Laryngeal cancer survival in Zaragoza (Spain): a population-based study

Objective  

This article aims to study larynx cancer survival from the Population-Based Cancer Registry of Zaragoza, Spain, for the period 1978–2002.     

Population-based incidence and survival of gastrointestinal stromal tumours (GIST) in Girona, Spain

BackgroundGastrointestinal stromal tumours (GIST) are rare malignancies characterised by their association with KIT oncogene mutations. Until now, population-based reports of the incidence or survival of kit-confirmed GIST have been rare, and none have originated in Southern Europe.Materials and methodsWe used the Girona Cancer Registry to identify malignant mesenchymal tumours of the digestive tract between 1994 and 2001, and performed c-kit testing in the tumour samples. Age-adjusted incidence rates and survival rates were calculated, and they were also analysed by sex and NIH risk categories.ResultsForty-six cases were categorised as GIST. Fifty percent were localised in the stomach, 43.5% in small intestine, 4.3% in the omentum, and 2.2% in colon. Thirty-seven percent were classified as high risk of an aggressive behaviour, 30.4% as intermediate risk and 32.6% as low or very low risk. Only one patient received treatment with imatinib mesilate. The annual incidence by 100,000 inhabitants in crude rate, European age-standardised rate and world age-standardised rate was, respectively, 1.09, 0.90 and 0.65 cases. The relative 5-year survival rate was 74.7% for the entire cohort, and it was markedly lower in the high-risk cases (20.3%).ConclusionsWe report the first population-based study of GIST incidence and survival in Southern Europe. The incidence rate is low and comparable with that of cancer registries from Northern Europe. Survival was favourable in our pre-imatinib population although it was low in high risk cases. Prognostic discrimination of the cases with intermediate, low, or very low risk is inadequate, and these categories should be considered jointly in the future. Our results will help researchers in establishing baseline values against which they can compare, in the future, the impact of imatinib and other Kit tyrosine inhibitors on survival.     

Epidemiology and survival of Merkel cell carcinoma in the Netherlands. A population-based study of 808 cases in 1993-2007

Background

Merkel cell carcinoma (MCC) is a rare and highly malignant neuroendocrine tumour, predominantly located on sun-exposed areas of the skin. The aim of this study was to evaluate data in the Netherlands concerning incidence, stage, age, sex, location, treatment and survival.

Methods

Using nationwide data from the Netherlands Cancer Registry from 1993 to 2007, we compared 808 MCCs with European data and the US Surveillance, Epidemiology and End Results (SEER) Program.

Results

The annual age standardised incidence rate per million of MCC increased from 1.7 in 1993-1997 to 3.5 in 2003-2007. Median age at diagnosis was 76 years. The most common location was the head and neck. Three quarters of patients had localised disease, 16% regional and 6% distant metastasis. Surgery was performed in 89% of patients, with adjuvant radiotherapy in 26% of them. One-, five- and ten-year relative survival was 85%, 62% and 47%, respectively. Negative predictive factors for the risk of death were male sex, increasing T-stage, regional and distant metastasis and no treatment. Survival after combined surgery and radiotherapy was borderline significantly better than surgery alone (HR 0.82, p = 0.09). Our results are comparable to SEER data except for the ratio localised/regional disease. We observed less regional cases (16% versus 31%); while ten-year survival of localised cases was lower (51% versus 71%).

Conclusions

MCC incidence rates have doubled in the Netherlands over the period 1993-2007. The relatively high number of localised cases and their relatively low survival as compared to SEER data suggest that a substantial proportion is undertreated.     

Risk and survival of chronic myeloid leukemia after breast cancer: A population-based study

BackgroundWe aimed to investigate the risk and survival of chronic myeloid leukemia (CML) after breast cancer (BC) diagnosis.MethodsWe used the Surveillance, Epidemiology, and End Results ‘SEER’ database. Females, diagnosed with BC between 1992 and 2014, were selected and followed for the development of CML after a 6-month latency period from BC diagnosis. We used the Multiple Primary Standardized Incidence Ratios session of the SEER*Stat software (version 8.3.4) to calculate the Observed/Expected (O/E) ratios with 95% confidence intervals (CI). To calculate the overall survival, we performed an unadjusted Kaplan-Meier analysis using the SPSS software.ResultsWe included 474,866 females with BC, of which 178 were later diagnosed with CML. We found the risk of CML to increase significantly after BC diagnosis (O/E = 1.26, 95% CI: 1.08-1.45) and the risk was specifically higher within the first 5 years of diagnosis (O/E = 1.45, 95% CI: 1.16-1.8). When the risk was stratified by cancer stage, localized BC was associated with a significant increase in CML risk within 5 years of diagnosis (O/E = 1.4, 95% CI: 1.06-1.82), while regional BC was associated with a significant increase in CML risk after more than 5 years of diagnosis (O/E = 1.59, 95% CI: 1.09-2.25). Moreover, radiotherapy, chemotherapy, and presence of hormonal receptors were associated with a significant increase in CML risk in BC patients. The median overall survival of CML after BC was 28 months.ConclusionBreast cancer patients have an increased risk of developing CML and further investigation is required to establish the causes of this finding.     

Temporal Trends of Cancer Incidence in Vietnam, 1993-2007

Purpose: There is a lack of an overview of overall and site-specific cancer incidence time trends in Vietnam,especially for the period after the year 2000. This paper aims at describing the development of cancer incidencefor some cancer sites during 1993-2007. Methods: The Age Standardized Rate (ASR) of cancer incidence datafrom population based cancer registries of Hanoi, Ho Chi Minh and Cantho cities were used to analyze temporarytrends of cancer incidence by site, age and sex group. Results: The ASR of cancer incidence increased from151.1/105 in the period 1993-1998 to 160.0/105 in the period 2006-2007 for males and from 106.8/105 to 143.9/105for females. By age, the highest ASR was found in the group of 75+ years in males and between 70-74 yearsin females, with ASRs of 1,109/105 and 619/105, respectively (2006-2007). Lung remains the most frequent site,followed by stomach and liver in males. In females, the most commonly affected site has shifted from cervixuteri in 1993-1998 to breast in recent years, followed by stomach and lung. Increasing trends were observed inincidence rates of 21 out of 34 cancer sites in males and 27 out of 35 cancer sites in females. Conclusion: Cancerincidences in general have continuously increased during 1993-2007. More efforts should be concentrated ondeveloping and implementing tobacco-related cancer prevention interventions.     

Risk assessment in chronic myelomonocytic leukemia (CMML)

The clinical course of chronic myelomonocytic leukemia (CMML) is extremely variable, and disease progression can occur at any time from diagnosis. Median survival is about 20 months. About 20% of patients develop acute myeloid leukaemia (AML). Multivariate analyses performed by several groups showed that elevated medullary blast count, low haemoglobin, elevated serum lactate dehydrogenase (LDH), and perhaps an increased lymphocyte count, are the most important independent prognostic parameters, whereas karyotype analysis was not consistently shown to yield additional prognostic information. Applying different scoring systems to 288 CMML patients included in the Düsseldorf MDS Registry, we found that the International Prognostic Scoring System (IPSS) was not useful for defining risk groups in CMML, while the Spanish Score, the modified Bournemouth Score, the Düsseldorf Score, and probably the MDAP Score, identified patient groups differing significantly in survival. These scores should therefore be employed for clinical decision making and for risk stratification in the context of clinical trials.     

Incidence and survival of oesophageal and gastric cancer in England between 1998 and 2007, a population-based study

Background  

Major changes in the incidence of oesophageal and gastric cancers have been reported internationally. This study describes recent trends in incidence and survival of subgroups of oesophageal and gastric cancer in England between 1998 and 2007 and considers the implications for cancer services and policy.     

Breast cancer in Norway 1970-1993: a population-based study on incidence, mortality and survival.

The incidence, mortality and survival of breast cancer patients from 1970 to 1993 were studied using data from the Cancer Registry of Norway. The age-adjusted incidence rate increased from 62.0 to 76.9 per 100,000 person-years during the period, and more than 2000 cases are now registered annually. The increase tends to be highest in the age group below 40 years. The increase is mainly found in cases with localized tumours at the time of diagnosis. The mortality rate has been almost unchanged in the period; the age adjusted mortality rate is 27.0 per 100,000 person-years at the end of the study period. The 5-year overall survival has increased among cases with axillary lymph node metastases at the time of diagnosis; the other stages show only little improvement.     

Incidence and survival of brain tumors: a population-based study

Since 1976 the Lombardy Cancer Registry (RTL) has recorded all malignant tumors and the benign tumors of the nervous system, bladder and liver occurring in the Varese province. The aims of this report are: to describe age-specific incidence rates of the different oncotypes of primary nervous system tumors (PNSTs) and to analyze the duration of survival of the patients according to the histotype of the neoplasm. From 1976 to 1981, the RTL recorded 498 PNST: 213 tumors of glial origin, 132 tumors of mesodermal tissues, 15 tumors of multipotential cell origin, 37 tumors of nerve roots, 8 of vascular origin, and 54 PNST in which the histotype was not specified. For tumors of glial origin, the 60-69 year age class showed the highest incidence rate: 16 per 100,000/year. The incidence rate of tumors of mesodermal tissues reached the maximum value of 7.9 per 100,000/year in the age group of 75 years and over. Tumors of multipotential cell origin had a maximum incidence of 2.2 in the 60-69 year age class. The probability of survival at 5 years after diagnosis was computed by the method of Kaplan and Meier, and the log rank procedure was used to test statistical differences among survivors. Tumors of glial origin, excluding ependymomas and papillomas of choroid plexus, had a 5-year survival probability of 14%. Age (categorized in less than or equal to 50 and greater than 50 years) and histologic grade (grades I + II and grades III + IV) were analyzed as prognostic factors for tumors of glial origin: a younger age was the most predictive prognostic factor in grade I + II tumors (probability of survival = 53% in the group less than or equal to 50 years vs 0 in the group greater than 50 years; p less than 0.005). In the less differentiated tumors there were no differences between the two age groups. Patients with tumors of mesodermal tissues had a 5-year probability of survival of 83%. Subjects younger than 50 years had a probability of survival of 95%, whereas older patients had a probability of 79%. For the other oncotypes, the 5-year survival probability was: 50% for tumors of multipotential cell origin, 63% for ependymomas and papillomas of the choroid plexus, 64% for tumors of vascular derivation, and 21% for PNST with not specified histotype.     

Prognostic factors and survival in chronic myelomonocytic leukaemia (CMML)

Ninety-seven cases of chronic myelomonocytic leukaemia (CMML) were examined retrospectively for survival and possible prognostic factors including age, total white cell count, peripheral blood and bone marrow monocyte counts, % double esterase (DE) positive cells in bone marrow and serum lysozyme. Age, absolute monocyte counts and serum lysozyme proved to be significant independent prognostic indicators but Cox model analyses showed serum lysozyme to be the most important factor whether taken as a continuous or discrete (two groups) variable. Twelve cases of second malignancy were found, including 2 cases of multiple myeloma, but this was not significantly greater than expected when compared with an age and sex matched group.     

Adrenocortical carcinoma: A population-based study on incidence and survival in the Netherlands since 1993

BackgroundThe reported annual incidence of adrenocortical carcinoma (ACC) is 0.5–2.0 cases per million individuals. Updated population-based studies on incidence are lacking. The aim of this nationwide survey was to describe the incidence and survival rates of ACC in the Netherlands. Secondary objectives were to evaluate changes in both survival rates and the number of patients undergoing surgery.MethodsAll ACC patients registered in the Netherlands Cancer Registry (NCR) between 1993 and 2010 were included. Data on demographics, stage of disease, primary treatment modality and survival were evaluated.ResultsIncluded were 359 patients, 196 of whom were female (55%). Median age at diagnosis was 56 years (range 1–91). The 5-year age-standardised incidence rate decreased from 1.3 to 1.0 per one million person-years. Median survival for patients with stage I–II, stage III and stage IV disease was 159 months (95% confidence interval (CI) 93–225 months), 26 months (95% CI: 4–48 months) and 5 months (95% CI: 2–7 months), respectively (P < 0.001). Improvement in survival was not observed, as reflected by the lack of association between survival and time of diagnosis. The percentage of patients receiving treatment within 6 months after diagnosis increased significantly from 76% in 1993–1998 to 88% in 2005–2010 (P = 0.047), mainly due to an increase in surgery for stage III–IV patients.ConclusionThese nationwide data provide an up-to-date survey of the epidemiology of ACC in the Netherlands. A trend towards a decreasing overall incidence rate was observed. Survival rates did not change during this period despite an increased number of surgical procedures.     

Cutaneous lymphoma in Israel, 1985-1993: a population-based incidence study.

The incidence rate of cutaneous lymphomas (CL) [including mycosis fungoides (MF) and non-mycosis fungoides (non-MF)] for the period 1985-93 in Israel was determined using data from the population-based Cancer Registry supplemented by a field survey that covered approximately 80% of lymphoma cases. After the field survey, corrected rates were 49% and 24% higher for MF and non-MF respectively (37% for CL overall). The age-adjusted incidence rates per 100,000 were 1.18 and 0.63 for Jewish men and women respectively. MF rates (0.77 in men and 0.35 in women) were higher than non-MF (0.41 and 0.28 respectively). Rates of CL were significantly lower in non-Jews. There were no significant differences in incidence among Jewish ethnic subgroups. However, the lack of variability in the incidence of these neoplasms among subpopulations is in contrast with findings for cutaneous malignant melanoma; the observed high rates of CL could, nonetheless, be consistent with the sunlight exposure hypothesis, operating perhaps through a different mechanism.     

Trends in survival of leukemia among children,adolescents, and young adults: A population-based study in Osaka,Japan

This study focused on children as well as adolescents and young adults (AYAs) and aimed to examine trends in survival of leukemia over time using population-based cancer registry data from Osaka, Japan. The study subjects comprised 2254 children (0-14 years) and 2,905 AYAs (15-39 years) who were diagnosed with leukemia during 1975-2011. Leukemia was divided into four types: acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), chronic myeloid leukemia (CML), and other leukemias. We analyzed 5-year overall survival probability (5y-OS), using the Kaplan-Meier method and expressed time trends using the joinpoint regression model. For recently diagnosed (2006-2011) patients, a Cox proportional hazards model was applied to determine predictors of 5y-OS, using age group, gender, and treatment hospital as covariates. Over the 37-year period, 5y-OS greatly improved among both children and AYAs, for each leukemia type. Among AYAs, 5y-OS of ALL improved, especially after 2000 (65% in 2006-2011), when the pediatric regimen was introduced but was still lower than that among children (87% in 2006-2011, P < .001). Survival improvement was most remarkable in CML, and its 5y-OS was over 90% among both children and AYAs after the introduction of molecularly targeted therapy with tyrosine kinase inhibitors. Among patients with recently diagnosed AML, the risk of death was significantly higher for patients treated at nondesignated hospitals than those treated at designated cancer care hospitals. The changes in survival improvement coincided with the introduction of treatment regimens or molecularly targeted therapies. Patient centralization might be one option which would improve survival.     

Incidence and survival of malignant bone sarcomas in England 1979-2007

Primary malignant bone sarcomas (MBS) are rare and there are few studies examining their incidence and outcome. Here, the incidence and survival of all subtypes of MBS registered in England between 1979 and 2007 were analysed from patient registry data held by the National Cancer Intelligence Network (NCIN). Over 11,002 new cases of MBS were registered, an average of 379 per year. There was no change in incidence demonstrated over the study period (p = 0.08). Although a peak incidence is observed in adolescence, approximately half of MBS are diagnosed in patients over 50 years. An improvement in outcome of MBS was observed between those patients registered from 1979 to 1983 and 1983 to 1987 (p < 0.0001), but there has been no improvement since. In the most recent period studied (patients diagnosed 1998-2002) 5-year survival was 55% in Ewing sarcoma, 70% in chondrosarcoma, 56% in chordoma and 43% in osteosarcoma. Patients diagnosed with osteosarcoma over the age of 40 years or with a non-extremity tumour have a significantly inferior outcome; 22% 5-year survival >40 years compared with 53% <40 years (p < 0.0001) and 16% non-extremity tumour compared to 48% extremity tumour (p < 0.0001). This population-based study has allowed us to confidently define the English incidence and survival rates of both the commoner bone tumours such as osteosarcoma, and rarer entities such as chordoma as well as groups with inferior outcome. The lack of significant improvement over recent decades for these diseases is cause for concern and further research.     

Trends in incidence,treatment and survival of gastric adenocarcinoma between 1990 and 2007: A population-based study in the Netherlands

BackgroundSurvival of gastric cancer in the Western world remains poor. We conducted a retrospective population-based study to evaluate trends in incidence, treatment and outcome of gastric adenocarcinoma.MethodsAll patients diagnosed with gastric adenocarcinoma during 1990–2007 in the Dutch Eindhoven Cancer Registry area were included (n = 4797). Trend analyses were conducted for incidence, mortality, tumour and patient characteristics, treatment and crude overall survival, according to tumour location (cardia versus non-cardia). Temporal changes in the odds of undergoing surgery and the risk of death were analysed by means of multivariable regression methods.ResultsAge-standardised incidence decreased among males (24–12 per 100,000 inhabitants) and females (10–6); mortality rates decreased at a similar pace. The proportion of cardia tumours remained stable. Stage distribution worsened over time among patients with cardia (stages I and II: 32% in 1990–1993 and 22% in 2006–2007, p = 0.005) and non-cardia (stage IV: 33% in 1990–1993 and 40% in 2006–2007, p = 0.0003) cancer. Chemotherapy rates increased in all settings. Five-year survival worsened over time for patients with non-cardia tumours. Age and stage had significant influence on survival after stratification for tumour localisation. After adjustments for relevant factors (i.e. stage), the risk of death decreased since the late 90s for patients with a cardia tumour (hazard ratio 0.8, p = 0.01).ConclusionThe absence of improvement in survival rates indicates the need for earlier detection and prospective studies to evaluate new therapy regimens with standardised surgery and pathology.     

Socioeconomic status and event free survival in pediatric acute lymphoblastic leukemia: A population-based cohort study

The impact of socioeconomic status (SES) upon childhood cancer outcomes has not been extensively examined. Our objective was to determine the association between SES and event-free survival (EFS) among children with acute lymphoblastic leukemia (ALL) diagnosed in Ontario, Canada from 1995–2011 (N = 1541) using Cox proportional hazards. Neither neighborhood-level median income quintile, distance from tertiary center, or rural residence significantly predicted EFS in the context of a universal healthcare system. Immigrant children experienced significantly superior EFS; confounding by ethnicity could not be ruled out. Confirmatory studies using additional individual-level SES variables are warranted.     

Therapeutic effect of ranimustine(MCNU) on myeloproliferative disorder and chronic myelomonocytic leukemia

Seventeen patients with myeloproliferative disorders and one patient with chronic myelomonocytic leukemia (CMMoL) were treated with ranimustine++ (MCNU), and the efficacy was evaluated. MCNU was given intravenously by drip infusion at an usual dose of 100 approximately 150 mg with intervals arranged according to the counts of peripheral blood cells. A complete remission was achieved in all 10 patients with chronic myelogenous leukemia (CML) in chronic phase. In three of patients with polycythemia vera (PV) the excellent effects were obtained, and the other 2 cases showed moderate effect. An excellent effect was obtained in both 2 patients with essential thrombocythemia (ET). A patient with CMMoL revealed partial remission. The overall efficacy rate was 100%. The cases with CML needed more long term and much more dose of the drug in order to get remission compared with PV and ET. After remission in both PV and ET, well controlled states were maintained for a relatively long period with no additional administration. In CMMoL, MCNU combined with 6-mercaptopurine also showed remarkable anti-tumor effects. It suggests that MCNU may be one of the useful drugs for the treatment of CMMoL. The side effects observed with MCNU were a slight degree of nausea and vomiting (28%), however they showed no trouble on carrying out the therapy.     

Pericardial effusion in chronic myelomonocytic leukemia (CMML): a case report and review of the literature

Pericardial effusion is a rare but potentially life-threatening complication of chronic myelomonocytic leukemia. A case of a 70-year-old female with CMML is reported, who developed a severe pericardial effusion after an initially stable course of disease. She underwent pericardiocentesis and subsequent intracardial instillation of mitoxantrone with poor response. Subxiphoid pericardiotomy was successfully performed. Leukocytosis did not respond to hydroxyurea. Because of decreasing performance status of the patient systemic chemotherapy was not applied and the patient died within 2 weeks. A review of the literature shows that there is no effective treatment for pericardial effusion in CMML, and some common features may be noted.