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Introduction:This study presents the incidence of gastrointestinal stromal tumors (GISTs) in an Italian region of over 4 million inhabitants monitored for 10 years and is the largest incidence study of this type of cancer conducted so far in Italy.Methods:In order to ensure the registration of all GISTs, including those with nonmalignant behavior, a cancer list was integrated with the cases found through an ad hoc data mining process that covered all the pathologic reports of Puglia. Case distributions by sex, age groups, site, and prognostic groups according to Miettinem and Lasota classification and crude and age-standardized incidence rates were produced.Results:In the 10-year period 2006 to 2015, 708 cases of GIST were recorded in Puglia. The average crude incidence rate was 1.7 per 100,000 person-years and the age-standardized incidence rate, using 2013 European standard population, was 1.8 per 100,000 person-years (95% confidence interval [CI], 1.6–1.9). Incidence was higher in men than in women: crude incidence rate was 2.0 per 100,000 person-years and age-standardized incidence rate 2.2 per 100,000 person-years (95% CI, 2.0–2.4) in men and 1.5 per 100,000 person-years and 1.4 per 100,000 person-years (95% CI, 1.2–1.6) in women.Discussion:Our incidence rates are comparable with those of other international studies and they are located in the medium to high end of the range. The comparisons are affected by a different capacity of the cancer registries to intercept and record GISTs with nonmalignant behavior. Distribution of cases for sex, age groups, sites, and prognostic risk groups are consistent with previous results.

The Cancer Registry of Puglia (RTP) was instituted in 2008 as a regional population-based cancer registry. It consists of six sections (Foggia, Barletta-Andria-Tran, Bari, Brindisi, Lecce, and Taranto) and covers more than 4 000 000 inhabitants. At present, four of six sections have received accreditation by AIRTUM (53% of regional population). To point out possible regional geographic variability in cancer incidence and also to support health services planning, we developed an original estimation method to ensure a complete territorial coverage. Incidence data of the four accredited RTP sections for the shared incidence period 2006–2008, the 2001–2009 hospitalization regional data, and 2006–2009 mortality data were considered. To take into account specific health features of different provinces, we performed an estimate of cancer incidence rates of nonaccredited sections using a combination of accredited sections rates and a factor that combines mortality and hospitalization ratios available for all the sections. Finally, we validated the method and we applied it to estimate regional cancer rates as the population-weighted average of accredited sections and nonaccredited sections adjusted rates. The validation process shows that estimated rates are close to real incidence data. The most frequent neoplasms in Apulia are breast (direct standardized rates 96.8 per 100 000 inhabitants), colon–rectum (36.6), and thyroid cancer (25.3) in women and prostate (70.2), lung (68.4), and colon–rectum cancer (52.2) in men. This method could be useful to assess the cancer incidence when complete cancer registration data are not available, but hospitalization, mortality, and neighbouring incidence data are available.

We conducted a population-based study of peripheral lymphomas (PL) that had been diagnosed between 1997 and 2003 in the province of Modena, Italy, with the aim of providing updated incidence, clinical and survival data for these cancers. We evaluated the incidence patterns and time trends of 1582 cases of PL that had been reclassified according to the WHO classification of hematological malignancies. Data regarding clinical characteristics, treatment and outcome were also collected for each case. The World Age-Standardized Rate (ASR) was calculated as 13.4, 2.2 and 3.4 per 100,000 people for B-cell non-Hodgkin's lymphoma (NHL), T-cell NHL and Hodgkin's Lymphoma (HL), respectively, with an increase of 1.62% per year during the study period. The lymphoma subtype showing the highest incidence was found to be diffuse large B-cell lymphoma (DLBCL) with an ASR of 4.8. Compared with reports from other western countries, our series is characterized by a higher incidence of HL and indolent B-NHL in general, and of CLL/SLL (ASR = 3.3) and marginal zone NHL (ASR = 1.5), in particular, and also by a lower incidence of FL (ASR = 2). After a median follow-up of 54 months, the 5-year relative survival for the whole series was found to be 70% with a statistically significant improvement for cases diagnosed during 2002-2003 (from 66 to 74%; p = 0.03). Survival improvement within the study period was also evident for patients with DLBCL, HL and T-NHL. Our study provides a comprehensive description of both the epidemiological and clinical features of PL cases in Modena and our data also reflect the major advances in the curability of some histological subtypes of this disease. The usefulness of a population-based approach to better characterizing different lymphoma subtypes is also demonstrated.

Background Few studies have been carried out to date that have addressed the epidemiology of extranodal marginal zone lymphomas (EN-MZLs). Patients and methods We carried out a population-based study to investigate incidence rates (IRs) and time trends of EN-MZL diagnosed in the province of Modena (Italy) from 1997 to 2007. Results One hundred and sixty-five cases were identified from the Modena Cancer Registry that corresponded to an age-standardized IR of 2.3 cases per 100 000. A bimodal distribution of age was shown with the group of young patients mostly represented by males with cutaneous lymphoma. No time trends were observed for the IR; the incidence of gastric mucosa-associated lymphoid tissue (g-MALT) lymphomas (N = 51) markedly declined during the study period, dropping from 1.4 in 1997 to 0.2 in 2002 and then remaining stable until 2007; the calculated annual percent change for g-MALT was -17.0% (95% confidence interval -26.6% to -6.2%). We also observed a significant decrease in the rate of g-MALT associated with Helicobacter pylori (HP) infection from 61% to 17% of patients diagnosed before and after 2002 (P = 0.007; P for trend = 0.016). Conclusion This population-based study provides new insights into recent changes in the epidemiology of EN-MZL, mainly represented by the sharp reduced incidence of HP-positive g-MALT lymphomas.

The aim of this study was to provide further quantitative data on the risk of second nonmelanoma cancers in patients with cutaneous malignant melanoma (CMM). A cohort of 14 560 population-based patients from the Italian Network of Cancer Registries incident during 1985-2002 were included and followed up for further incident cases and vital status. Standardized incidence ratios (SIR) were used to compare the number of observed second cancers with expected cancers. In a total of 69 581 person-years, 1020 second cancers were registered, of which 804.6 were expected (SIR=1.27; 95% confidence interval 1.19-1.35). The risk was similar for males and females, (SIR=1.27 and 1.26, respectively). The risk was slightly higher among younger (

Many empirical studies have proven the usefulness of period analysis in providing more up-to-date estimates of cancer patient survival than cohort-based methods. The aim of this paper is to provide a non-empirical evaluation of several survival approaches over a comprehensive range of scenarios using computer simulations. The simulation model included the following input parameters: number of annual patients, length of survival calculation, number of years of diagnosis, prognosis of cancer, follow-up period, and two additional parameters for modeling assumption on incidence and survival trends. The current study also introduced alternative cohort- and period-based approaches in addition to more traditional methods. Simulations showed that the choice of an appropriate survival approach is strongly dependent on the given scenario: period analysis was effective only for a limited number of circumstances, while alternative approaches appeared to be suitable for more realistic situations, when the follow-up period is different from the incidence period. The results of simulations could be useful for a quick identification of the most appropriate approach when estimating up-to-date cancer survival rates.

Background Because in the past Chronic Myeloproliferative Disorders (CMPD) were not considered to be malignant conditions, cancer registries rarely recorded data on these diseases. Thus, information on incidence and outcome of CMPD in the population is limited. The aim of the present study was to better define epidemiological data of CMPD by examining all cases identified by the Modena Cancer Registry (MCR). Materials and methods We considered all cases of CMPD diagnosed in the Province of Modena (population 633.993 at 2001 Census). Cases, except Chronic Myeloid Leukemia, diagnosed from 1997 to 2005, were identified using the MCR database and the archival files of the centralized hemolymphopathological laboratory at Modena Cancer Centre according to ICD-O-3 codes 9950, 9960–64. Death certificate, cytology and histology report, both local and national reports of hospital admission, ICD-9 code reported in medical records were used as sources for identifying new CMPD cases and their outcome. All cases were checked and validated by a hematologist (A.B.) and a pathologist (G.B.) by a review of the original pathology report. Uniform diagnostic criteria were adopted, because the large majority of bone marrow aspirate and biopsy were examined by the same pathologist (G.B.). Clinical and follow-up data were retrieved by active search of discharge letters, review of hospital records and interview of general practitioners. Information on vital status was achieved from official population registries. Age standardized rates (ASR) were calculated according to the World Standard population. The dates of diagnosis and death or the closing date of study (December 2006) were used to estimate survival. Observed survival and relative survival were calculated according to Kaplan-Meier method and the Hakulinen approach, respectively. Results According to the 2001 World Health Organization (WHO) classification, a total of 380 cases of CMPD were identified. There were 155 Essential Thrombocythemia (ET) (41% of all CMPD), 114 Policythemia Vera (PV) (30%), 75 Idiopathic Myelofibrosis (20%), 2 Hypereosinophilic Syndrome/Chronic Eosinophilic Leukaemia (0.5%), 1 Chronic Neutrophilic Leukemia (0.3%) and 31 CMPD not otherwise specified (8%). The ASR of CMPD was 3.2/100,000 varying slightly (from 2.5 to 4.1/100,000) during the study period (p = 0.11); the crude incidence rate was 6.6/100,000. Median age at diagnosis was 69 years. No statistically significant differences were observed between sex regarding incidence and age at diagnosis. Overall relative survival was 97%, 89% and 88% at 1, 3 and 5 years after diagnosis, respectively. Analyzing CMPD, we observed a better survival for ET and PV in comparison with other subtypes (p = 0.01). Conclusions To our knowledge, this study is the first in Italy providing information on the incidence and outcome of CMPD using population-based data. Our results confirm that the risk of developing CMPD increases with age. The incidence of CMPD was substantially stable during the study period. Overall survival patterns reflect the well known chronic course of these diseases. As expected, we observed important differences in overall survival by WHO subtypes. We believe that the availability of precise epidemiological data, in particular those regarding outcome could help clinicians in choosing the most appropriate cost-effective treatments.

Background The reduced mortality rate from breast carcinoma among women offered screening mammography is demonstrated after 15–20 years of follow-up. However, the assessment of 5-year overall and event-free survival could represent an earlier measure of the efficacy of mammography screening program (MSP). Methods All cases of breast cancer diagnosed in the Province of Modena between years 1996 and 2000 in women aged 50 to 69 years, were identified through the Modena Cancer Registry (MCR). Stage of disease and treatment information were obtained from clinical records. All the events occurring up to June 30, 2003 were retrieved by experienced monitors. Five-year overall and event-free survival were the principal end-points of the study. Results During a 5-year period, 587 primary breast cancers were detected by the MSP and 471 primary breast cancers were diagnosed out of the MSP. The screen-detected breast cancers were smaller, more likely node negative, with low histological grade, low proliferative activity and positive receptors status. Furthermore, the breast cancer diagnosed through the MSP more frequently received a conservative surgery. The 5-year survival rate was 94% in the screen-detected group, versus 84% in the other group (p = 0.0001). The rate of 5-year event-free survival was 89% and 75% for the MSP participants and not participants, respectively (p = 0.0001). Conclusions Our data confirm a favourable outcome of screen-detected breast cancers in terms of five-year overall and event-free survival, which reflect the good quality assurance parameters of the MSP. Finally, a cancer registry should be implemented in every area covered by screening programs.

Background As in the past Myelodisplastic Syndromes (MDS) were considered preneoplastic conditions, rarely data on these diseases were collected by cancer registries. Thus, there are few well documented population-based studies on the incidence and outcome of MDS. The aim of this study was to collect epidemiological data and clinical characteristics of MDS by studying all cases identified by the Modena Cancer Registry (MCR). Materials and methods We examined all cases of MDS diagnosed in the Province of Modena (population 633.993 at 2001 Census). MDS from 1997 to 2005 were identified using the MCR database and the archival files of the centralized hemolymphopathological laboratory at Modena Cancer Centre according to ICD-O-3 codes 9980,9982–87,9989. Death certificate, cytology and histology report, both local and national reports of Hospital admission, ICD-9 code reported in medical records were used as sources for identifying new MDS cases and their outcome. After collection, all cases were checked and validated by a hematologist (A.B.) and a pathologist (G.B.) by a review of the original pathology report. The large majority of bone marrow aspirate and biopsy were examined by the same pathologist (G.B.) making diagnostic criteria uniform. Clinical and follow-up data were retrieved by active search of discharge letters, review of hospital records, and interview of general practitioners. Information on vital status was achieved from official population registries. Age standardized rates (ASR) were calculated according to the World Standard population (Doll et al, 1966). The dates of diagnosis and death or the closing date of study (December 2006) were used to estimate survival. Observed survival and relative survival were calculated according to Kaplan-Meier method and the Hakulinen approach, respectively. Results A total of 205 cases of MDS were identified. The ASR of MDS was 1.2/100,000 varying slightly (from 0.9 to 1.5/100,000; p > 0.05) during the study period, and the crude incidence rate was 3.6/100,000. Median age at diagnosis was 75 years for men and 78 for women. Overall, 58% of patients aged more than 75 years, while only 1% were less than 45 years old. According to French, American and British (FAB) classification there were 35 cases (17% of all MDS) of Refractory Anemia (RA), 51 (25%) of RA with ringed sideroblasts, 73 cases (36%) of RA with excess of blasts (RAEB), 11 (5%) of RAEB in transformation, 31 (15%) of MDS not otherwise specified and 4 (2%) of other MDS. Overall the prognosis of MDS was poor, although we found statistically significant differences by clinical subtypes. In our MDS population the relative survival was 68%, 36% and 26% at 1, 3 and 5 years, respectively. Conclusions To our knowledge, this study is the first in Italy providing information on the incidence and outcome of MDS using population-based data. Our results confirm that the risk of developing MDS increases with age for both men and women. The incidence of MDS was substantially stable during the study period. Overall survival was poor reflecting the aggressiveness of these diseases and the advanced age of patients at time of diagnosis. As expected, we observed important differences in overall survival by FAB subtypes. In the last few years, innovative treatments for MDS are emerging and we believe that the availability of precise epidemiological data could help clinicians in choosing the most appropriate treatment.

The incidence of lymphomas has increased in many parts of the world in recent decades. We conducted a population-based study of peripheral lymphoma (PL) in the province of Modena (Italy). According to the international classification for lymphomas (WHO/ICD-O-3) we evaluated incidence patterns and time trends of 1,582 cases of PLs diagnosed between 1997–2003, also providing details of clinical characteristics, treatment and outcome of different entities. World Age Standardized Rates (ASR) varied substantially among lymphoid neoplasm subtypes. Overall, ASR (per 100,000) was 13.4 for B-cell lymphomas (16.5 and 10.6 for males and females respectively), 2.2 for T-cell lymphomas (3.1 and 1.5) and 3.4 for Hodgkin lymphoma. Among B-NHL the highest ASR was observed for Diffuse large B-cell lymphoma (DLBCL, 4.8) and Small lymphocytic lymphoma (CLL/SLL, 3.3). Among T-cell lymphomas, ASR was 1.4 for Mycosis fungoides/Sezary syndrome (MF/SS) and 0.4 for both Peripheral T-cell lymphoma (PTCL) and Anaplastic large cell lymphoma (ALCL). The overall annual percent change (APC) of the World ASR was 1.62 (C.I.95%: −4.32; 7.93). Extranodal (EN) involvement was observed in 49% of cases; a diagnosis of Primary EN lymphoma (PENL) was confirmed in 27.8% of cases. In addition to MF/SS, the most frequently reported PENL were marginal zone lymphomas (MZL) and DLBCL. The documented five years Overall Survival (OS) for the whole series was 61.9%, being 61.7%, 55.3% and 83% for B-NHL, T-NHL and HL, respectively. These data confirm an overall improvement when compared with available historical controls for the main European countries. In addition, using period analysis we could estimate more up-to-date survival data within our studied series (all patients vs those diagnosed after 2002): survival of patients with MZL, DLBCL and HL strongly improved during the study period. Among different lymphoma subtypes, patients with Hairy cell leukemia (HCL), HL and MF/SS had the best survival with 5-year OS of 94%, 83% and 82%, respectively. In particular, for HCL and MF/SS the analysis of the Relative Survival suggested that there was no substantial difference in survival compared with healthy population. Our study provides a comprehensive description of both epidemiological and clinical features of PL in the province of Modena, recognizing in a population-based approach, major advances in the curability of some histological subtypes.