[Role of the nurse coordinator in geriatric oncology]. / Rôle de l'infirmière de coordination en oncogériatrie.

Cancers currently affect one third of people over the age of 75. According to the French Health Watch Institute, by 2050, 50% of cancers will affect this category of the population. The difficult of treating elderly people is that the initial symptoms are not always obvious: anorexia, asthenia, irregular bowel movements, etc. The nurse coordinator in geriatric oncology provides specific care to elderly people with cancer.

[Coordinating complex nursing care: building a guidance tool for cancer patients, to direct them towards the coordination nurse].

Introduction : following the 2009-2013 Cancer Plan, the experimental oncology nursing coordination (IDEC) showed a positive impact on the fluidity of care pathways. The 2014-2019 cancer plan guides their mission to complex cases. The objective of this study is to build a tool to facilitate the recruitment of patients likely to experience a complex path. Method : two phases have formed this research. The first one collected the elements of the dimensions that can predict the complexity of the care path, by focus group. The second consisted of reduction and selection of priority items and to estimate their importance by the Delphi method. Results : from the 12 selected items, two are recognized as a significant risk scoring, seven probably correlated with a complex pathway and three unrelated to the complexity of the pathways. Discussion : later this instrument would be validated by a test sample to evaluate its psychometric properties, metrological and feasibility.

Paracentric inversion of chromosome 2 associated with cryptic duplication of 2q14 and deletion of 2q37 in a patient with autism.

We describe a patient with autism and a paracentric inversion of chromosome 2q14.2q37.3, with a concurrent duplication of the proximal breakpoint at 2q14.1q14.2 and a deletion of the distal breakpoint at 2q37.3. The abnormality was derived from his mother with a balanced paracentric inversion. The inversion in the child appeared to be cytogenetically balanced but subtelomere FISH revealed a cryptic deletion at the 2q37.3 breakpoint. High-resolution single nucleotide polymorphism array confirmed the presence of a 3.5 Mb deletion that extended to the telomere, and showed a 4.2 Mb duplication at 2q14.1q14.2. FISH studies using a 2q14.2 probe showed that the duplicated segment was located at the telomeric end of chromosome 2q. This recombinant probably resulted from breakage of a dicentric chromosome. The child had autism, mental retardation, speech and language delay, hyperactivity, growth retardation with growth hormone deficiency, insulin-dependent diabetes, and mild facial dysmorphism. Most of these features have been previously described in individuals with simple terminal deletion of 2q37. Pure duplications of the proximal chromosome 2q are rare and no specific syndrome has been defined yet, so the contribution of the 2q14.1q14.2 duplication to the phenotype of the patient is unknown. These findings underscore the need to explore apparently balanced chromosomal rearrangements inherited from a phenotypically normal parent in subjects with autism and/or developmental delay. In addition, they provide further evidence indicating that chromosome 2q terminal deletions are among the most frequently reported cytogenetic abnormalities in individuals with autism.

Trends in response rate and survival in small-cell lung cancer patients between 1997 and 2017.

INTRODUCTION: Median survival of small-cell lung cancer (SCLC) patients is usually around 1 year. The advent of new drugs may have slightly improved their prognosis. We aimed to assess whether SCLC response to chemotherapy and survival had changed over time. METHODS: Consecutive SCLC patients were included at Grenoble University Hospital, France. We compared the patients' characteristics, response to chemotherapy and survival between 1997-2009 (period 1) and 2010-2017 (period 2). RESULTS: A total of 529 patients were identified, of whom 498 received a first line of chemotherapy and 279 a second line. The majority (n = 290, 58%) had extensive disease. The objective response rate (ORR) to first-line chemotherapy in metastatic patients was 63% in period 1 and 62% in period 2; the ORRs to second-line chemotherapy were 39% and 29%, respectively. Median overall survival from first-line chemotherapy was 13.2 months (interquartile range [IQR] 7.4-24.4) in period 1 and 11.2 months (IQR 7.1-21.2) in period 2. Mortality in these two periods did not differ significantly even after adjustment for prognostic factors (hazard ratio [HR] = 0.82, 95% confidence interval [CI] 0.66-1.00). The factors independently associated with death were cardiovascular comorbidities (HR = 1.28 [95%CI 1.05-1.55]), liver comorbidities (HR = 1.31 [95%CI 1.03-1.65]), poor ECOG performance status (3-4vs. 0-1, HR = 2.45 [95%CI 1.83-3.30]) and extensive disease (HR = 2.69 [95%CI 2.18-3.33]). CONCLUSIONS: Since 1997, there has been no improvement in the survival or response rate to chemotherapy of SCLC patients. There is a desperate need for new approaches in this setting.

Operation and Chemotherapy: Prognostic Factors for Lung Cancer With One Synchronous Metastasis.

BACKGROUND: Stage IV non-small cell lung cancer (NSCLC) is considered incurable; however, some patients with only few metastases may benefit from treatment with a curative intent. We aimed to identify the prognostic factors for stage IV NSCLC with synchronous solitary M1. METHODS: A database constructed from our weekly multidisciplinary thoracic oncology meetings was retrospectively screened from 1993 to 2012. Consecutive patients with NSCLC stages I to IV were included. RESULTS: Of the 6,760 patients found, 4,832 patients were studied. Among the 1,592 patients (33%) with stage IV NSCLC, 109 (7%) had a synchronous solitary M1. Metastasis involved the brain in 64% of patients. Median overall survival was significantly longer in synchronous solitary M1 than in other stage IV (18.9 months, interquartile range [IQR]: 9.9 to 34.6 months versus 6.1 months, IQR: 2.3 to 13.7 months], respectively, p < 10-4). Among patients with synchronous solitary M1, 90 (83%) received a local treatment with curative intent at the primary and metastatic sites. Factors independently associated with survival were age older than 63 years (hazard ratio [HR] 1.63, 95% confidence interval [CI]: 1.01 to 2.63), Performance status of 3 or 4 (HR 7.91, 95% CI: 2.23 to 28.03), use of chemotherapy (HR 0.38, 95% CI: 0.23 to 0.64), and operation conducted at both sites (HR 0.35, 95% CI: 0.19 to 0.65). CONCLUSIONS: Synchronous solitary M1 treated with chemotherapy and operation at both sites resulted in better survival. Survival of NSCLC with synchronous solitary M1 was more similar to stage III than other stage IV NSCLCs. The eighth TNM classification takes this into account by distinguishing between stages M1b and M1c.

Intrauterine growth and cerebral palsy in twins: a European multicenter study.

Population-based studies in twins have been of insufficient size to explore the relationship between risk of cerebral palsy and intrauterine growth. Earlier studies in singletons have suggested an optimum size at birth for minimum cerebral palsy risk between the 75th and 90th percentiles of weight for gestational age. We aggregated data from nine European cerebral palsy registers for 1976 to 1990. Using sex-specific fetal growth standards for twins, a z score of weight-for-gestation was derived for each of the 373 twin cases. The rates of cerebral palsy in each z-score band were compared to the rate in the a priori reference band of 0.67 to less than 1.28 (equivalent to the 75th to less than 90th percentiles). In twins born at 32 weeks' gestation or more (92% of all twins), cerebral palsy rates were higher for both light and heavy-for-gestation babies compared to an optimum (i.e., minimum risk) in the reference band. However, the rate ratio for heavy babies (90th percentile or greater) did not reach conventional (95% confidence intervals [CI]) statistical significance (rate ratios = 1.76; 90% CI 1.02-3.03). For twins born at less than 32 weeks, the significantly higher risk for cerebral palsy was observed consistently in all z-score bands less than average compared to the reference band. This multi-center study demonstrates that for twins born at 32 weeks' gestation or more, an increased risk of cerebral palsy is associated with deviations from optimal intrauterine growth at about 1 standard deviation above mean weight, as was earlier reported for singletons. For twins born at less than 32 weeks' gestation, this pattern is only demonstrable for babies weighing below the optimum weight-for-gestation.

SCPE work, standardization and definition--an overview of the activities of SCPE: a collaboration of European CP registers.

The main aim of the Surveillance of Cerebral Palsy in Europe (SCPE) network was to develop a central database of cerebral palsy (CP) cases across Europe. Monitoring trends in prevalence rates of CP should contribute to collaborative studies on risk factors or quality of life for children living with CP. A multi-centre collaboration of CP registries used a clear definition of CP to accurately and consistently identify cases of CP. The rate of CP within the collaboration varied from 1.5 to 3 per 1000 live births. For the birth cohort 1980 to 1996 (n=9128), 53.9% of the CP children had a bilateral spastic CP, 31.0% had unilateral spastic CP, 6.6% were dyskinetic and 4.1% ataxic. Among CP children, 20.4% had a birth weight less than 1500 g and 25.5% were born before 32 weeks gestational age. Intellectual impairment corresponding to an IQ<50 was found in 29.5% of CP children. The proportion of CP children unable to walk, even with aids, was 30.3%. Twelve and a half percent of CP children were known to have a severe visual impairment. It was concluded that registers are the best means to implement epidemiological research into CP.

Prognostic impact of paraneoplastic cushing's syndrome in small-cell lung cancer.

INTRODUCTION: Paraneoplastic Cushing's syndrome (CushingPS) in small-cell lung cancer is rare but severe. METHODS: We studied 383 patients with small-cell lung cancer diagnosed between 1998 and 2012. Among them, 23 patients had CushingPS, 56 had other paraneoplastic syndrome (OtherPS), and 304 had no paraneoplastic syndrome (NoPS). RESULTS: After comparison of the three groups, we observed that CushingPS patients had more extensive disease: 82.6% versus 67.8% versus 53.3% (p = 0.005), respectively, with more than two metastatic sites: 63.2% versus 15.8% and 24.1% (p ≤ 0.001), a higher World Health Organization performance status (2-4): 73.9% versus 57.1% versus 43.7% (p = 0.006), greater weight loss (≥10%): 47.8% versus 33.9% versus 16.4% (p ≤ 0.001), reduced objective response to first-line treatment: 47.6% versus 74.1% versus 71.1% (p = 0.04), and poorer sensitivity to first-line treatment: 19% versus 38.9% versus 48.6% (p = 0.01). NoPS patients, with World Health Organization performance status of 3-4, had extensive disease at diagnosis, with response, sensitivity to first-line treatment, and survival similar to the CushingPS group. At relapse, the CushingPS group had no objective response to second-line treatment versus 25% versus 42.8% in OtherPS and NoPS groups, respectively (p = 0.005). The median survival of CushingPS patients was 6.6 months versus 9.2 months for OtherPS and 13.1 months for NoPS patients (p ≤ 0.001). CushingPS is a prognostic factor of death (hazard ratio, 2.31; p ≤ 0.001). CONCLUSION: CushingPS is the worst form of the paraneoplastic syndromes with particularly extensive tumors. Reduced objective response and sensitivity to first-line treatment and no response to second-line treatment suggest starting palliative care early at first line and exclusively at relapse.

Outcomes in recurrent small-cell lung cancer after one to four chemotherapy lines: a retrospective study of 300 patients.

Standard treatment of small-cell lung cancer (SCLC) is a combination of etoposide and platinum for patients with extensive disease, associated with radiotherapy for patients with limited disease (LD). Therapeutic strategies for relapse, although well characterized, are disappointing. Between 1997 and 2009, 300 patients were treated for SCLC at Grenoble University Hospital. We analyzed patients' characteristics and outcomes at different treatment steps, to determine prognostic factors and propose subsequent treatment strategies according to "sensitive", "resistant" or "refractory" status established after first-line treatment (L1). The median patient age was 63.2 years, 46.3% had LD, and 23% were female. The objective response rate (ORR) to first-line chemotherapy was 73% [CI(95%): 67.6-77.9] and median survival was 13 months. After L1, comparison between "refractory" and "sensitive" groups showed more extensive disease (76.6% vs. 34.3%, p=0.003), poorer Performance Status (PS 0-1: 48.4% vs. 67.8%, p=0.008), more endocrine paraneoplastic syndrome (18.7% vs. 8.4%, p=0.03) and more composite histology (17.2% vs. 4.9%, p=0.004) in "refractory" patients. After second line (L2), ORR was 55.8% [CI(95%): 45.2-66.0] in "sensitive", 18.2% [CI(95%): 8.2-32.7] in "resistant", and 14.7% [CI(95%): 4.9-31.0] in "refractory" groups; with partial response only for the last two groups. After L3 and L4, ORR was 24.0% [CI(95%): 14.9-35.2] in "sensitive", 9.1% [CI(95%): 11.2-29.2] in "resistant" with partial response only. No response was observed for "refractory". After L1, the median survival was respectively 23, 10 and 6.4 months for "sensitive", "resistant" and "refractory" groups (p<0.001). Multivariate analysis showed that LD and classical SCLC histology were positive predictors of belonging to the "sensitive" group. Positive factors for survival were sensitivity to L1, PS 0-1, LD, Charlson score <4, no endocrine paraneoplastic syndrome and no occupational exposure. Limited disease is the major predictive factor for sensitivity to treatments and survival. Factors linked to the patients' clinical presentation also impact on survival. With currently recommended drugs, the "sensitivity" of the patient determined by the response to L1 indicates that it is pointless to treat "sensitive" with L4, "resistant" with L3 and "refractory" with L2, except for a few selected patients after multidisciplinary group discussion.

[Multidisciplinary team meetings in cancerology: setting priorities for improvement]. / Évaluation des réunions de concertation pluridisciplinaire en cancérologie : quelles priorités pour quelles améliorations ?

Resulting medical decision from a multidisciplinary team (MDT) meeting has to be accurate regarding to various patient criteria and relevant specialists participation. The target is to optimize treatment or management options for patients taking into account patients' benefit. The aim of our study was to examine quality criteria of MDT meeting processes, implementation of the MDT decision, and the follow-up of national or regional clinical guidelines. The results lead us to discuss about care management in cancer. Ten various medical specialities of MDT meetings were studied. Relevant multidisciplinarity varied between MDT meetings specialities and was effective between 55 and 100%. Implementation of the decisions that arise from MDT meetings was 86.3%. The most frequent grounds of non-application were patient refusal and new or previous unknown clinical data. The percentage of MDT meetings decisions following national or regional recommendations was 74%. The main reason of not following was the complexity of clinical patient circumstances. Participation in MDT meetings is more and more time-consuming related to enforce the completeness referred to the Plan Cancer (National recommendations). Leading to completeness raises questions about medical time employment and meaning of the MDT meeting for standard clinical cases. The priority seems to enforce multidisciplinarity rather than reach completeness.

Trends, perinatal characteristics, and medical conditions in pervasive developmental disorders.

Our aim was to study trends in the prevalence of pervasive developmental disorders (PDD) and to quantify their association with morphogenetic anomalies and with perinatal characteristics such as gestational age, birthweight, and hospitalization in a neonatal care unit. Data from a French morbidity register of childhood disabilities with the use of consistent definitions over time within the same geographical area were analyzed. The data of a total of 454 children (312 males, 142 females) with PDD, born between 1980 and 1993 and residing in Isère county, were recorded at the age of 7 years. The overall prevalence of PDD was 22.2 out of every 10000. There was a significant increase, from 14.7 to 30.8 out of every 10 000, during the period of study. Among these children with PDD, morphogenetic anomalies were observed in 12.1% (95% confidence interval [CI] 9.3-15.5), and the hospitalization rate during the neonatal period was 22% (95% CI 17.0-27.5), which is significantly higher than the observed rates in the general population. The increase in the prevalence of PDD, the association with perinatal risk factors, and the high rate of neonatal hospitalization require further studies to investigate the reasons for and mechanisms of these developmental disorders.

Cerebral palsy of post-neonatal origin: characteristics and risk factors.

We aimed to study the rates and trends over time of children with cerebral palsy (CP) of post-neonatal origin (arising more than 28 days after birth, and before the age of 25 months), to examine their aetiology and associated significant risk factors, and to compare them with other CP cases. Children with post-neonatal CP born 1976-90 were identified from a European database and seven registers were included (Surveillance of Cerebral Palsy in Europe collaboration). Using a previously published classification it was possible to allocate an aetiology to 99% of cases. The prevalence rate of post-neonatal CP was 1.26 per 10 000 live births and a significant decrease was observed over the period 1976-90 (P = 0.011). Infection accounted for 50%, vascular episodes for 20% and head injury for 18% of the cases. Although there has been little change in the profile of underlying causes in this period, within the infection group, a significant downward trend was observed for Reye's syndrome (P < 0.001) and non-central nervous system (non-CNS) infection (P = 0.004), but not for meningitis/encephalitis. There was evidence of some increased risk of post-neonatal CP among children with low birthweight (<2500 g) (P < 0.001). Overall children with CP of post-neonatal origin showed a more severe functional pattern than non-post-neonatal CP children. In order to ascertain the impact of public health and other preventive measures aimed at reducing the frequency of brain injury in the first 2 years after birth, it is necessary to continue to monitor the frequency and characteristics of children with post-neonatal CP into the 1990s.