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PURPOSE: The aim of this study was to evaluate interobserver agreement (IOA) on target volume definition for pancreatic cancer (PACA) within the Radiosurgery and Stereotactic Radiotherapy Working Group of the German Society of Radiation Oncology (DEGRO) and to identify the influence of imaging modalities on the definition of the target volumes. METHODS: Two cases of locally advanced PACA and one local recurrence were selected from a large SBRT database. Delineation was based on either a planning 4D CT with or without (w/wo) IV contrast, w/wo PET/CT, and w/wo diagnostic MRI. Novel compared to other studies, a combination of four metrics was used to integrate several aspects of target volume segmentation: the Dice coefficient (DSC), the Hausdorff distance (HD), the probabilistic distance (PBD), and the volumetric similarity (VS). RESULTS: For all three GTVs, the median DSC was 0.75 (range 0.17-0.95), the median HD 15 (range 3.22-67.11) mm, the median PBD 0.33 (range 0.06-4.86), and the median VS was 0.88 (range 0.31-1). For ITVs and PTVs the results were similar. When comparing the imaging modalities for delineation, the best agreement for the GTV was achieved using PET/CT, and for the ITV and PTV using 4D PET/CT, in treatment position with abdominal compression. CONCLUSION: Overall, there was good GTV agreement (DSC). Combined metrics appeared to allow a more valid detection of interobserver variation. For SBRT, either 4D PET/CT or 3D PET/CT in treatment position with abdominal compression leads to better agreement and should be considered as a very useful imaging modality for the definition of treatment volumes in pancreatic SBRT. Contouring does not appear to be the weakest link in the treatment planning chain of SBRT for PACA.
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Adenocarcinoma , Neoplasias Pulmonares , Neoplasias Pancreáticas , Radiocirugia , Humanos , Radiocirugia/métodos , Adenocarcinoma/diagnóstico por imagen , Adenocarcinoma/radioterapia , Adenocarcinoma/cirugía , Tomografía Computarizada por Tomografía de Emisión de Positrones , Variaciones Dependientes del Observador , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/radioterapia , Neoplasias Pancreáticas/cirugía , Planificación de la Radioterapia Asistida por Computador/métodos , Neoplasias Pulmonares/radioterapia , Neoplasias PancreáticasRESUMEN
BACKGROUND: Although Hizentra is indicated for immunoglobulin replacement therapy in patients with primary and secondary immunodeficiencies, phase III trials have focused on patients with primary immunodeficiencies. In this 9-month, real-life, prospective, non-interventional, longitudinal, multicenter study of patients with primary and secondary immunodeficiencies in France, treatment modalities (primary endpoint), efficacy, safety, tolerability, quality of life, and treatment satisfaction were evaluated using descriptive statistics. RESULTS: Starting in January 2012, 117 patients were enrolled (99 adults, 18 children). Secondary immunodeficiencies were present in 48.7 % of patients. At follow-up, injections were administered every 7 days in 92.2 % of patients. Nine patients (7.8 %) were taking Hizentra every 10-14 days. The median dose of Hizentra administered was 0.1 g/kg/injection. Fifty-six patients were administered doses <0.1 g/kg/injection and 13 patients were administered doses >0.2 g/kg/injection. Mean trough IgG titers were 9.0 ± 3.3 g/L (median 8.3 g/L). The mean yearly rate of infection was 1.2 ± 1.9. Mean scores on the Short Form-36 physical and mental component summaries were 46.3 ± 10.0 and 46.6 ± 9.3, respectively. Scores on the Treatment Satisfaction Questionnaire for Medication ranged from 69.9 ± 19.9 to 88.3 ± 21.2 depending on the domain. Treatment with Hizentra was well tolerated. No single drug-related systemic reaction occurred in more than one patient and few local reactions were reported (n = 5). CONCLUSIONS: Under real-life conditions and in a cohort that included patients with primary and secondary immunodeficiencies, treatment with Hizentra was effective and well tolerated and patients were generally satisfied with the treatment.
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INTRODUCTION: According to the guidelines, the mammograms obtained in a screening program must be evaluated to ensure that the quality of the images obtained is above 75% of score 1 (perfect and good) and less than 3% of score 3 (inadequate). This is performed by a person (usually a radiographer), so subjectivity may influence the final evaluation of the images. The aim of this study was to evaluate the impact of subjectivity on breast positioning assessments on resultant screening mammograms. MATERIALS AND METHODS: Five radiographers evaluated a total of 1000 mammograms. One radiographer was an expert in assessing mammography images, while the other four evaluators had varying levels of experience. All images were anonymized, and the ViewDEX software was used for visual grading analysis. The evaluators were divided into two groups, each with two evaluators. Each group evaluated 600 images, with 200 images identical between the two groups. All images had already been evaluated by the expert radiographer. All scores were compared using the Fleiss' and Cohen's kappa coefficient and accuracy score. RESULTS: The results from Fleiss' kappa showed fair agreement in the mediolateral oblique (MLO) projection in the first group of evaluators whereas the other results showed poor agreement. When comparing the results from Cohen's kappa a maximum value of agreement between the evaluators was moderate 0.433 [95% CI 0.264-0.587] for the craniocaudal (CC) projection and 0.374 [95% CI 0.212-0.538] for the MLO projection. CONCLUSIONS: Based on our results, we can conclude that the agreement between all five raters was poor for both CC (κ = 0.165) and MLO (κ = 0.135) projections, based on the results of Fleiss' kappa statistic. The results show that the influence of subjectivity has a great impact on the evaluation of the quality of mammography images. IMPLICATIONS FOR PRACTICE: Thus, the images are evaluated by a person, which has a high impact on subjectivity in the assessment of positioning in mammography. To achieve a more objective assessment of the images and the resulting agreement between the evaluators, we would propose to change the method of assessment. The images could be evaluated by two persons, and in the event of a discrepancy, the images would be evaluated by a third person. A computer programme could also be developed that would allow a more objective evaluation based on the geometric characteristics of the image (angle and length of the pectoral muscle, symmetry, etc.).
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Mamografía , Programas Informáticos , Humanos , Mamografía/métodos , Detección Precoz del CáncerRESUMEN
Purpose: This study evaluated pretreatment 2[18F]fluoro-2-deoxy-D-glucose (FDG)-PET/CT-based radiomic signatures for prediction of hyperprogression in metastatic melanoma patients treated with immune checkpoint inhibition (ICI). Material and method: Fifty-six consecutive metastatic melanoma patients treated with ICI and available imaging were included in the study and 330 metastatic lesions were individually, fully segmented on pre-treatment CT and FDG-PET imaging. Lesion hyperprogression (HPL) was defined as lesion progression according to RECIST 1.1 and doubling of tumor growth rate. Patient hyperprogression (PD-HPD) was defined as progressive disease (PD) according to RECIST 1.1 and presence of at least one HPL. Patient survival was evaluated with Kaplan-Meier curves. Mortality risk of PD-HPD status was assessed by estimation of hazard ratio (HR). Furthermore, we assessed with Fisher test and Mann-Whitney U test if demographic or treatment parameters were different between PD-HPD and the remaining patients. Pre-treatment PET/CT-based radiomic signatures were used to build models predicting HPL at three months after start of treatment. The models were internally validated with nested cross-validation. The performance metric was the area under receiver operating characteristic curve (AUC). Results: PD-HPD patients constituted 57.1% of all PD patients. PD-HPD was negatively related to patient overall survival with HR=8.52 (95%CI 3.47-20.94). Sixty-nine lesions (20.9%) were identified as progressing at 3 months. Twenty-nine of these lesions were classified as hyperprogressive, thereby showing a HPL rate of 8.8%. CT-based, PET-based, and PET/CT-based models predicting HPL at three months after the start of treatment achieved testing AUC of 0.703 +/- 0.054, 0.516 +/- 0.061, and 0.704 +/- 0.070, respectively. The best performing models relied mostly on CT-based histogram features. Conclusions: FDG-PET/CT-based radiomic signatures yield potential for pretreatment prediction of lesion hyperprogression, which may contribute to reducing the risk of delayed treatment adaptation in metastatic melanoma patients treated with ICI.
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BACKGROUND: Pain symptoms in the upper abdomen and back are prevalent in 80% of patients with metastatic pancreatic ductal adenocarcinoma (mPDAC), where the current standard treatment is a systemic therapy consisting of at least doublet-chemotherapy for fit patients. Palliative low-dose radiotherapy is a well-established local treatment option but there is some evidence for a better and longer pain response after a dose-intensified radiotherapy of the primary pancreatic cancer (pPCa). Stereotactic body radiation therapy (SBRT) can deliver high radiation doses in few fractions, therefore reducing chemotherapy-free intervals. However, prospective data on pain control after SBRT of pPCa is very limited. Therefore, we aim to investigate the impact of SBRT on pain control in patients with mPDAC in a prospective trial. METHODS: This is a prospective, double-arm, randomized controlled, international multicenter study testing the added benefit of MR-guided adaptive SBRT of the pPca embedded between standard of care-chemotherapy (SoC-CT) cycles for pain control and prevention of pain in patients with mPDAC. 92 patients with histologically proven mPDAC and at least stable disease after initial 8 weeks of SoC-CT will be eligible for the trial and 1:1 randomized in 3 centers in Germany and Switzerland to either experimental arm A, receiving MR-guided SBRT of the pPCa with 5 × 6.6 Gy at 80% isodose with continuation of SoC-CT thereafter, or control arm B, continuing SoC-CT without SBRT. Daily MR-guided plan adaptation intents to achieve good target coverage, while simultaneously minimizing dose to organs at risk. Patients will be followed up for minimum 6 and maximum of 18 months. The primary endpoint of the study is the "mean cumulative pain index" rated every 4 weeks until death or end of study using numeric rating scale. DISCUSSION: An adequate long-term control of pain symptoms in patients with mPDAC is an unmet clinical need. Despite improvements in systemic treatment, local complications due to pPCa remain a clinical challenge. We hypothesize that patients with mPDAC will benefit from a local treatment of the pPCa by MR-guided SBRT in terms of a durable pain control with a simultaneously favorable safe toxicity profile translating into an improvement of quality-of-life. TRIAL REGISTRATION: German Registry for Clinical Trials (DRKS): DRKS00025801. Meanwhile the study is also registered at ClinicalTrials.gov with the Identifier: NCT05114213.
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Adenocarcinoma/radioterapia , Dolor en Cáncer/radioterapia , Imagen por Resonancia Magnética , Neoplasias Pancreáticas/radioterapia , Radiocirugia/métodos , Radioterapia Guiada por Imagen , Adenocarcinoma/secundario , Humanos , Neoplasias Pancreáticas/patología , Estudios ProspectivosRESUMEN
BACKGROUND: Carcinomatous meningitis (CM) is a severe complication of breast cancer. The Breast International Group (BIG) carried out a survey to describe the approach to CM internationally. PATIENTS AND METHODS: A questionnaire on the management of CM was developed by the Brain Metastases Task Force of BIG and distributed to its groups, requesting one answer per group site. RESULTS: A total of 241 sites responded, 119 from Europe, 9 from North America, 39 from Central/South America, 58 from Asia, and 16 in Australia/New Zealand, with 24.5% being general hospitals with oncology units, 44.4% university hospitals, 22.4% oncology centers, and 8.7% private hospitals. About 56.0% of sites reported seeing <5 cases annually with 60.6% reporting no increase in the number of cases of CM recently. Nearly 63.1% of sites investigate for CM when a patient has symptoms or radiological evidence, while 33.2% investigate only for symptoms. For diagnosis, 71.8% of sites required a positive cerebrospinal fluid cytology, while magnetic resonance imaging findings were sufficient in 23.7% of sites. Roughly 97.1% of sites treat CM and 51.9% also refer patients to palliative care. Intrathecal therapy is used in 41.9% of sites, mainly with methotrexate (74.3%). As many as 20 centers have a national registry for patients with breast cancer with central nervous system metastases and of those 5 have one for CM. Most (90.9%) centers would be interested in participating in a registry as well as in studies for CM, the latter preferably (62.1%) breast cancer subtype specific. CONCLUSIONS: This is the first study to map out the approach to CM from breast cancer globally. Although guidelines with level 1 evidence are lacking, there is a high degree of homogeneity in the approach to CM globally and great interest for conducting studies in this area.
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Neoplasias Encefálicas , Neoplasias de la Mama , Carcinomatosis Meníngea , Neoplasias Cutáneas , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/secundario , Neoplasias Encefálicas/terapia , Neoplasias de la Mama/patología , Neoplasias de la Mama/terapia , Femenino , Humanos , Oncología MédicaRESUMEN
BACKGROUND: Integration of specialist palliative care (PC) into standard oncology care is recommended. This study investigated how integration at the Cantonal Hospital St. Gallen (KSSG) was manifested 10 years after initial accreditation as a European Society for Medical Oncology (ESMO) Designated Center (ESMO-DC) of Integrated Oncology and Palliative Care. METHODS: A chart review covering the years 2006-2009 and 2016 was carried out in patients with an incurable malignancy receiving PC. Visual graphic analysis was utilized to identify patterns of integration of PC into oncology based on the number and nature of medical consultations recorded for both specialties. A follow-up cohort collected 10 years later was analyzed and changes in patterns of integrating specialist PC into oncology were compared. RESULTS: Three hundred and forty-five patients from 2006 to 2009 and 64 patients from 2016 were included into analyses. Four distinct patterns were identified using visual graphic analysis. The 'specialist PC-led pattern' (44.9%) and the 'oncology-led pattern' (20.3%) represent disciplines that took primary responsibility for managing patients, with occasional and limited involvement from other disciplines. Patients in the 'concurrent integrated care pattern' (18.3%) had medical consultations that frequently bounced between specialist PC and oncology. In the 'segmented integrated care pattern' (16.5%), patients had sequences of continuous consultations provided by one discipline before alternating to a stretch of consultations provided by the other specialty. In the 2016 follow-up, while the 'oncology-led pattern' occurred significantly less frequently relative to the 'specialist PC-led pattern' and the 'segmented integrated care pattern', the 'concurrent integrated care pattern' emerged more frequently when compared with the 2006-2009 follow-up. CONCLUSION: The 'specialist PC-led pattern' was the most prominent pattern in this data. The 2016 follow-up showed that a growing number of patients received a collaborative pattern of care, indicating that integration of specialist PC into standard oncology can manifest as either segmented or concurrent care pathways. Our data suggest a closer, more dynamic and flexible collaboration between oncology and specialist PC early in the disease course of patients with advanced cancer and concurrent with active treatment.
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Enfermería de Cuidados Paliativos al Final de la Vida , Neoplasias , Estudios de Cohortes , Humanos , Oncología Médica , Neoplasias/terapia , Cuidados PaliativosRESUMEN
INTRODUCTION: Pancreatic adenocarcinoma (PAC) is a highly malignant tumor with relevant morbidity and mortality. The role of adjuvant chemoradiotherapy (CRT) for primarily resected tumors remains controversial. We aimed to assess the outcome of patients treated at our institution with postoperative CRT for PAC. METHODS: We present a retrospective case series of patients with pancreatic adenocarcinoma at a single center in Switzerland. These patients were treated by primary surgery followed by adjuvant CRT between 1995 and 2015. The results were compared with published data. RESULTS: Median follow-up for the 60 patients was 33 months (range 19.9-193.9); median overall survival (OS) for patients undergoing a resection followed by combined CRT was 25.5 months. Overall, disease-free survival (DFS) was 15.2 months. A local recurrence occurred in 14 patients (23.3%) after a median time of 8.8 months, and in 43 patients (71.7%) distant metastasis was demonstrated with a median time to metastasis of 10.6 months. CONCLUSION: This retrospective study represents one of the sole reviews of outcome data after adjuvant CRT in resected PAC in Europe within the past years. OS was comparable to that of other institutional outcome data published previously but inferior when compared to most recent published results with an intense chemotherapy. However, not all patients are suitable to undergo such an intense chemotherapy with modified FOLFIRINOX after the extensive surgery for the PAC - these patients could benefit from adding adjuvant CRT to a less intensive chemotherapy with gemcitabine to enhance the benefit regarding locoregional recurrence-free survival.
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Adenocarcinoma/terapia , Quimioradioterapia Adyuvante/mortalidad , Pancreatectomía/mortalidad , Neoplasias Pancreáticas/terapia , Adenocarcinoma/patología , Adenocarcinoma/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/cirugía , Pronóstico , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
The human platelet antigens (HPA) are genetically defined polymorphisms expressed on platelet membrane glycoproteins. As platelet antigens are very important in several clinical situations and in population genetics, we used the polymerase chain reaction with sequence-specific primers (PCR-SSP) to investigate HPA-1, -2, -3 and -5 allele frequencies in the Croatian population. The HPA frequencies obtained in 219 Croatians were: 1a-0.854, 1b-0.146, 2a-0.890, 2b-0.110, 3a-0.575, 3b-0.425, 5a-0.895 and 5b-0.105. These data are similar to the frequencies reported in most European studies with some significant differences in HPA-2 when compared with the Dutch and German population, in HPA-3 when compared with the Swiss population and in HPA-5 when compared with the Finnish population. The three most common condensed HPA genotypes in the Croatian population were: HPA-1a/a, -2a/a, -3a/b, -5-a/a (0.283), HPA-1a/a, -2a/a, -3a/a, -5-a/a (0.137) and HPA-1a/b, -2a/a, -3a/b, -5-a/a (0.087). Data obtained in this study can be used for better understanding and treatment of immune-mediated platelet disorders in our population.
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Antígenos de Plaqueta Humana/genética , Glicoproteínas de Membrana Plaquetaria/genética , Adolescente , Adulto , Anciano , Alelos , Niño , Preescolar , Croacia , Europa (Continente) , Femenino , Frecuencia de los Genes , Genotipo , Humanos , Lactante , Masculino , Persona de Mediana Edad , Fenotipo , Adulto JovenRESUMEN
BACKGROUND: Careful selection of malignant pleural mesothelioma (MPM) patients for curative treatment is of highest importance, as the multimodal treatment regimen is challenging for patients and harbors a high risk of substantial toxicity. Radiomics-a quantitative method for image analysis-has shown its prognostic ability in different tumor entities and could therefore play an important role in optimizing patient selection for radical cancer treatment. So far, radiomics as a prognostic tool in MPM was not investigated. MATERIALS AND METHODS: This study is based on 72 MPM patients treated with surgery in a curative intent at our institution between 2009 and 2017. Pre-treatment Fluorine-18 fluorodeoxyglucose (FDG) PET and CT scans were used for radiomics outcome modeling. After extraction of 1404 CT and 1410 FDG PET features from each image, a preselection by principal component analysis was performed to include only robust, non-redundant features for the cox regression to predict the progression-free survival (PFS) and the overall survival (OS). Results were validated on a separate cohort. Additionally, SUVmax and SUVmean, and volume were tested for their prognostic ability for PFS and OS. RESULTS: For the PFS a concordance index (c-index) of 0.67 (95% CI 0.52-0.82) and 0.66 (95% CI 0.57-0.78) for the training cohort (n = 36) and internal validation cohort (n = 36), respectively, were obtained for the PET radiomics model. The PFS advantage of the low-risk group translated also into an OS advantage. On CT images, no radiomics model could be trained. SUV max and SUV mean were also not prognostic in terms of PFS and OS. CONCLUSION: We were able to build a successful FDG PET radiomics model for the prediction of PFS in MPM. Radiomics could serve as a tool to aid clinical decision support systems for treatment of MPM in future.
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BACKGROUND: In view of the lack of recommendations on central venous catheter (CVC)-associated thrombosis in cancer patients, we established guidelines according to the well-standardized Standards, Options and Recommendations methodology. MATERIAL AND METHODS: A literature review (1990-2007) on CVC-associated thrombosis was carried out. The guidelines were developed on the basis of the corresponding levels of evidence derived from analysis of the 36 of 175 publications selected. They were then peer reviewed by 65 independent experts. RESULTS: For the prevention of CVC-associated thrombosis, the distal tip of the CVC should be placed at the junction between the superior cava vein and right atrium; anticoagulants are not recommended. Treatment of CVC-associated thrombosis should be based on the prolonged use of low-molecular weight heparins. Maintenance of the catheter is justified if it is mandatory, functional, in the right position, and not infected, with a favorable clinical evolution under close monitoring; anticoagulant treatment should then be continued as long as the catheter is present. CONCLUSIONS: Several rigorous studies do not support the use of anticoagulants for the prevention of CVC-associated thrombosis. Treatment of CVC-associated thrombosis relies on the same principles as those applied in the treatment of established thrombosis in cancer patients.
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Cateterismo Venoso Central/efectos adversos , Neoplasias/complicaciones , Trombosis de la Vena/prevención & control , Anticoagulantes/uso terapéutico , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Neoplasias/terapiaRESUMEN
INTRODUCTION: Agranulocytosis or allergic skin reactions are common side effects of antithyroid drugs. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis is very uncommon. CASE REPORT: We report a 29-year-old woman treated with propylthiouracil for Graves' disease who developed a vasculitic skin involvement. ANCA with antimyeloperoxidase specificity were documented. Symptoms resolved after discontinuation of the drug. CONCLUSION: ANCA associated vasculitis is an unusual complication of propylthiouracil. Prognosis is conditioned by renal and pulmonary involvement.
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Anticuerpos Anticitoplasma de Neutrófilos/metabolismo , Antitiroideos/efectos adversos , Propiltiouracilo/efectos adversos , Vasculitis/inducido químicamente , Vasculitis/inmunología , Adulto , Femenino , HumanosRESUMEN
Bleomycin is a cytotoxic agent used in the treatment of various neoplasias. Its cutaneous adverse effects are diverse. Some of them are rare but specific. We report the case of a 40-year-old man presenting with a non-seminomatous testicular germ cell tumour who developed a flagellate erythema related to a bleomycin administration. Clinical features, histopathology and disease course are presented. This side effect is apparently neither related to the dose nor to the mode of administration of bleomycin. The etiopathogenic mechanism remains unknown.
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Antibióticos Antineoplásicos/efectos adversos , Bleomicina/efectos adversos , Eritema/inducido químicamente , Adulto , Erupciones por Medicamentos/etiología , Humanos , MasculinoRESUMEN
PURPOSE: Relationships between granulomatosis and cancers have been suspected for a long time. Nevertheless, few evidence has been reported until recently. Here, we present a literature review about the association of granulomatosis and neoplasia. CURRENT KNOWLEDGE AND KEY POINTS: Aside from granulomatosis due to infectious disease, granulomas can be observed in cancer patients, mainly in two situations. Patients may rarely present with typical sarcoidosis occurring before, during or after the diagnosis of cancer. Recent studies have documented such a relationship particularly with lymphomas, testicular and lung cancers, melanomas and hepatocarcinomas. Secondly granulomas may be found as a sarcoid reaction in the vicinity of the tumour itself or more frequently in regional lymph nodes. Sarcoid reaction, reported in Hodgkin's disease and gastric adenocarcinomas, may be associated with a better prognosis. Granulomatous reaction could play an important role in the host's defences against metastatic extension. Immunotherapy such as interferon has been reported to induce systemic sarcoidosis probably by reproducing some physiopathological mechanisms involved in sarcoidosis. FUTURE PROSPECTS AND PROJECTS: Clinicians need novel non invasive diagnostic methods to differentiate neoplasia from benign sarcoid reactions. The 18-fluorodeoxyglucose (18-FDG) PET-scan has failed in this indication but the adjunction of a [3-(18)F]-alpha-methyltyrosine ((18)F-FMT) PET-scan could be useful. Biopsies is still necessary in most of cases.
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Granuloma/inmunología , Neoplasias/inmunología , Sarcoidosis/inmunología , Neoplasias de la Mama/inmunología , Diagnóstico Diferencial , Neoplasias del Sistema Digestivo/inmunología , Femenino , Granuloma/diagnóstico , Neoplasias Hematológicas/inmunología , Humanos , Neoplasias Pulmonares/inmunología , Masculino , Neoplasias/diagnóstico , Sarcoidosis/diagnóstico , Neoplasias Cutáneas/inmunología , Neoplasias Testiculares/inmunologíaRESUMEN
INTRODUCTION: Agnogenic myeloid metaplasia, associated with myelofibrosis, is a myeloproliferative disorder. Extramedullary hematopoiesis in the pleura is rare and its prognosis is often severe. EXEGESIS: Herein we report a 64-year-old woman, who presented with pleural extramedullary hematopoiesis, treated by hydroxyurea-based chemotherapy with disease control. CONCLUSION: Clinical, histological, therapeutic and evolutive aspects of this uncommon entity will be reviewed.
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Hematopoyesis , Hidroxiurea/uso terapéutico , Derrame Pleural/tratamiento farmacológico , Femenino , Hematopoyesis/efectos de los fármacos , Humanos , Persona de Mediana Edad , Derrame Pleural/etiologíaRESUMEN
PURPOSE: Systemic granulomatosis (SG) are frequently encountered in internal medicine. Despite a large list of aetiologies, the investigations remain often negative leading to the diagnosis of atypical sarcoidosis. The spectrum of the causes, as well as evolution of these SG is not clearly delineated in the literature. METHOD: We analyzed the case reports of all but tuberculous GS submitted at the National Meetings of the National French Society of Internal Medicine from 1990 to 2006. RESULTS: Sixty-seven cases were included in the study. The average age at the beginning of the symptoms was 47.8 years and 28.4% of the patients were female. The median diagnostic delay was one year. General symptoms were present in 73.1% of the cases. The involved organs were the liver (46.3%), lungs (25.4%), lymph nodes (22.4%), digestive tract (16.4%), skin (16.4%), spleen (14.9%). The granuloma were detected mainly in the liver (38.8%), lymph nodes (17.9%), bone marrow (16.4%) and lungs (11.9%). Elevated erythrocyte sedimentation rate or increased C reactive protein serum levels were noted in 65.6% of the patients. Before diagnosis, 19.4% of the patients received a corticotherapy. The most common diagnoses were infections (65.6%) followed by drugs (19.5%), "toxic substances" or various foreign bodies (5.9%), neoplasias (5.9%) and immune deficiencies (3%). The evolution was favourable in 80% of the cases but 8.3% of the patients died. The disease course of the patients having received a corticotherapy prior to the diagnosis was more unfavourable with a death rate of 45%. CONCLUSION: In atypical sarcoidosis (fever, advanced age, increased acute phase reactants...) a specific aetiology and especially an infectious disease should be ruled out before considering the diagnosis of sarcoidosis. Corticotherapy is a factor of poor prognosis.
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Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/etiología , Sarcoidosis/diagnóstico , Sarcoidosis/etiología , Tuberculosis/diagnóstico , Adolescente , Corticoesteroides/administración & dosificación , Corticoesteroides/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Sedimentación Sanguínea , Proteína C-Reactiva/metabolismo , Niño , Diagnóstico Diferencial , Femenino , Granuloma , Granulomatosis con Poliangitis/microbiología , Granulomatosis con Poliangitis/mortalidad , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Sarcoidosis/microbiología , Sarcoidosis/mortalidad , Análisis de Supervivencia , Tuberculosis/microbiología , Tuberculosis/mortalidadRESUMEN
INTRODUCTION: The paraganglioma is a rare tumour that can sometimes develop near to the pancreatic gland and can occasionally mimic an intra pancreatic mass. Invasive investigations with puncture or primary surgical resection must be avoided because of the risk of vital complications. EXEGESIS: We report on the case of a 41 years old woman presenting with a mass apparently affecting the pancreatic gland. She also presented with malaises. Paraganglioma was suspected because of the malaises and the blood pressure crises. The diagnosis was permitted by the findings of high urinary metanephrine levels. So the tumour was resected without previous fine needle aspiration and with specific anaesthetic measures. No complication was observed. CONCLUSION: When a patient presents with a pancreatic mass, physician must carefully take into account the possibility of a paraganglioma particularly when malaises are present.
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Neoplasias Pancreáticas/diagnóstico , Paraganglioma/diagnóstico , Adulto , Biomarcadores de Tumor/sangre , Biomarcadores de Tumor/orina , Cromogranina A/sangre , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Metanefrina/sangre , Metanefrina/orina , Normetanefrina/sangre , Normetanefrina/orinaRESUMEN
OBJECTIVES: Increased incidence of cancers and the development of totally implanted venous access devices that contain their own port to deliver chemotherapy will lead to a greater than before numbers of central venous catheter related thrombosis (CVCT). Medical consequences include catheter dysfunction and pulmonary embolism. Compared with lower extremity deep venous thrombosis (DVT) (3 d) and with non CVC associated thrombosis (5 d), CVCT is associated with an increased duration of hospitalisation (9 d). CVCT oftentimes leads to the need to replace such ports at an average cost of 4500 euros. CURRENT KNOWLEDGE AND KEY POINTS: Vessel injury caused by the procedure of CVC insertion is the most important risk factor for development of CVCT. This event could cause the formation of a fresh thrombus, which is reversible in the large majority of patients. The incidence of CVC-related DVT assessed by venography has been reported to vary from 30 to 60% but catheter-related DVT in adult patients is symptomatic in only 5% of cases. The majority of patients with CVC-related DVT is asymptomatic or has non-specific symptoms: arm or neck swelling or pain, distal paresthesias, headache, congestion of subcutaneous collateral veins. In the case of clinical suspicion of CVC-related DVT, compressive ultrasonography (US), especially with Doppler and color imaging, currently is first used to confirm the diagnosis. The main criteria of color-Doppler US are visualization of mural thrombi or incompressibility of the veins. Consequently, contrast venography is reserved for clinical trials and difficult diagnostic situations. There is no consensus on the optimal management of patients with CVC-related DVT. Treatment of CVC-related VTE requires a 5- to 7-day course of adjusted-dose unfractionated heparin or LMWH followed by oral anticoagulants. Long-term LMWH that has been shown to be more effective than oral anticoagulant in cancer patients with lower limb DVT could be used in these patients. The optimal duration of oral anticoagulation treatment for CVC-related DVT is unknown, but patients with active cancer should be treated for at least 6 months or indefinitely. FUTURE PROSPECTS AND PROJECTS: The efficacy and safety of pharmacologic prophylaxis for CVC related thrombosis is not established. Additional studies performed in high risk populations are needed to define if LMWH or oral anticoagulation is indicated in this clinical setting.
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Cateterismo Venoso Central/efectos adversos , Neoplasias/tratamiento farmacológico , Tromboembolia/etiología , Cateterismo Venoso Central/instrumentación , Cateterismo Venoso Central/métodos , Diseño de Equipo , Femenino , Humanos , Masculino , Tromboembolia/epidemiologíaRESUMEN
INTRODUCTION: Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. It presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia. CASE REPORT: We describe a patient with SCLS with recurrent lipothymia who presented first with delayed oedema that was thought to be due to orlistat treatment. On the second episode the patient was seen with a pulmonary hypertension when plasma came back into vessels. On the third time the characteristic triad led to the diagnosis of SCLS. DISCUSSION: SCLS should be considered in the differential diagnosis of recurrent hypovolemic shock without identifiable cause. Nevertheless, symptoms may be restricted to sole lipothymia or transient oedema or delayed hypoalbuminemia rendering the diagnosis difficult.
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Síndrome de Fuga Capilar/diagnóstico , Diagnóstico Diferencial , Hematócrito , Humanos , Hipoalbuminemia/diagnóstico , Hipotensión Ortostática/diagnóstico , Masculino , Choque/diagnóstico , Síncope/diagnósticoRESUMEN
OBJECTIVES: The risk of venous thrombosis during cancer is largely increased especially in case of chemotherapy, surgery, advanced stage disease, coagulation abnormalities. Survival of patients with cancer experiencing venous thrombosis seems to be worse. Although thrombosis may be a presenting feature of occult malignancy, there are insufficient data to support a more extensive screening than comprehensive medical history, physical examination, routine laboratory tests and chest radiography. CURRENT KNOWLEDGE AND KEY POINTS: Pathophysiology of venous thrombosis during cancer is unspecific: venous stasis, vessel wall damage, hypercoagulability). Other factors like platelet abnormalities or the direct responsibility of chemotherapy or hormonotherapy have recently been though to play a causative role. Treatment of cancer-associated thrombosis usually requires at least 6 months of low-molecular-weight heparin therapy rather than oral anticoagulant. Inferior vena cava filters are not indicated. Primary prophylaxis of thrombosis during cancer could safely been achieved with low-molecular-weight heparin. Central venous catheters can be associated with thrombotic complications. Many risks factors have been identified: catheter's type, modalities of catheter's implantation, type of perfusion, bulky mediastinal mass... Prophylactic anticoagulation is not routinely recommended. FUTURE PROSPECTS AND PROJECTS: Knew oral anticoagulants could facilitate the treatment of venous thrombosis occurring during cancer in the next years.