Registri di Patologia

  • Emanuele Crocetti1

  1. UO Epidemiologia clinica e descrittiva, ISPO Firenze
Emanuele Crocetti -

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Ricerca bibliografica periodo dal 1 giugno 2012 al 15 agosto 2012

Per leggere le caratteristiche di questa ROUTINE di ricerca clicca qui

Stringa: (("registries"[MeSH Terms] OR "registries"[All Fields] OR "registry"[All Fields]) OR ("registries"[MeSH Terms] OR "registries"[All Fields])) AND (("italy"[MeSH Terms] OR "italy"[All Fields]) OR italian[All Fields]) AND "humans"[MeSH Terms] AND ("2012/06/01"[PDat] : "2012/08/15"[PDat])
1. Ricci S, Dinia L, Del Sette M, Anzola P, Mazzoli T, Cenciarelli S, Gandolfo C. Sonothrombolysis for acute ischaemic stroke. Cochrane Database Syst Rev. 2012 Jun 13;6:CD008348.
UO Neurologia, ASL 1 dell’ Umbria, Città di Castello, Italy.

BACKGROUND: Sonothrombolysis is a promising but unproven tool for treating acute ischaemic stroke. There is an ongoing debate about the efficacy, safety, technical aspects of ultrasound administration and the possible potentiating effect of microbubbles.
OBJECTIVES: To assess the effectiveness and safety of sonothrombolysis in patients with acute ischaemic stroke.
SEARCH METHODS: We searched the Cochrane Stroke Group Trials Register (last searched in November 2011), the Cochrane Controlled Trials Register (The Cochrane Library 2011, Issue 12), MEDLINE (1950 to November 2011), EMBASE (1980 to November 2011), Database of Abstract and Review of Effects (DARE) (The Cochrane Library 2011, Issue 11), Stroke Trials Registry, and Current Controlled Trials. We also searched the reference lists from relevant articles and reviews, and contacted colleagues, authors and researchers active in the field. Searching was completed in November 2011.
SELECTION CRITERIA: Randomised trials of sonothrombolysis (any duration, any frequency of ultrasound, with or without microbubbles administration) started within 12 hours of symptom onset compared with intravenous tissue plasminogen activator (tPA) or conventional treatment.
DATA COLLECTION AND ANALYSIS: Two review authors selected trials for inclusion, assessed trial quality and extracted the data independently. We contacted study authors for missing data.
MAIN RESULTS: We identified five eligible studies (233 patients). For the primary outcome (death or dependency at three months), five studies with a total number of 206 patients were available (four defined independence as a modified Rankin score of 0 to 2 and one used 0 to 1). Patients treated with sonothrombolysis were no more likely to be dead or disabled at three months (odds ratio (OR) 0.80, 95% confidence interval (CI) 0.45 to 1.44). For the secondary outcomes, failure to recanalise was lower in the sonothrombolysis group (230 patients) (OR 0.28, 95% CI 0.16 to 0.50), no significant difference was found in mortality (206 patients) and in cerebral haemorrhage (233 patients).
AUTHORS' CONCLUSIONS: Sonothrombolysis did not reduce death or dependency at three months, but appeared to increase recanalisation without clear hazard. A larger clinical trial is warranted.

2. Mathew MC, Ervin AM, Tao J, Davis RM. Antioxidant vitamin supplementation for preventing and slowing the progression of age-related cataract. Cochrane Database Syst Rev. 2012 Jun 13;6:CD004567.
MetroWest Medical Center, Framingham, Massachusetts, USA.

BACKGROUND: Age-related cataract is a major cause of visual impairment in the elderly. Oxidative stress has been implicated in its formation and progression. Antioxidant vitamin supplementation has been investigated in this context.
OBJECTIVES: To assess the effectiveness of antioxidant vitamin supplementation in preventing and slowing the progression of age-related cataract.
SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2012, Issue 2), MEDLINE (January 1950 to March 2012), EMBASE (January 1980 to March 2012), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to March 2012), Open Grey (System for Information on Grey Literature in Europe) (, the metaRegister of Controlled Trials (mRCT) (, ( and the WHO International Clinical Trials Registry Platform (ICTRP) ( There were no date or language restrictions in the electronic searches for trials. The electronic databases were last searched on 2 March 2012. We also checked the reference lists of included studies and ongoing trials and contacted investigators to identify eligible randomized trials.
SELECTION CRITERIA: We included only randomized controlled trials in which supplementation with one or more antioxidant vitamins (beta-carotene, vitamin C and vitamin E) in any form, dosage or combination for at least one year was compared to another antioxidant vitamin or to placebo.
DATA COLLECTION AND ANALYSIS: Two authors extracted data and assessed trial quality independently. We pooled results for the primary outcomes, i.e., incidence of cataract and incidence of cataract extraction. We did not pool results of the secondary outcomes - progression of cataract and loss of visual acuity, because of differences in definitions of outcomes and data presentation. We pooled results by type of cataract when data were available. We did not perform a sensitivity analysis.
MAIN RESULTS: Nine trials involving 117,272 individuals of age 35 years or older are included in this review. The trials were conducted in Australia, Finland, India, Italy, the United Kingdom and the United States, with duration of follow-up ranging from 2.1 to 12 years. The doses of antioxidant vitamins were higher than the recommended daily allowance. There was no evidence of effect of antioxidant vitamin supplementation in reducing the risk of cataract, cataract extraction, progression of cataract or in slowing the loss of visual acuity. In the pooled analyses, there was no evidence of effect of beta-carotene supplementation in reducing the risk of cataract (two trials) (relative risk (RR) 0.99, 95% confidence interval (CI) 0.91 to 1.08; n = 57,703) or in reducing the risk of cataract extraction (three trials) (RR 1.00, 95% CI 0.91 to 1.10; n = 86,836) or of vitamin E supplementation in reducing the risk of cataract (three trials) (RR 0.97, 95% CI 0.91 to 1.04; n = 50,059) or of cataract extraction (five trials) (RR 0.98, 95% CI 0.91 to 1.05; n = 83,956). The proportion of participants developing hypercarotenodermia (yellowing of skin) while on beta-carotene ranged from 7.4% to 15.8%.
AUTHORS' CONCLUSIONS: There is no evidence from RCTs that supplementation with antioxidant vitamins (beta-carotene, vitamin C or vitamin E) prevents or slows the progression of age-related cataract. We do not recommend any further studies to examine the role of antioxidant vitamins beta-carotene, vitamin C and vitamin E in preventing or slowing the progression of age-related cataract. Costs and adverse effects should be weighed carefully with unproven benefits before recommending their intake above recommended daily allowances.

3. Righi E, Bechtold P, Tortorici D, Lauriola P, Calzolari E, Astolfi G, Nieuwenhuijsen MJ, Fantuzzi G, Aggazzotti G. Trihalomethanes, chlorite, chlorate in drinking water and risk of congenital anomalies: a population-based case-control study in Northern Italy. Environ Res. 2012 Jul;116:66-73. Epub 2012 May 9.
Departiment of Public Health Sciences, University of Modena e Reggio Emilia, Modena, Italy.

BACKGROUND: Epidemiological evidence of an association between disinfection by-products (DBPs) exposure via drinking water and reproductive outcomes is still inconclusive.
OBJECTIVE: The aim of this study was to investigate the association between trihalomethanes (THMs), chlorite and chlorate exposure and congenital anomalies.
METHODS: A case-control study was carried out in Emilia-Romagna Region (Italy). Data on 1917 different congenital anomalies (neural tube, cardiac, diaphragm and abdominal wall, oesophagus, cleft lip and palate, respiratory, urinary tract and chromosomal anomalies) observed in the period 2002-2005 were extracted from the Regional Malformation Registry. Four controls (newborns without anomalies) were randomly selected form the Regional Birth Register and frequency matched to cases according to pregnancy period. The network supplying water during the first trimester of pregnancy was identified on the basis of mother's address: DBPs data, technical and structural information were linked to each subject.
RESULTS: Overall, THMs exposure was very low (mean: 3.8±3.6 μg/l), and no risk excess was observed. Chlorite and chlorate values were fairly high (mean: 427±184 μg/l and 283±79 μg/l, respectively). Women exposed to chlorite level >700 μg/l were at higher risk of newborns with renal defects (OR: 3.30; 95% IC: 1.35-8.09), abdominal wall defects (OR: 6.88; 95% IC: 1.67-28.33) and cleft palate (OR: 4.1; 95% IC: 0.98-16.8); women exposed to chlorate level >200 μg/l were at higher risk of newborns with obstructive urinary defects (OR: 2.88; 95% IC: 1.09-7.63), cleft palate (OR: 9.60; 95% IC:1.04-88.9) and spina bifida (OR: 4.94; 95% IC:1.10-22).
CONCLUSIONS: This was the first study showing an excess risk of different congenital anomalies related to chlorite and chlorate exposure via drinking water: further research is needed to confirm the observed relationships in large datasets, specifically for chlorate, an unregulated DBP.

Breve commento a cura di Emanuele Crocetti
Lo studio di Righi e coll., che fa parte di un progetto internazionale finanziato dalla Comunità Europea dal titolo “Health Impacts of long-term exposure to disinfection by-products in drinking water”, ha valutato la relazione fra l’esposizione delle madri durante il primo trimestre di gravidanza a trialometano, cloriti e clorati e l’insorgenza di malformazioni congenite nei nuovi nati. Le sostanze oggetto dello studio, chiamate nel complesso Disinfection by-products (DBPs), si formano come sottoprodotti del processo di clorazione delle acque per l’interazione fra il cloro, utilizzato per la potabilizzazione in varie forme chimiche, e le sostanze organiche originariamente presenti nelle acque.
Si tratta di un caso-controllo di popolazione che ha raccolto i casi, ovvero le malformazioni congenite, dal Registro delle malformazioni congenite (RMC) della regione Emilia-Romagna, un registro su base di popolazione che copriva nel periodo dello studio, 2002-2005, l’87% delle nascite in quella regione. In Italia non c’è un sistema di sorveglianza sulle malformazioni congenite a livello nazionale ma vi sono RMC in alcune aree e regioni (Registro lombardo malformazioni congenite, Registro malformazioni Nordest Italia, Indagine malformazioni Emilia-Romagna, Registro toscano difetti congeniti, Registro campano difetti congeniti, Indagine siciliana malformazioni congenite, Registro malformazioni congenite dell’ASL di Mantova).
Lo studio non ha evidenziato eccessi di rischio legati a trialometano per il quale le esposizioni sono risultate molto basse e comunque inferiori ai limiti di legge. Mentre per i cloriti ed i clorati oltre a identificare una quota di esposizioni superiori ai limiti stabiliti (cloriti) lo studio ha evidenziato un rischio aumentato per una serie di malformazioni. Si tratta di una prima segnalazione che solleva la necessità di confermare, con studi più numerosi e che superino i limiti presenti nello studio in questione, la relazione dei DBPs e in particolare dei clorati per i quali manca ancora una regolamentazione e le malformazioni congenite.

4. Rizzello V, Lucci D, Maggioni AP, Giampaoli S, Greco C, Di Pasquale G, Pallotti MG, Mureddu GF, Di Chiara A, Boccanelli A; IN-ACS Outcome Investigators. Clinical epidemiology, management and outcome of acute coronary syndromes in the Italian network on acute coronary syndromes (IN-ACS Outcome study). Acute Card Care. 2012 Jun;14(2):71-80. Epub 2012 Mar 27.
Collaborators: Boccanelli A, Giampaoli S, Bolognese L, Chiarella F, Di Pasquale G, Mafrici A, Scherillo M, Schweiger C, Di Chiara A, Maggioni AP, Greco C, Seccareccia F, Lucci D, Gonzini L, Sarti L, Priami P, Spinnler MT, Formato L, Guillevine R, Maffiotti A, Bobbio M, Meinardi F, Vanni A, Dallorto G, Nassiacos D, Pittana P, Klugmann S, Ferrari S, Bortolini F, Rodella P, Romanó M, Bertona R, Brunazzi MC, Negrelli M, Rossi R, Carbonieri E, Zampiero AA, Contessotto F, Fioretti P, Di Chiara A, Sinagra G, Maras P, Chiarella F, Rossi F, Domenicucci S, Lonati A, Filorizzo G, Petacchi R, Nardi R, Pozzati A, Pancaldi LG, Capecchi A, Di Pasquale G, Pavesi PC, Pallotti MG, Zuppiroli A, Sani F, Bolognese L, Sabini A, Cocchieri M, Severini D, Patriarchi F, Liberati R, Gelibter D, Palmieri C, Boccanelli A, Greco C, Luongo R, Ceci V, Ricci R, Caianiello G, Bini A, Lioy E, Sciahbasi A, Vacri A, De Finis A, Di Nucci G, Attademo G, Armogida N, Lucà S, Prinzi D, De Rosa P, Stanco G, Mariello C, Franchini G, Sublimi Saponetti L, Gallone V, Andriulo C, Pettinati G, Greco A, Tolaro S, Bottaro G, Marchi SM, Meloni M. San Giovanni-Addolorata Hospital, Department of Cardiology, Rome, Italy.

BACKGROUND: The Italian network on acute coronary syndromes outcome (IN-ACS Outcome) study is a nationwide observational, multicenter study with the aim to describe clinical epidemiology, management, 30-days and one-year outcomes of ACS in Italy.
METHODS: All consecutive patients admitted for ACS to 38 hospitals, between December 2005 and February 2007, were enrolled in the study. Patient in-hospital details and follow-up data at 30-days and one-year were collected using a web-based CRF and stored in a central database.
RESULTS: A total of 6045 patients (age 68 ± 13 years) were enrolled: 2313 patients (38.3%) had ST elevation myocardial infarction (STEMI) and 3732 (61.7%) patients had NSTE-ACS. Primary PCI was performed in 1085 (46.9%) STEMI patients, thrombolysis in 590 (25.5%) patients, whereas 638 (27.6%) patients were not reperfused. Among patients with NSTE-ACS, coronary angiography was performed in 2797 (75%) patients, PCI in 1797 (48.2%) patients and CABG in 213 (5.7%) patients. Thirty-days and one-year mortality rates were 5.8% and 9.8%, in STEMI patients and 3.1% and 8.6%, in NSTE-ACS patients.
CONCLUSIONS: The IN-ACS Outcome study showed that the management of ACS is still suboptimal. Although 30-days mortality is low, the one-year mortality is still substantial.

5. Luisetti M, Balfour-Lynn IM, Johnson SR, Miravitlles M, Strange C, Trapnell BC, van Bronswijk H, Vogelmeier C. Perspectives for improving the evaluation and access of therapies for rare lung diseases in Europe. Respir Med. 2012 Jun;106(6):759-68. Epub 2012 Mar 11.
Department of Molecular Medicine, Section of Pneumology, University of Pavia, IRCCS San Matteo Hospital Foundation, Pavia, Italy.

The approach to treating a rare disease is different to that taken for more common diseases. Small patient cohorts alter clinical trial design and limit enrollment, and the picture for rare lung diseases is further complicated by the fact that most are composed of a variety of clinical phenotypes. Since the outcome measures of lung impairment have considerable test-to-test variability, potential new therapies face a substantial challenge. In this paper we will review the current sources of clinical data for rare lung diseases and the regulatory challenges encountered by their treatment, with particular reference to alpha(1)-antitrypsin deficiency, lymphangioleiomyomatosis, cystic fibrosis, and pulmonary alveolar proteinosis. Strategies will also be identified for the better utilization of available data from patients with rare lung diseases, recognizing that the development cost of new therapies and the number of patients who will ultimately use them may not be aligned. Also important is improved communication between patients and their organizations, basic researchers, clinicians and their registries, drug developers, regulators such as the European Medicines Agency, and national health services. At present, licensing and reimbursement requirements are not aligned, either nationally or internationally, and variations also exist in drug availability between countries because of different national licensing and reimbursement rules. The changes needed to optimize European rare lung disease therapies include a commitment to develop empowered patient communities as advocates for therapy, the development of novel trial designs with new endpoints, and for regulatory bodies to be willing to accept nontraditional models of efficacy for orphan drugs.

6. Nordio M, Limido A, Maggiore U, Nichelatti M, Postorino M, Quintaliani G; Italian Dialysis and Transplantation Registry. Survival in patients treated by long-term dialysis compared with the general population. Am J Kidney Dis. 2012 Jun;59(6):819-28. Epub 2012 Feb 22.
Collaborators: Molino A, Salomone M, Cappelli G, Conte F, Arosio E, Antonucci F, Giacon B, Adorati M, Romanini D, Santoro A, Mancini E, Rosati A, Frascà GM, Gaffi G, Standoli M, Bonomini M, Di Liberato L, Di Giulio S, Cirillo M, Bilancio G, Schena FP, Torres D, Casino F, Zoccali C, Marino C, Sparacino V, Agnello V, Pinna AM. Nephrology and Dialysis Unit, ULSS 15, Camposampiero-Cittadella, Padua, Italy.

BACKGROUND: Relative survival, a methodology previously used in epidemiologic studies of cancer, compares the observed survival of a patient cohort with expected survival derived from general population life tables. We examined relative survival in patients treated by long-term dialysis in the Italian Dialysis and Transplantation Registry in order to determine the prognosis of dialysis patients.
STUDY DESIGN: Cohort study drawn from a registry.
SETTING & PARTICIPANTS: Patients enrolled in the Italian Dialysis and Transplantation Registry. FACTORS: Sex, age, primary kidney disease, renal replacement therapy modality, and main comorbid conditions.
OUTCOMES: Death from any cause.
MEASUREMENTS: Relative survival ratio (the ratio of observed survival in the population of interest to the survival expected given the age- and period-specific mortality of the general population) and excess mortality rate (difference between observed and expected mortality rates).
RESULTS: In January 2000 to December 2008, a total of 27,642 patients were included. The 5-year relative survival estimate was 55.6% (95% CI, 54.7%-56.5%). The excess mortality rate showed a peak at 3 months (21 deaths/100 patient-years), then decreased, becoming constant from the end of year 1 to year 8, with leveling off at about 10 deaths/100 patient-years. Older age, systemic diseases, and diabetes showed the strongest association with excess mortality. Peritoneal dialysis was associated with a lower relative excess risk in only the first year of treatment.
LIMITATIONS: The patient cohort comprises about half the Italian patients beginning dialysis therapy in the period.
CONCLUSIONS: This study highlights the applicability of relative survival methods in dialysis patients. This measure allows estimation of disease prognosis and severity comparisons among chronic diseases. The excess mortality rate appears to be a more sensitive and informative measure than the simple proportion of survivors.

7. Mavrogenis AF, Guerra G, Romantini M, Romagnoli C, Casadei R, Ruggieri P. Tumours of the atlas and axis: a 37-year experience with diagnosis and management. Radiol Med. 2012 Jun;117(4):616-35. Epub 2011 Nov 17.
Orthopaedic Oncology Service, Department of Orthopaedics, Istituto Ortopedico Rizzoli, University of Bologna, Via Di Barbiano 1/10, 40136, Bologna, Italy.

PURPOSE: This paper presents a single institution's longterm experience regarding the incidence and management of tumours of the atlas and axis and discusses clinical and imaging findings and treatment options.
MATERIALS AND METHODS: We searched the registry of the Istituto Ortopedico Rizzoli for patients admitted and treated for tumours of the upper cervical spine. We identified 62 patients over 37 years, from July 1973 to October 2010. There were 39 male and 23 female patients, with a mean age of 39.5 (range 5-77) years. For each patient, we collected data on clinical presentation, imaging and treatment. Mean follow-up was 10 years.
RESULTS: Benign bone tumours were diagnosed in 24 (39%) and malignant tumours in 38 (61%) patients. The most common tumours were bone metastases, followed by osteoid osteomas and chordomas. The atlas was involved in six and the axis in 52 patients; in four patients, both the atlas and axis were involved. The most common clinical presentation was pain, torticollis, dysphagia and neurological deficits. Surgical treatment was performed in 35 patients and conservative treatment, including intralesional methylprednisolone injections and halo-vest immobilisation with or without radiation therapy, chemotherapy or embolisation, in the remaining patients. One patient with osteoblastoma of the atlas had local recurrence. All patients with metastatic bone disease had local recurrence; four of the eight patients with plasmacytoma progressed to multiple myeloma within 1-4 years. All patients with chordomas had two to four local recurrences. Patients with osteosarcomas and chondrosarcoma died owing to local and distant disease progression.
CONCLUSIONS: Bone tumours of the cervical spine are rare. However, they should be kept in mind when examining patients with neck pain or neurological symptoms at the extremities. In most cases, only intralesional surgery can be administered. Combined radiation therapy and chemotherapy is indicated for certain tumour histologies.

8. Bisogno G, Pastore G, Perilongo G, Sotti G, Cecchetto G, Dallorso S, Carli M. Long-term results in childhood rhabdomyosarcoma: a report from the Italian Cooperative Study RMS 79. Pediatr Blood Cancer. 2012 Jun;58(6):872-6. doi: 10.1002/pbc.23292. Epub 2011 Aug 16.
Department of Pediatrics, University Hospital of Padova, Padova, Italy.

BACKGROUND: The results obtained by protocols for children with rhabdomyosarcoma (RMS) have improved in recent decades. Survival curves usually reach a plateau 3 years after the diagnosis, suggesting that long-term survival can be expected, but late events are known to occur. We analyzed the long-term results of the RMS 79 protocol to investigate the type and impact of such events.
PROCEDURE: From 1979 to 1987, 163 children with RMS diagnosed at 21 Italian institutions were registered. Each institution was contacted every year to record patients' status after the end of treatment. When patients were lost to follow-up, their status was checked by inquiring at the Registry Offices of the towns of residence and the cause of death or occurrence of second cancers was investigated by contacting the patients or their family by phone.
RESULTS: Overall, 16 patients had late events, that is, 7 tumor recurrences, 6 second tumors, and 3 deaths due to treatment-related complications. The overall survival rates dropped from 62.6 at 3 years to 52.8 at 20 years. By multivariate analysis, the characteristics influencing long-term survival were histology, tumor site and size, and IRS group. Factors predictive of any kind of late event were tumor site and IRS group.
CONCLUSIONS: Major late events can significantly affect the long-term survival of children with RMS. Modern protocols should provide for a much longer follow-up than is usually considered to confirm the results achieved and enable possible correlations between primary treatment and late events to be investigated.

9. Scaruffi P, Morandi F, Gallo F, Stigliani S, Parodi S, Moretti S, Bonassi S, Fardin P, Garaventa A, Zanazzo G, Pistoia V, Tonini GP, Corrias MV. Bone marrow of neuroblastoma patients shows downregulation of CXCL12 expression and presence of IFN signature. Pediatr Blood Cancer. 2012 Jul 15;59(1):44-51. doi: 10.1002/pbc.23339. Epub 2011 Oct 12.
Translational Oncopathology, National Cancer Research Institute, Genoa, Italy.

BACKGROUND: At diagnosis, children with neuroblastoma (NB) present with either localized or metastatic disease. Since the mechanisms responsible for BM invasion are not well known, we investigated the transcriptome of resident BM cells from NB patients as compared to healthy children.
PROCEDURE: Ninety-two and 88 children with localized and metastatic NB, respectively, and 15 healthy children were included in the study. BM resident cells recovered from BM aspirates by immunomagnetic bead manipulation were subjected to genome-wide microarray analysis. After validation in an independent set of samples, the genes significantly modulated in resident BM cells from NB patients were tested for their diagnostic/prognostic values.
RESULTS: BM resident cells, irrespective of neoplastic cell invasion, significantly overexpressed genes involved in innate immune responses, and interferon (IFN) and IFN-DRS signatures were enriched. Genes coding for metallothioneins and zinc finger proteins, and involved in histone and nucleosome/chromatin organization were also overexpressed. Resident BM cells from NB patients significantly downregulated genes involved in cell adhesion, and in erythrocyte, myeloid, and platelet differentiation pathways. Among downregulated genes, CXCL12 expression reached near complete silencing in patients with metastatic disease. The downregulation of CXCL12 expression was independent of contact between NB cell and resident BM cell.
CONCLUSIONS: We demonstrated that NB tumor growth at the primary site can alter the BM microenvironment, and the presence of BM-infiltrating NB cells makes the alterations more pronounced. Therefore, the restoration of a BM physiological state by means of IFN-α monoclonal antibody, Sifalimumab, and selective noradrenaline receptor blockers should be further studied to ameliorate patients' clinical management.

10. Sant M, Minicozzi P, Lagorio S, Børge Johannesen T, Marcos-Gragera R, Francisci S; EUROCARE Working Group. Survival of European patients with central nervous system tumors. Int J Cancer. 2012 Jul 1;131(1):173-85. doi: 10.1002/ijc.26335. Epub 2011 Nov 10.
Collaborators: Oberaigner W, Hackl M, Eycken EV, Verstreken M, Holub J, Jurickova L, Storm HH, Engholm G, Hakulinen T, Belot A, Hédelin G, Velten M, Guizard AV, Danzon A, Buemi A, Tretarre B, Colonna M, Bara S, Ganry O, Grosclaude P, Ziegler H, Tryggvadottir L, Comber H, Berrino F, Allemani C, Baili P, Ciampichini R, Ciccolallo L, Gatta G, Margutti C, Micheli A, Minicozzi P, Sant M, Sowe S, Tereanu C, Zigon G, Tagliabue G, Contiero P, Bellu F, Giacomin A, Ferretti S, Serraino D, Maso LD, De Dottori M, De Paoli A, Zanier L, Vercelli M, Orengo MA, Casella C, Quaglia A, Pannelli F, Federico M, Rashid I, Cirilli C, Fusco M, De Lisi V, Bozzani F, Tumino R, La Rosa MG, Spata E, Sigona A, Mangone L, Falcini F, Foca F, Giorgetti S, Senatore G, Iannelli A, Budroni M, Zanetti R, Patriarca S, Rosso S, Piffer S, Franchini S, Paci E, Crocetti E, La Rosa F, Stracci F, Cassetti T, Zambon P, Guzzinati S, Caldora M, Capocaccia R, Carrani E, De Angelis R, Francisci S, Grande E, Inghelmann R, Lenz H, Martina L, Roazzi P, Santaquilani M, Simonetti A, Tavilla A, Verdecchia A, Dalmas M, Langmark F, Bray F, Johannesen TB, Rachtan J, Góźdź S, Siudowska U, Mezyk R, Bielska M, Sklodowska M, Zwierko M, Miranda A, Diba CS, Primic- Źakelj M, Izarzugaza I, Marcos-Gragera R, Vilardell ML, Izquierdo A, Navarro C, Chirlaque MD, Ardanaz E, Moreno C, Galceran J, Klint Å, Talbäck M, Jundt G, Usel M, Ess SM, Bordoni A, Luthi JC, Konzelmann I, Lutz JM, Pury P, Visser O, Otter R, Siesling S, van der Zwan JM, Schaapveld M, Coebergh JW, Janssen-Heijnen ML, van der Heijden L, Greenberg DC, Coleman MP, Moran T, Forman D, Cooper N, Roche M, Verne J, Møller H, Meechan D, Poole J, Lawrence G, Gavin A, Black RJ, Brewster DH, Steward JA. Analytical Epidemiology Unit, Department of Preventive and Predictive Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, Milan, Italy.

We present estimates of population-based 5-year relative survival for adult Europeans diagnosed with central nervous system tumors, by morphology (14 categories based on cell lineage and malignancy grade), sex, age at diagnosis and region (UK and Ireland, Northern, Central, Eastern and Southern Europe) for the most recent period with available data (2000-2002). Sources were 39 EUROCARE cancer registries with continuous data from 1996 to 2002. Survival time trends (1988 to 2002) were estimated from 24 cancer registries with continuous data from 1988. Overall 5-year relative survival was 85.0% for benign, 19.9% for malignant tumors. Benign tumor survival ranged from 90.6% (Northern Europe) to 77.4% (UK and Ireland); for malignant tumors the range was 25.1% (Northern Europe) to 15.6% (UK and Ireland). Survival decreased with age at diagnosis and was slightly better for women (malignant tumors only). For glial tumors, survival varied from 83.5% (ependymoma and choroid plexus) to 2.7% (glioblastoma); and for non-glioma tumors from 96.5% (neurinoma) to 44.9% (primitive neuroectoderm tumor/medulloblastoma). Survival differences between regions narrowed after adjustment for morphology and age, and were mainly attributable to differences in morphology mix; however UK and Ireland and Eastern Europe patients still had 40% and 30% higher excess risk of death, respectively, than Northern Europe patients (reference). Survival for benign tumors increased from 69.3% (1988-1990) to 77.1% (2000-2002); but survival for malignant tumors did not improve indicating no useful advances in treatment over the 14-year study period, notwithstanding major improvement in the diagnosis and treatment of other solid cancers.

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