ITALIAN CANCER FIGURES - REPORT 2015: The burden of rare cancers in Italy = I TUMORI IN ITALIA - RAPPORTO 2015: I tumori rari in Italia

OBJECTIVES: This collaborative study, based on data collected by the network of Italian Cancer Registries (AIRTUM), describes the burden of rare cancers in Italy. Estimated number of new rare cancer cases yearly diagnosed (incidence), proportion of patients alive after diagnosis (survival), and estimated number of people still alive after a new cancer diagnosis (prevalence) are provided for about 200 different cancer entities. MATERIALS AND METHODS: Data herein presented were provided by AIRTUM population- based cancer registries (CRs), covering nowadays 52% of the Italian population. This monograph uses the AIRTUM database (January 2015), which includes all malignant cancer cases diagnosed between 1976 and 2010. All cases are coded according to the International Classification of Diseases for Oncology (ICD-O-3). Data underwent standard quality checks (described in the AIRTUM data management protocol) and were checked against rare-cancer specific quality indicators proposed and published by RARECARE and HAEMACARE (www.rarecarenet.eu; www.haemacare.eu). The definition and list of rare cancers proposed by the RARECAREnet "Information Network on Rare Cancers" project were adopted: rare cancers are entities (defined as a combination of topographical and morphological codes of the ICD-O-3) having an incidence rate of less than 6 per 100,000 per year in the European population. This monograph presents 198 rare cancers grouped in 14 major groups. Crude incidence rates were estimated as the number of all new cancers occurring in 2000-2010 divided by the overall population at risk, for males and females (also for gender-specific tumours).The proportion of rare cancers out of the total cancers (rare and common) by site was also calculated. Incidence rates by sex and age are reported. The expected number of new cases in 2015 in Italy was estimated assuming the incidence in Italy to be the same as in the AIRTUM area. One- and 5-year relative survival estimates of cases aged 0-99 years diagnosed between 2000 and 2008 in the AIRTUM database, and followed up to 31 December 2009, were calculated using complete cohort survival analysis. To estimate the observed prevalence in Italy, incidence and follow-up data from 11 CRs for the period 1992-2006 were used, with a prevalence index date of 1 January 2007. Observed prevalence in the general population was disentangled by time prior to the reference date (≤2 years, 2-5 years, ≤15 years). To calculate the complete prevalence proportion at 1 January 2007 in Italy, the 15-year observed prevalence was corrected by the completeness index, in order to account for those cancer survivors diagnosed before the cancer registry activity started. The completeness index by cancer and age was obtained by means of statistical regression models, using incidence and survival data available in the European RARECAREnet data. RESULTS: In total, 339,403 tumours were included in the incidence analysis. The annual incidence rate (IR) of all 198 rare cancers in the period 2000-2010 was 147 per 100,000 per year, corresponding to about 89,000 new diagnoses in Italy each year, accounting for 25% of all cancer. Five cancers, rare at European level, were not rare in Italy because their IR was higher than 6 per 100,000; these tumours were: diffuse large B-cell lymphoma and squamous cell carcinoma of larynx (whose IRs in Italy were 7 per 100,000), multiple myeloma (IR: 8 per 100,000), hepatocellular carcinoma (IR: 9 per 100,000) and carcinoma of thyroid gland (IR: 14 per 100,000). Among the remaining 193 rare cancers, more than two thirds (No. 139) had an annual IR <0.5 per 100,000, accounting for about 7,100 new cancers cases; for 25 cancer types, the IR ranged between 0.5 and 1 per 100,000, accounting for about 10,000 new diagnoses; while for 29 cancer types the IR was between 1 and 6 per 100,000, accounting for about 41,000 new cancer cases. Among all rare cancers diagnosed in Italy, 7% were rare haematological diseases (IR: 41 per 100,000), 18% were solid rare cancers. Among the latter, the rare epithelial tumours of the digestive system were the most common (23%, IR: 26 per 100,000), followed by epithelial tumours of head and neck (17%, IR: 19) and rare cancers of the female genital system (17%, IR: 17), endocrine tumours (13% including thyroid carcinomas and less than 1% with an IR of 0.4 excluding thyroid carcinomas), sarcomas (8%, IR: 9 per 100,000), central nervous system tumours and rare epithelial tumours of the thoracic cavity (5%with an IR equal to 6 and 5 per 100,000, respectively). The remaining (rare male genital tumours, IR: 4 per 100,000; tumours of eye, IR: 0.7 per 100,000; neuroendocrine tumours, IR: 4 per 100,000; embryonal tumours, IR: 0.4 per 100,000; rare skin tumours and malignant melanoma of mucosae, IR: 0.8 per 100,000) each constituted <4% of all solid rare cancers. Patients with rare cancers were on average younger than those with common cancers. Essentially, all childhood cancers were rare, while after age 40 years, the common cancers (breast, prostate, colon, rectum, and lung) became increasingly more frequent. For 254,821 rare cancers diagnosed in 2000-2008, 5-year RS was on average 55%, lower than the corresponding figures for patients with common cancers (68%). RS was lower for rare cancers than for common cancers at 1 year and continued to diverge up to 3 years, while the gap remained constant from 3 to 5 years after diagnosis. For rare and common cancers, survival decreased with increasing age. Five-year RS was similar and high for both rare and common cancers up to 54 years; it decreased with age, especially after 54 years, with the elderly (75+ years) having a 37% and 20% lower survival than those aged 55-64 years for rare and common cancers, respectively. We estimated that about 900,000 people were alive in Italy with a previous diagnosis of a rare cancer in 2010 (prevalence). The highest prevalence was observed for rare haematological diseases (278 per 100,000) and rare tumours of the female genital system (265 per 100,000). Very low prevalence (<10 prt 100,000) was observed for rare epithelial skin cancers, for rare epithelial tumours of the digestive system and rare epithelial tumours of the thoracic cavity. COMMENTS: One in four cancers cases diagnosed in Italy is a rare cancer, in agreement with estimates of 24% calculated in Europe overall. In Italy, the group of all rare cancers combined, include 5 cancer types with an IR>6 per 100,000 in Italy, in particular thyroid cancer (IR: 14 per 100,000).The exclusion of thyroid carcinoma from rare cancers reduces the proportion of them in Italy in 2010 to 22%. Differences in incidence across population can be due to the different distribution of risk factors (whether environmental, lifestyle, occupational, or genetic), heterogeneous diagnostic intensity activity, as well as different diagnostic capacity; moreover heterogeneity in accuracy of registration may determine some minor differences in the account of rare cancers. Rare cancers had worse prognosis than common cancers at 1, 3, and 5 years from diagnosis. Differences between rare and common cancers were small 1 year after diagnosis, but survival for rare cancers declined more markedly thereafter, consistent with the idea that treatments for rare cancers are less effective than those for common cancers. However, differences in stage at diagnosis could not be excluded, as 1- and 3-year RS for rare cancers was lower than the corresponding figures for common cancers. Moreover, rare cancers include many cancer entities with a bad prognosis (5-year RS <50%): cancer of head and neck, oesophagus, small intestine, ovary, brain, biliary tract, liver, pleura, multiple myeloma, acute myeloid and lymphatic leukaemia; in contrast, most common cancer cases are breast, prostate, and colorectal cancers, which have a good prognosis. The high prevalence observed for rare haematological diseases and rare tumours of the female genital system is due to their high incidence (the majority of haematological diseases are rare and gynaecological cancers added up to fairly high incidence rates) and relatively good prognosis. The low prevalence of rare epithelial tumours of the digestive system was due to the low survival rates of the majority of tumours included in this group (oesophagus, stomach, small intestine, pancreas, and liver), regardless of the high incidence rate of rare epithelial cancers of these sites. This AIRTUM study confirms that rare cancers are a major public health problem in Italy and provides quantitative estimations, for the first time in Italy, to a problem long known to exist. This monograph provides detailed epidemiologic indicators for almost 200 rare cancers, the majority of which (72%) are very rare (IR<0.5 per 100,000). These data are of major interest for different stakeholders. Health care planners can find useful information herein to properly plan and think of how to reorganise health care services. Researchers now have numbers to design clinical trials considering alternative study designs and statistical approaches. Population-based cancer registries with good quality data are the best source of information to describe the rare cancer burden in a population

Screening for distant metastases before salvage surgery in patients with recurrent head and neck squamous cell carcinoma: why and how?

International audienceABSTRACT   To evaluate the role of 18-fluoro-deoxy-d-glucose (FDG)-positron emission tomography (PET) scan for detecting distant metastases in the preoperative assessment of patients with recurrent head and neck squamous cell carcinoma (HNSCC).  Retrospective study.  University Teaching Hospital.  Thirty-seven consecutive patients who presented, between April 2008 and April 2010, a local and/or regional recurrence of head and neck squamous cell carcinoma after treatment with radio-chemotherapy were studied. The work-up included thoraco-abdominal computed tomography (CT), fluoro-deoxy-D-glucose-positron emission tomography scan and abdominal ultrasound. The imaging results, when positive, were compared to histology or cytology (conducted during targeted examinations, for example, fiberoptic oesophago-gastro-duodenal search, colonoscopy, bronchoscopy, liver biopsy) or targeted imaging examination (Abdominal MRI, sonography) combined with clinical follow-up. All patients were followed-up for at least 6 months. Positron emission tomography performances were then analysed and compared with those of conventional imaging for detecting distant metastases.  Among the 37 patients, 9 (24%) had visceral metastases. The sensitivity, specificity, positive predictive value and negative predictive value for detecting metastasis or second primary were, respectively, 100%, 94%, 86% and 100% for CT and 92%, 87%, 74% and 97% for positron emission tomography. Computed tomography and positron emission tomography were strictly concordant in 32/37 (86%) of cases. No false-negative result was found for CT, while we found one case of false-negative positron emission tomography. The number of false-positive results was two for CT and four for positron emission tomography.  From our study, positron emission tomography does not appear to offer a first-choice technique for the detection of metastases before salvage surgery as CT detected all lesions visible on positron emission tomography

Coarticulation and VOT in an Italian child from 18 to 48 months of age

This study brings new data to two understudied topics in Italian child language development: VOT and anticipatory C-V coarticulation. One female subject was recorded every three months from 18 to 48 months, while interacting with the clinician in front of some toys, repeating several times each bisyllabic pseudo-word beginning with voiceless and voiced stops. The acoustic signals were annotated using Praat and scripts were created for the automatic extraction of VOT and F2 values (Hz) for the Locus of Equations methods. RESULTS: Voiced stops appeared more difficult to produce than voiceless stops, but voicing contrast was finally achieved from 30 months of age. The degree of coarticulation increased with age, but at 48 months bilabials and alveolars were still less coarticulated than in adults

How do French nuclear physicians deal with ethical aspects of ă communication results to patients after PET performed for oncological ă indications? A national french survey

Annual Congress of the European-Association-of-Nuclear-Medicine (EANM), ă Barcelona, SPAIN, OCT 15-19, 2016International audienceno abstrac

System capacity evaluation of DVB-S2X based medium earth orbit satellite network operating at Ka band

In this paper, the performance of a Medium Earth Orbit (MEO) satellite trunking system operating in Ka-band in terms of capacity statistics is studied, taking into account sophisticated propagation and physical layer tools. First, the satellite system model is presented and some general considerations of the MEO constellation and network are given. Afterwards, a tool for generating atmospheric attenuation time series induced on links operating at Ka-band with time dependent elevation angle, such as Earth-MEO links, is described. The tool is derived modifying the atmospheric attenuation time series generators for links with fixed elevation angles. Moreover, an optimized in terms of spectral efficiency physical layer design is provided, employing also some higher constellation modulations from the recent DVB-S2X standard. Finally, considering Adaptive Coding and Modulation which allows providing the instantaneously highest data rate for a given link budget, we evaluate the total system capacity provided by the MEO constellation. In the numerical results section, capacity results are presented for a variety of ground stations located in different climatic areas

Cardiac CT or MRI in pediatric practice: Which one to choose?

International audienceThe different factors involved in the choice of the best cardiovascular ă imaging examination for pediatric patients are justification, radiation ă protection, sedation, resolutions (spatial and contrast), morphology or ă function, intervention and contrast enhancement. Computed tomography is ă preferable for all coronary artery conditions, any arterial or venous ă abnormalities in newborns and infants and in the preoperative assessment ă for tetralogy of Fallot. Magnetic resonance imaging is used for any ă tumoral or functional assessment, cardiomyopathy or arrhythmia or if the ă child's participation and/or size of the structures being examined ă allows using this technique. (C) 2016 Editions francaises de radiologie. ă Published by Elsevier Masson SAS. All rights reserved

Comment les médecins nucléaires prennent-ils en compte les aspects éthiques dans la communication des résultats aux patients réalisant une TEP d'indication oncologique? Une étude nationale française

Objectif: There are no guidelines regarding the content of information to be delivered in nuclear medicine. This study aimed at describing practices of French nuclear medicine physicians concerning results communication to patients undergoing PETs for oncological indications. Methodes: A survey was performed among French nuclear medicine physicians using an electronic questionnaire. Resultats: The study included 250 nuclear medicine physicians. Their practices regarding communication of PET results to patients were heterogeneous: (1) pre- and/or post-PET consultations were systematic for 56%, adapted on a case-to-case basis for 35%, and never performed for 9% of respondents; (2) oral communication of results was systematic for 13%, adapted on a case-to-case basis for 63%, and never performed for 24% of respondents. Working in a private center, presence of post-PET consultations, being older and more years of experience were significantly associated with more oral communication of results. Finally, 80% of physicians admit that current recommendations are not clear enough. Conclusion: The heterogeneity of practices among French nuclear medicine physicians concerning communication of PET results to patients probably results from lack of clear recommendations. An inter-collegial, ethical, and multi-disciplinary reflection is needed to better guide nuclear medicine physicians in relation to the communication of results to patients suffering from serious disease.SCOPUS: sh.jinfo:eu-repo/semantics/publishe