cell proliferation and transformation to identify new prognostic factors in intracranial meningiomas. Between 1989 and 2003, 120 intracranial meningiomas were studied biochemically. The levels of estrogen receptors (RE), progesterone receptors (RP), cathepsin B (CB), cathepsin L (CL), stefin A (ATA), stefin B (STB), cystatin C (CYSC), urokinase (u-PA), type 1 plasminogen activator inhibitors (PAI-1), cathepsin D (CD) and thymidine kinase activity (TK) were measured in tumor extracts using biochemical assays., Results: Out of 120 meningiomas, 73 were grade I, 39 grade II and eight grade III according to the WHO classification. Of these patients, 17 showed recurrence. The mean follow-up was 47 months. Monofactorial analysis showed that expression of progesterone receptors (RP) had an inverse correlation with recurrence (p=0.0025 %) and that thymidine kinase activity (TK), cathepsin L (CL), the WHO grade and the degree of tumor resection correlated with recurrence (p<0.05). Principal component analysis and linear discriminant analysis confirmed these results. The results of this study confirm the importance of biological parameters (PR, CL, TK) as prognostic factors for the risk of recurrence in intracranial meningiomas.

Introduction: Neoangiogenesis is a critical feature that can differentiate high-grade from low-grade glioma. Conventional MR imaging does not assess this histological feature accurately. The goal of this study was to evaluate the gain in relative cerebral blood volume measurement using perfusion MRI in the management of cerebral gliomas., Materials and Methods: Between 1998 and 2001, 32 histologically proven glial tumors were assessed by perfusion MRI using echoplanar imaging (EPI) and gradient-echo techniques. Relative cerebral blood volume (rCBV) was measured in all patients and compared to histological data., Results: rCBV values were significantly correlated to histological grading in all 32 patients (P<0.001). Mean rCBV values were 8.74 (+/-3.79) for glioblastomas, 7.37 (+/-2.83) for anaplastic gliomas and 0.84 (+/-0.61) for low-grade gliomas. Mean rCBV values were significantly different between low- and high-grade gliomas, making it possible to determine a threshold (2.5-3) that can separate these two types of lesion. In determining the histological grading, rCBV was shown to be significantly more accurate than conventional MRI (P<0.005)., Conclusion: Perfusion MRI using the EPI technique reliably assesses tumoral neoangiogenesis in gliomas preoperatively. The specificity and sensitivity of this technique make this radiological modality a valuable tool in the assessment of cerebral gliomas.

Ependymomas are rare intracranial tumors observed in adults. Prognostic factors as well as proper therapeutic management remain controversial. We report a retrospective study of 121 cases intracranial ependymomas diagnosed between 1990 and 2004 in adult patients. Mean age was 46 years with a 1/1 sex-ratio. Supratentorial and infratentorial localization was noted for 41 (33.9%) and 80 (66.1%) patients respectively. Total gross resection was achieved for 62.8% of tumors. WHO staging was grade II for 72.7% and III for 27.3%. Recurrence developed in 41 (33.9%) patients. Median follow-up was 70 months. The 5-year and 10-year overall survivals were 85 and 76% respectively; the respective progression-free survivals were 64 and 43%. At univariate analysis, age, KPS, localization, extent of surgery and histological grade were correlated with overall survival. At multivariate analysis age, location, histological grade and extent of surgery contributed most to prediction of overall survival. Concerning progression-free survival, univariate analysis found age, KPS, localization, extent of surgery, complementary treatment and histological grade to be correlated with recurrence. Multivariate analysis retained extent of surgery, histological grade and complementary treatment as the most important predictors of progression-free survival. This study demonstrated that extent of surgery and tumor grade are the two main prognostic factors in adult intracranial ependymomas with respect to overall and progression-free survival. Furthermore, our data suggest that postoperative radiotherapy significantly increases progression-free survival in patients with incompletely resected grade II tumors.

Background: Intracranial ependymomas are rare in adults and histopathological prognostic factors are poorly determined., Purpose: A retrospective multicentric study was conducted in France in order to assess the prognostic value of histology., Material: Between 1990 and 2004, 216 adult patients with newly diagnosed ependymomas were treated in 19 French centers. Eligibility required institutional histopathological confirmation of an ependymoma and available clinical history and MRI features (see comparison paper)., Methods: Histological preparations and one paraffin embedded block from each patient were sent to Pr D. Figarella-Branger in Marseille. Central review by four neuropathologists (D. Figarella-Branger, A. Maues de Paula, C. Fernandez and A. Jouvet) was performed. Specimens for which all pathologists agreed with the histological diagnosis of ependymomas were included, whereas cases for which all disagree were excluded and reclassified. In the event of doubt and/or discrepancies between pathologists immunostaining was performed in order to reach a consensus diagnosis. Diagnostic of ependymomas was confirmed in 121 cases (56%). In theses cases, ependymomas were classified according to the WHO system (subtype and grade). The potential prognostic value (overall survival OS and disease free survival DFS) of the following histological parameters was examined: perivascular pseudorosettes, ependymal rosettes, hyalinized vessels, mitotic index, microvascular proliferation, necrosis, area of increased cellularity, nuclear atypia, brain invasion and Mib-1 labelling index., Results: Among the 121 ependymomas, 88 were grade II (47 classic, 17 cellular, 2 papillar, 6 clear cells and 16 tanicytic) and 33 grade III. WHO grading, occurrence of microvascular proliferation, necrosis, nuclear atypia and high proliferative index were correlated with both OS and DFS. Moreover, quantification of certain parameters enabled a reproducible grading system correlated with both OS and DFS.

The AP-HM tissue bank is the largest one in France regarding some collections, including brain tumors. This collection was used to better characterize some gliomas. In particular because some benign gliomas such as pilocytic astrocytomas (WHO grade I) can be misclassified as malignant ones such as glioblastomas (grade IV) the first aim of our study was to find accurate diagnostic markers. This was done mainly by suppressive substractive hybridization (SSH). This study also provides a restrictive list of genes selectively involved in angiogenesis and invasion, which were highly expressed in GBM. Results were confirmed by real time quantitative RT-PCR in a large cohort of patients. In addition in order to find accurate markers which can predict GBM overall survival (OS) we selected three cohorts of GBM patients with distinctive OS (short survival < 6 months, long survival > 18 months and intermediate). Quantification of a series of markers involved in angiogenesis and invasion was done as well as cDNA array analysis.

During these last 25 years, despite numerous phases III studies, standard of treatment in glioblastoma multiforme (GBM) consisted of surgery and post-operative radiotherapy, while benefit of chemotherapy was a matter of debate. A phase III study, conducted by EORTC and NCI Canada and involving 573 patients, concluded clearly to the benefit of adding temozolomide during and after radiotherapy as adjuvant treatment. Using this schedule, median survival increase from 12,1 to 14,6 months, and 2 year survival rate from 8 to 26%. In the same time, for anaplastic oligodendroglioma (AO) the phase III study conducted by Cairncross, that compared radiotherapy to a schedule that deliver a chemotherapy with PCV followed by radiotherapy, failed to determine a significant benefit on overall survival, despite the particular chemosensitivity of theses tumors. Moreover, these two studies did underlined impact of biological markers (methylation of the promoter of O6 methylguanine DNA transferase (MGMT) gene for GBM and chromosomes 1p and 19q deletion for AO), that may become decisional markers in the future. We review here the new place of chemotherapy in the adjuvant treatment of GBM and anaplastic gliomas, as well as the impact of these pivotal studies on second lines therapies, and future clinical research.

Glioblastoma multiforme is the most malignant of the primary brain tumors and is almost always fatal. The treatment strategies for this disease have not changed appreciably for many years and most are based on a limited understanding of the biology of the disease. Growth factors are potential targets for therapeutic strategies because they are essential for tumor growth and progression. Adrenomedullin (AM) is a multifunctional regulatory peptide with mitogenic and angiogenic capabilities among others. Real-time quantitative reverse transcriptase-polymerase chain reaction analysis showed that AM mRNA was correlated to the tumor type and grade, with high expression in all glioblastomas analysed, whereas a low expression was found in anaplastic astrocytomas and barely detectable levels in low-grade astrocytomas and oligodendriogliomas. The correlation of AM expression to the grade of glioma support the hypothesis that AM may participate in the progression of gliomas. We demonstrate that AM may function as an autocrine/paracrine growth factor for glioblastoma cells. The data demonstrated that the anti-AM antibody significantly suppress the growth of established glioblastoma xenografts. The action of AM is specific and is mediated by the calcitonin receptor-like receptor/receptor activity-modifying protein-2 and -3 (CRLR/RAMP2, CRLR/RAMP3). Furthermore, the proangiogenic action of AM on cultured endothelial cells via CRLR/RAMP2 and CRLR/RAMP3 receptors may translate in vivo into enhanced neovascularization and therefore identify AM and its receptors acting as potential new targets for antiangiogenic therapies.

Magnetic resonance spectroscopy (MRS), that may be added to conventional magnetic resonance imaging (MRI) exam exhibit an increasing role in the management of brain tumors. These technique allow quantitative analysis of metabolites, either cell specific, either reflecting physiological and/or pathological process. With a rigorous approach, MRS explore brain metabolism that may improve MRI data in clinical practice in neuro-oncology. Positive diagnosis of brain tumor, differential diagnosis between infiltrative glioma (grade II) and gliomatosis, determination of the limits of tumor infiltration, and distinction between tumor and post-therapeutic images are some of the potential applications of MRS. Ongoing and future studies may also precise the place of MRS in the differential diagnosis between high grade glioma, metastasis and CNS lymphoma, as well as in monitoring therapy in glioma.

Announcement of cancer diagnosis and first contact with medical team in charge of treatment is critical for the patient as well as for the quality of care. The French Cancer Plan include a special focus on these announcement which should be performed in adequate conditions and which should include the presentation of a personalised treatment plan, that reflect multidisciplinary discussion and decision performed before. In order to generalized a formal announcement consultation all over the country in 2005, an experimentation has been performed in 58 centers grouped in 37 projects. Neuro-Oncology Unit of the Assistance Publique-Hopitaux de Marseille did participate to that experimentation. Evaluation of these experiment focus on physician, nurse, and patient perception as well as cost impact and exploration of language during that particular time. Implication of a referent nurse in that time was a strong option of our center. We analyzed the result in 80 patients referred in our center for initial diagnosis (78%) or announcement of recurrence (14%) in a 5 months time. Implication of the nurse appear to be critical for patient confidence and accessibility to information for the all medical team. Organization of a particular time devoted to cancer announcement appear to have a favourable impact on quality of care and patient perception, and constitute a first step to a personalized management of patients with cancer.

Background: Management of unresectable progressive meningioma remains controversial and constitutes a major challenge since therapeutic options including chemotherapy and hormone modulation are limited. Recent data have suggested that hydroxyurea treatment may have an antitumoral effect. The purpose of this prospective phase II study was to evaluate the efficacy of hydroxyurea treatment for unresectable progressive meningioma., Methods: From 1997 to 1999, consecutive patients presenting unresectable meningioma with clinically and/or neuroradiologically documented progression were considered for entry into this protocol. Previous radiotherapy was not a mandatory inclusion criteria. Treatment consisted of continuous oral administration of hydroxyurea at a dose of 20 mg/kg per day. Follow-up assessment included physical examination, computed tomography (CT), and magnetic resonance imaging (MRI) performed every three months, as well as regular blood testing. The primary endpoint was documentation of objective response by MRI or CT., Results: The intent-to-treat population was 43 patients with at least 18 months follow-up. Median age was 60.4 years. Twenty-eight patients had undergone surgery following initial diagnosis. The meningioma was located in the skull base in 67% of patients. Histology was benign in 18 and atypical in 10. The eligible population included 36 patients with documented progressive disease at the time of inclusion; with progression documented clinically in 29 (67.5%) and/or radiologically in 20 (46%). In 7 patients, clinical or radiological progression could not be confirmed. The intent-to-treat analysis at median 26 months follow-up revealed objective response to hydroxyurea in only 3 patients (7%) including one on the basis of improvement in visual symptoms and two on MRI analysis. Progressive disease was observed clinically or radiologically in 26 patients (60.5%). Of the eligible population (n=36), 2 achieved an objective response and 13 (36%) exhibited stabilization under hydroxyurea therapy, while 21 (58%) progressed under treatment. Overall tolerance was good but anemia (grade I-II) and asthenia (grade I-II) were observed in 28% and 23.5% respectively. Treatment was discontinued in 3 patients because of chronic skin toxicity in one and anemia and asthenia in two., Conclusion: Hydroxyurea treatment is of marginal efficacy for meningioma and must not be considered as an alternative if radiotherapy or surgery is feasible. New efficient medical treatments are still required for progressive meningiomas.

Context: The "Standards, Options and Recommendations" (SOR) project, which started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French Regional Cancer Centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients., Objective: To define clinical practice guidelines for the management of adult patients with intracranial glioma in collaboration with the Association of French-speaking Neuro-oncologists (Anocef) and the French society of neurosurgeons (SNCLF). These recommendations cover diagnosis, classification, treatment and follow-up of patients with these tumors., Method: The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines has been defined, the document is submitted for review by independent reviewers., Results: This article is a summary version of the full document presenting the clinical practice guidelines with algorithms. The main recommendations concern the place of the surgery, radiotherapy, chemiotherapy, imagery and concomitant medical treatments in the specific treatment strategy of grade III and IV glioma, grade II glioma, gliomatosis cerebri, pilocytic astrocytoma, subependymoma, xanthoastrocytoma, intracranial ependymoma and brain stem glioma.