[Management of non-small cell lung cancer patients harboring activating mutations and CNS progression]. / Caractéristiques et prise en charge de l'évolutivité cérébro-méningée des cancers bronchiques dans un contexte de mutation activatrice.

The central nervous system (CNS), through carcinomatous meningitis or solid brain metastases, is the most common site of recurrence in non-small cell lung cancers (NSCLC) with activating mutations. Our retrospective study describes the population of patients with CNS metastases of NSCLC harboring activating mutation with targeted therapy (EGFR, ALK, BRAF, HER2) in 4 French regional reference hospitals. 60 patients were analyzed. The proposed treatments were heterogeneous and included combinations of chemotherapy, targeted therapy and radiotherapy±associated with topical treatments. Median overall survival following CNS metastasis in these patients was 15.8 months for meningitis carcinoma and 26 months for brain metastases. In patients with brain metastases, the addition of targeted therapy treatment allows a significant improvement in median progression free survival from 5.9 months to 10.6 months (HR 0.48 CI95 [0.24 to 0.97] P=0.035). These patients seem therefore benefit from systemic therapy and particularly targeted therapy with better survival than usual.

[Cohort of patients initiated to home ventilation. Observational and prospective study]. / Cohorte de patients nouvellement traités par ventilation non invasive à domicile. Étude observationnelle, prospective et multicentrique.

Cohort of patients initiated to home ventilation. Observational and prospective study. The effectiveness of home noninvasive ventilation (NIV) for chronic respiratory failure (CRF) is well established. However, few data are available about home NIV prescription and utilization according to the different etiologies of respiratory failure. The ANTADIR Federation, in partnership with the Ventilatory Support Group of the French Speaking Pulmonary Society, has set up a national, observational and multicenter cohort study. The main goal of this study is to analyze the clinical data justifying home NIV prescription in patients with chronic respiratory insufficiency. The secondary objectives will be to assess: the evolution of comorbidities or their occurrence, hospitalizations, NIV compliance, dropout and survival. The population includes patients with chronic respiratory failure newly initiated onto NIV, both in a stable state and following an acute exacerbation who qualify for long-term NIV. Data collected include: diagnosis and comorbidities, age, sex, BMI, biomarkers (hematocrit, arterial blood gases, total CO2) and functional data (FEV1, VC, TLC), nocturnal results (SaO2, PtcCO2), type of ventilator used, ventilator parameters and mask type. Follow-up data will be collected at 4 months, 1 year and 2 years and will include: hospitalizations, changes in prescription, adherence, dropouts and deaths. This work will make it possible to obtain new scientific information on long-term NIV use in France.

Lung function parameters in omalizumab responder patients: An interesting tool?

BACKGROUND: Omalizumab, an anti-IgE antibody, is used to treat patients with severe allergic asthma. The evolution of lung function parameters over time and the difference between omalizumab responder and nonresponder patients remain inconclusive. The objective of this real-life study was to compare the changes in forced expiratory volume in 1 second (FEV1) of omalizumab responders and nonresponders at 6 months. METHODS: A multicenter analysis was performed in 10 secondary and tertiary institutions. Lung function parameters (forced vital capacity (FVC), pre- and postbronchodilator FEV1, residual volume (RV), and total lung capacity (TLC) were determined at baseline and at 6 months. Omalizumab response was assessed at the 6-month visit. In the omalizumab responder patients, lung function parameters were also obtained at 12, 18, and 24 months. RESULTS: Mean prebronchodilator FEV1 showed improvement in responders at 6 months, while a decrease was observed in nonresponders (+0.2±0.4 L and -0.1±0.4 L, respectively, P<.01). After an improvement at 6 months, pre- and postbronchodilator FEV1 remained stable at 12, 18, and 24 months. The FEV1/FVC remained unchanged over time, but the proportion of patients with an FEV1/FVC ratio <0.7 decreased at 6, 12, 18, and 24 months (55.2%, 54.0%, 54.0%, and 44.8%, respectively, P<.05). Mean RV values decreased at 6 months but increased at 12 months and 24 months (P<.05). Residual volume/total lung capacity (RV/TLC) ratio decreased at 6 months and remained unchanged at 24 months. CONCLUSION: After omalizumab initiation, FEV1 improved at 6 months in responder patients and then remained stable for 2 years. RV and RV/TLC improved at 6 months.

[Hypoglycaemic coma revealing a malignant recurrence during Doege-Potter syndrome]. / Coma hypoglycémique révélant une récidive maligne lors du syndrome de Doege-Potter.

INTRODUCTION: The Doege-Potter syndrome is a rare paraneoplastic syndrome, first described by Doege and Potter in 1930, that usually involves solid fibrous pleural tumours. Hypoglycaemia is a rare complication of these tumours, due to secretion of insulin-like growth factors, and it can be the presenting symptom. The incidence of malignancy is low, about 12-15 %. CASE REPORT: An 81-year-old Caucasian man, operated on 20 years previously for a benign pleural fibroma, presented with several episodes of loss of consciousness due to profound hypoglycaemia and a history of increasing dyspnea. The chest X-ray revealed an intrathoracic mass. Following excision histological examination confirmed recurrence with malignant transformation to a high-grade sarcoma. CONCLUSION: The Doege-Potter syndrome is a rare condition but it should be suspected in episodes of hypoglycaemia associated with a history of dyspnea. These patients should be followed up postoperatively because of the risk of tumour recurrence.

[Assessment of recent weight history before instituting continuous positive airways pressure in a cohort of patients with severe obstructive sleep apnoea/hypopnea syndrome]. / Histoire pondérale récente, avant l'institution de la pression positive continue, dans une cohorte de patients ayant un syndrome d'apnées-hypopnées obstructives du sommeil sévère.

OBJECTIVES: The aim of this study is to evaluate the recent weight history in patients with the obstructive sleep apnoea/hypopnoea syndrome (OSAHS) needing treatment with continuous positive airways pressure (CPAP). PATIENTS AND METHODS: Over a period of 18 months, a self-administered questionnaire concerning recent weight history was given to patients during the installation of CPAP. RESULTS: The study population consisted of 1337 patients. An increase in weight of at least 5% over the 5 years preceding the institution of CPAP was found in 44% of patients. This increase was moderate (5-10%), important (10-20%) and very important (>20%) in respectively 12.3%, 16.1% and 15.6% of patients. The patients with important or very important weight gain differed from those whose weight remained steady in a higher percentage of women, young people, and patients with a higher prevalence of obesity, persistent smoking, a history of venous thromboembolism and treatment with anti-depressants. CONCLUSION: A recent increase in weight is frequently seen in severe OSAHS and is associated with some clinical characteristics.

Therapy response evaluation with FDG-PET/CT in small cell lung cancer: a prognostic and comparison study of the PERCIST and EORTC criteria.

INTRODUCTION: Small cell lung cancer (SCLC) is an aggressive form of lung cancer with poor prognosis. Adequate staging and therapeutic evaluation is necessary for therapy planning. Fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) has been shown to be useful for staging and therapy response evaluation. The European Organization for Research and Treatment of Cancer (EORTC) and Positron Emission Tomography Response Criteria In Solid Tumors (PERCIST) criteria were compared in the evaluation of response assessment and prognostic factors were defined in a cohort of SCLC patients. METHODS: Twenty-nine consecutive patients with SCLC were included in this study. Sixteen patients had extensive disease and 13 had limited disease. All patients had chemotherapy, 21 had thoracic radiotherapy. FDG-PET/CT scans were performed before and after therapy to evaluate treatment response. Metabolic responses were assessed using the EORTC criteria and PERCIST criteria. Univariate and multivariate analysis were performed using a Cox model to investigate the association between progression-free and overall survival time with a number of covariates. RESULTS: There was perfect concordance between the EORTC and PERCIST criteria. Eight patients had a complete metabolic response (CMR), 9 had a partial metabolic response (PMR), 5 had stable metabolic disease (SMD) and 7 had progressive metabolic disease (PMD). Overall survival time in patients with CMR was significantly longer compared with patients who did not have CMR. The initial or delayed CMR and post-therapeutic standardized uptake value corrected for lean body mass were significantly associated with overall survival. CONCLUSION: CMR on post-therapeutic FDG-PET/CT in patients with SCLC is an important prognostic factor and may help decision making for therapeutic management.

Bortezomib-induced bronchiolitis obliterans organizing pneumonia.

Introduction. Bortezomib is a proteasome inhibitor indicated for the treatment of multiple myeloma patients. The most frequent side effects are gastrointestinal and neurological. Serious pulmonary complications have been described rarely. Observation. This case involves a 74-year-old man suffering from IgG Kappa myeloma treated with bortezomib, melphalan, and dexamethasone. After administering chemotherapy, the patient developed an acute respiratory distress syndrome (ARDS). A surgical pulmonary biopsy proved the existence of bronchiolitis obliterans organizing pneumonia (BOOP) lesions. Systemic corticotherapy led to a rapid improvement in the patient's condition. Conclusion. This is the first reported histologically confirmed case of bortezomid-induced BOOP. Faced with severe respiratory symptoms in the absence of other etiologies, complications due to bortezomid treatment should be evoked and corticotherapy considered.

[The Lady Windermere syndrome: clinical and bacteriological data and progress in seven cases]. / Le syndrome de Lady Windermere : données cliniques, bactériologiques et évolutives.

Described by Reich and Johnson in 1992 [2], the Lady Windermere syndrome occurs exclusively in non-smoking women over the age of 60 years, without significant pre-existing pulmonary disease. It comprises bronchial dilatation, typically in the middle lobe and lingula, together with secondary infection by atypical mycobacteria (Mycobacterium avium in the first cases). Among the 17 cases of atypical mycobacterial infection that we have seen in the past 14 years, there were seven cases of this syndrome. It was associated with cough, sputum, sometimes haemoptysis, febrile episodes and deterioration of general health. The diagnostic criteria and treatment were defined by the American Thoracic Society. The pathophysiological hypothesis proposed by Reich and Johnson was that voluntary suppression of the cough led to congestion of the bronchi and secondary infection with atypical mycobacteria. Currently it is thought more likely that the following factors are involved: progressive increase in dilatation of small bronchi, delayed diagnosis, morphological abnormalities of the thorax, hormonal factors, immune deficiency, genetic neutrophil dysfunction, and even heterozygous forms of cystic fibrosis.

[Oxygen therapy by a portable concentrator with a demand valve: a randomised controlled study of its effectiveness in patients with COPD]. / L'oxygénothérapie par concentrateur portable à valve à la demande : étude randomisée et contrôlée de son efficacité clinique chez des patients BPCO.

INTRODUCTION: There is doubt concerning the clinical effectiveness of portable oxygen concentrators with a control valve (PCDV) and their appreciation by patients. Objectives. To compare the effectiveness and appreciation of oxygen therapy by PCDV and liquid oxygen by continuous f low (O(2)Liq). METHODS: Nineteen patients with COPD were randomised to receive PCDV or O(2)Liq at rest and during a 6 minute walk test (6MWT). For each mechanism they assessed, by visual analogue scales, the convenience and portability, the noise, and the discomfort of the nasal oxygen delivery. RESULTS: The 6MW distance was 315 ± 120 m with PCDV and 325 ± 114 m with O(2)Liq (P>0.05). Dyspnoea and the desaturation induced by the 6MWT were identical with both systems (P>0.05). The time spent with a SaO(2)<90 % was 289 ± 69 s with PCDV and 242 ± 130 s with O(2)Liq (P=0.08). PCDV was noisier than O(2)Liq (P<0.05); there was no difference in convenience and portability or in nasal discomfort. CONCLUSION: The PCDV model that we tested was equally effective to O(2)Liq. However, the prescription of this type of system is a matter of personal choice.

Randomized follow-up study of resected NSCLC patients: conventional versus 18F-DG coincidence imaging.

The aim of this study was to compare the utility of gamma camera using a coincidence detection system imaging (CDET) with 18-fluorodeoxyglucose to conventional imaging techniques in the detection of recurrence of non-small cell lung cancer. Sixty-nine patients were randomized into two groups for follow-up after surgery from October 2000 to December 2002. Each patient was evaluated every 6 months by conventional technique imaging in group A (n=33) or CDET imaging in group B (n=36) over two years. The direct costs of each procedure were evaluated. The major endpoint was the number of recurrences or new tumours detected. The two groups were similar. A total of 25 recurrences was detected (9 in group A and 16 in group B). Overall survival was similar in the two groups. CDET imaging was more expensive. CDET imaging provides earlier detection of recurrence, but does not modify survival outcome. Further studies are necessary to demonstrate the impact, if any, of 18-FDG imaging.

[Social and housing conditions of patients with chronic respiratory failure treated in the ANTADIR Federation]. / La vie quotidienne et sociale des patients insuffisants respiratoires chroniques sévères pris en charge dans le réseau ANTADIR.

INTRODUCTION: Chronic respiratory failure (CRF) has an impact on quality of life because of respiratory handicap. METHODS: The purpose of this study was to analyse the daily life and social activities of patients with CRF. A questionnaire was addressed to over 9000 patients being cared for in the ANTADIR homecare network, with over a 60% response rate. RESULTS: The data showed that patients were old, and frequently had comorbidity. The predominant diagnosis was chronic obstructive airways disease (COPD) and the number of women in this population continues to increase. Respiratory handicap led to a loss of autonomy, a reduction in social activity and mobility of patients, but the impact differed markedly according to the cause of the respiratory failure, as well as age and social class. A typological study of behavioural characteristics revealed three groups of patients with contrasting profiles. CONCLUSION: These results lead to recommendation for better management of social aspects of patient care in the ANTADIR network.

[Pleural metastases of sclerosing epithelioid fibrosarcoma]. / Métastases pleurales d'un fibrosarcome épithélioïde sclérosant.

Sclerosing epithelioid fibrosarcoma is a rare tumor recently described. The histological presentation can be confused with certain soft tissue benign tumors and certain sarcomas. Metastatic spread is usually late in the natural course of the disease. We report a case of recurrent sclerosing epithelioid fibrosarcoma with pleural metastases which developed ten years after surgical resection of the primary tumor. The tumor was formed by small uniform round epithelioid cells with a clear cytoplasm. The tumor cells were strongly positive for vimentin. This clinical case is discussed in light of other cases reported in the literature.

[Economic analysis of home based versus hospital outpatient chemotherapy in stage IV non small cell lung cancer]. / Analyse économique de l'administration d'une chimiothérapie en hospitalisation à domicile (HAD) comparée à l'hospitalisation de jour dans les cancers bronchopulmonaires non à petites cellules de stade IV.

BACKGROUND: French law and government decisions have induced new development for home base treatments. The objective was to compare cost of home base chemotherapy (HBC) versus outpatient infusions, for non small cell lung cancer (NSCLC). METHODS: 10 patients were selected in each category. D8 of their cycles was performed at home compared to outpatient infusion. Costs were based on national fees with cytostatic drugs as supplement. The real cost was also assessed through a specific questionnaire. RESULTS: 30 D8 infusions were carried out at hospital and 24 D8 infusions at home. Average cost by cycle was 2,829.51 euros [2 560.74-3 147.02] for hospital infusion, 2,372.50 euros [1 962.75-2 792.88] for HBC (-16.15%). Difference was -457.01 euros by cycle [-919.74; 26.82]. Real costs by injection for BHC was 484.42 euros [424.18; 540.32] versus a fee of 699.89 euros [643.64; 750.23]. There were no difference in terms of adverse events. CONCLUSION: HBC for NSCLC is feasible. Average costs by cycle is lower of 16% versus hospital infusion. The results of this non randomized study had to be confirm by further clinical trials.

[Investigation of allergic reactions to platinum salts]. / Exploration d'une allergie aux sels de platine.

INTRODUCTION: We report two cases occurring in 2004 of patients being treated for pleural mesothelioma with a combination of cisplatin or carboplatin and pemetrexed. Investigation by skin tests and flow cytometry confirmed the clinical diagnosis in both cases. CASE REPORTS: Case 1: a man of 62 developed, after 12 courses of cisplatin-pemetrexed, an anaphylactic reaction 5 minutes after the infusion of cisplatin. Treatment was withdrawn permanently. Case 2: a man of 66 developed, after 7 courses of cisplatin-pemetrexed, an anaphylactic reaction within the first minute of the infusion of cisplatin. Subsequently, in March 2004, he received pemetrexed alone without any problems. In August 2004 he was prescribed carboplatin-pemetrexed. Within 5 minutes he developed urticaria, pruritus and abdominal pain. He was treated later with pemetrexed alone with no problems. CONCLUSION: Hypersensitivity to platinum salts usually occurs after several courses of treatment. Skin tests and flow cytometry are a simple, concordant, and reliable way of confirming the diagnosis.

[Diagnosis of lung cancer. Role of PET/CT fusion scan in lung cancer]. / Diagnostic des CBP. L'apport de la fusion TEP-TDM dans le diagnostic des cancers bronchiques.

Whole-body positron emission tomography (PET) with radiolabeled [18F]-fluoro-2-deoxy-D-glucose (18FDG) plays an important role in the diagnosis, staging, and management of lung cancer. The preferential accumulation of FDG in malignant cells assists in the differentiation of benign and malignant tissue. However, PET alone does not allow an accurate anatomic localisation of FDG uptake. The combination of PET with CT images improves the spatial resolution, sensitivity and specificity of the test. PET/CT, as with PET alone, is indicated for the diagnosis of pulmonary nodules as well as the locoregional and extrathoracic staging of non-small-cell lung cancer (NSCLC). In many published studies, PET/CT has been shown to be superior to CT alone, to PET alone, or to both imaging techniques used separately to evaluate pulmonary nodules or for staging NSCLC. Now, PET/CT imaging is being investigated in the staging and management of small-cell lung cancer, in radiation treatment planning, in response prediction following treatment and in the detection of lung cancer recurrence in NSCLC. PET/CT is likely to have an important role in the management of lung cancer. New machines are in development and new radiopharmaceutical agents must be assessed.

Assessment of 18F-fluorodeoxyglucose dual-head gamma camera in asbestos lung diseases.

The purpose of this study was to evaluate the performance of 18F-fluorodeoxyglucose (18FDG) imaging via coincidence detection emission tomography (CDET) in identifying malignant lesions in subjects exposed to asbestos. A total of 30 patients exposed to asbestos underwent 18FDG-CDET between January 2000 and June 2003. A CDET scan of the thorax and abdomen was performed 60 min after injection of 18FDG in fasting patients, and results were obtained in slices in three axes. The CDET results were compared to those from computed tomography (CT), and pleural or surgical biopsy in patients with positive 18FDG-CDET results. All primary malignant mesotheliomas accumulated 18FDG (n=6), and, in two patients, CDET findings were superior to those of CT, allowing early detection. In two cases, lung carcinomas with malignant pleural effusion were also detected. There were five false positive CDET results: three unilateral pleural thickening, one rounded atelectasis, and one benign lung nodule. All patients with pleural plaques showed no significant 18FDG uptake. Malignant diseases were detected by 18FDG-CDET imaging with a sensitivity of 89% and specificity of 71%. Coincidence detection emission tomography can identify malignant mesothelioma in selected subjects exposed to asbestos. Coincidence detection emission tomography appears to be a useful noninvasive method for the follow-up of subjects with exposure risk of asbestosis.

[Review of nutritional and respiratory support in ALS]. / Prise en charge nutritionnelle et respiratoire des patients atteints de Sclérose Latérale Amyotrophique (SLA).

During the evolution of amyotrophic lateral sclerosis (ALS), quality of life and survival of patients are related to respiratory and nutritional status. After diagnosis, a multidisciplinary care has to be promptly organized and coordinated by the referring neurologist. The nutritional and respiratory support imply that neurologists know their specific means of evaluation with their sensitivity and sensibility and be able to recognize clinical risk situations. The informations of patients on assisted-ventilation and nutritional support by using gastrostomy may be done early, precisely and trustfully. Well informed patient's choices must be respected. Nutritional and respiratory supports may be based on recommendations established by the American Academy of Neurology. This review will present and discuss their main aspects in patients with ALS.