Childhood malignancies in French Polynesia during the 1985-1995 period

We report an estimation of the incidence of childhood cancer among natives of French Polynesia (FP) during the 1985-1995 period. Our data were acquired from the Cancer Registry of FP and through an extensive investigation of other potential sources of information. The mean population of children between 1985 and 1995 was estimated to be 63 401 inhabitants, 32 487 of whom were boys and 30 914 girls, born and residing in FP. During the 1985-1995 period, 87 incident cases of childhood cancer were recorded among inhabitants born in FP or of an unknown place of birth (n = 2). Childhood cancer incidence had attained 125 cases/million child years and was very similar among girls (126 x 10(-6)) and boys (123 x 10(-6)), this incidence being slightly lower than among other populations of similar ethnic origin: Standardized Incidence Ratio (SIR) = 0.8 (95% CI: 0.7-1.0) when compared with New Zealand Maoris and SIR = 0.8 (95% CI: 0.6-1.0) when compared with natives from Hawaii. For both sexes considered together, the most frequent cancer type was leukaemia, followed by central nervous system (CNS) malignancies, neuroblastoma, and non-Hodgkin's lymphoma (NHL). Only one case of gonadal and germ cell tumours and one case of carcinoma were reported. Childhood cancer incidence was predominant among children living in the Windward, Leeward and Marquesas Islands and the Tuamotu-Gambier archipelago, but lower in the Austral Islands. The incidence of acute non-lymphocytic leukaemia (ANLL) decreased from 3.3 x 10(-5) between 1985 and 1989, an unexpectedly high incidence, to 0.8 x 10(-5) between 1990 and 1995.     

Interdisciplinary tumour boards in Switzerland: quo vadis?

BACKGROUND/AIMS: The objective of the present investigation is twofold: first, to assess how interdisciplinary tumour boards in Switzerland are designed, established, and rated in clinical practice; and second, to evaluate perceptions regarding the determination of cancer centres as required by the Swiss National Cancer Programme. METHODS: An anonymous questionnaire was sent to the heads of surgical departments in Switzerland (n = 110). Among the clinics contacted were 11 large referral centres (type A clinics), 48 surgical departments of cantonal hospitals (type B clinics), and 51 regional surgical departments (type C clinics). RESULTS: For most type A and B clinics, tumour boards are held on a weekly basis (A: 100%, B: 88%, C: 26%).On average, 66% of respondents (A: 90%, B: 71 %, C: 52 %) consider tumour boards a standard of care for every cancer patient. Determination of cancer centres was favoured by 49% of all respondents (A: 80%, B: 56%, C: 32 %). CONCLUSION: The present survey in Switzerland clearly shows significant differences between type A, B, and C clinics in the use of tumour boards and in their perception as a standard of care. There are wide discrepancies in the perceived need to determine cancer centres in Switzerland as required by the Swiss National Cancer Programme. Since the implementation of tumour boards is associated with optimised cancer patient care, continuing education on their importance is a vital necessity.     

Second malignancies after treatment of childhood non-Hodgkin lymphoma – a report of the Berlin-Frankfurt-Muenster study group

Second malignant neoplasms (SMN) pose a concern for survivors of childhood cancer. We evaluated incidence, type and risk factors for SMN in patients included in Berlin-Frankfurt-Muenster protocols for childhood non-Hodgkin lymphoma.3,590 patients <15 years of age at diagnosis, registered between 01/1981 and 06/2010, were analyzed. SMN were reported by the treating institutions and the German Childhood Cancer Registry. After a median follow-up of 9.4 years (quartile [Q] range, Q1 6.7 and Q3 12.1) 95 SMN were registered (26 carcinomas including nine basal cell carcinomas, 21 acute myeloid leukemias/myelodysplastic syndromes, 20 lymphoid malignancies, 12 central nervous system [CNS]-tumors, and 16 others). Cumulative incidence at 20 years was 5.7±0.7%, standard incidence ratio, excluding basal cell carcinomas, was 19.8 (95% Confidence Interval [CI]: 14.5-26.5). Median time from initial diagnosis to second malignancy was 8.7 years (range, 0.2-30.3 years). Acute-lymphoblasticleukemia- type therapy, cumulative anthracycline dose, and cranial radiotherapy for brain tumor-development were significant risk factors in univariate analysis only. In multivariate analysis including risk factors significant in univariate analysis, female sex (hazard ratio [HR] 1.87, 95% CI: 1.23-2.86, P=0.004), CNS-involvement (HR 2.24, 95% CI: 1.03-4.88, P=0.042), lymphoblastic lymphoma (HR 2.60, 95% CI: 1.69-3.97, P<0.001), and cancer-predisposing condition (HR 11.2, 95% CI: 5.52-22.75, P<0.001) retained an independent risk. Carcinomas were the most frequent SMN after non-Hodgkin lymphoma in childhood followed by acute myeloid leukemia and lymphoid malignancies. Female sex, lymphoblastic lymphoma, CNS-involvement, or/and known cancer-predisposing condition were risk factors for SMN-development. Our findings set the basis for individualized long-term follow-up and risk assessment of new therapies.     

Population-based surveillance by colonoscopy: effect on the incidence of colorectal cancer. Telemark Polyp Study I.

BACKGROUND: Most cases of colorectal cancer (CRC) develop from adenomas. Polypectomy is believed to reduce the incidence of CRC, but this effect has never been explored in prospective controlled studies. The aim of the present study was to evaluate the effect of polypectomy on colorectal cancer incidence in a population-based screening program. METHODS: In 1983, 400 men and women aged 50-59 years were randomly drawn from the population registry of Telemark, Norway. They were offered a flexible sigmoidoscopy and, if polyps were found, a full colonoscopy with polypectomy and follow-up colonoscopies in 1985 and 1989. A control group of 399 individuals was drawn from the same registry. In 1996 both groups (age, 63-72 years) were invited to have a colonoscopic examination. Hospital files and the files of The Norwegian Cancer Registry were searched to register any cases of CRC in the period 1983-96. RESULTS: At screening endoscopy 324 (81%) individuals attended in 1983 and 451 (71%) in 1996. From 1983 to 1996, altogether 10 individuals in the control group and 2 in the screening group were registered to have developed CRC (relative risk, 0.2; 95% confidence interval (CI), 0.03-0.95; P = 0.02). A higher overall mortality was observed in the screening group, with 55 (14%) deaths, compared with 35 (9%) in the control group (relative risk, 1.57; 95% CI, 1.03-2.4; P = 0.03). CONCLUSION: Endoscopic screening examination with polypectomy and follow-up was shown to reduce the incidence of CRC in a Norwegian normal population. The possible effect of screening on overall mortality should be addressed in larger studies.     

Thyroid function in paediatric and young adult patients after sarcoma therapy: a report from the Late Effects Surveillance System

OBJECTIVE: The role of chemotherapy in thyroid sequelae after cancer treatment has not been studied systematically, especially in sarcoma patients. The aim of this study was to determine the incidence of post-therapeutic thyroid disorders and their contributing factors in a cohort of paediatric sarcoma patients. DESIGN: Late effects of sarcoma treatment have been collected prospectively within the Late Effects Surveillance System (LESS) in Germany, Austria and Switzerland since 1998. PATIENTS: We studied 340 relapse-free paediatric patients (median age at diagnosis 12.2 [interquartile range (IQR) = 7.3-15.6 years] treated for osteosarcoma, soft tissue sarcoma or Ewing's sarcoma within the COSS-96, CWS-96/CWS-2002P or EICESS-92/EURO-E.W.I.N.G.-99 therapy trials. In addition to polychemotherapy, 127 patients were irradiated (mean cumulative dose 47 +/- 9.7 Gy), including 51 patients with irradiation to the head/neck region. Median follow-up was 24.6 (IQR = 11.9-44.9) months. MEASUREMENTS: We reviewed the results of yearly examinations of serum TSH and fT4 levels and thyroid ultrasound examinations. RESULTS: The incidence of thyroid disorders was 37% (19/51, 95% CI 24-52%) in patients with head/neck irradiation, and 11% (32/289, 95% CI 8-15%) in patients without irradiation to the head/neck. Thyroid disorders were more frequent in patients treated with idarubicin (P = 0.027) and trofosfamide (P = 0.016). We also found a significant association between raised TSH levels and treatment with trofosfamide (P = 0.008) or idarubicin (P = 0.037) (n = 250). CONCLUSIONS: The incidence of thyroid disorders in the head/neck-irradiated group was high. Even without head/neck irradiation, we found an increased proportion of patients with thyroid disorders, possibly as a result of chemotherapy.     

Expected number of childhood cancers in Italy from 2001 to 2015

The total number of children with incident cancer in Italy has never been specifically estimated. Specialized population-based Childhood Cancer Registries have only been operating in Piedmont (CCRP) and in the Marche region, while general population cancer registries cover about 20% of the Italian population. The number of expected cases of childhood cancer (0-14 years) in Italy in the period 2001-2015 has been estimated using CCRP incidence rates and annual percentage changes. The expected number of cases of all cancer types were 8,132, 8,672 and 8,944 in the periods 2001-2005, 2006-2010 and 2011-2015 respectively. These figures help evaluate the allocation of resources for the care of child cancer patients in Italy, and to estimate the number of cases expected to enter clinical trials.     

Alcohol consumption patterns and risk factors among childhood cancer survivors compared to siblings and general population peers

AIMS: This study describes alcohol consumption among adult survivors of pediatric cancer compared to sibling controls and a national sample of healthy peers. Risk factors for heavy drinking among survivors are described. DESIGN, SETTING AND PARTICIPANTS: Cross-sectional data were utilized from the Childhood Cancer Survivor Study including adult survivors of pediatric cancer (n = 10 398) and a sibling cohort (n = 3034). Comparison data were drawn from the National Alcohol Survey (n = 4774). MEASUREMENT: Alcohol consumption, demographic, cancer diagnosis, treatment and psychosocial factors were measured. FINDINGS: Compared to peers, survivors were slightly less likely to be risky [adjusted odds ratio (ORadj) = 0.9; confidence interval (CI) 0.8-1.0] and heavy drinkers (ORadj = 0.8; CI 0.7-0.9) and more likely to be current drinkers. Compared to siblings, survivors were less likely to be current, risky and heavy drinkers. Risk factors for survivors' heavy drinking included being age 18-21 years (ORadj = 2.0; 95% CI 1.5-2.6), male (ORadj = 2.1; 95% CI 1.8-2.6), having high school education or less (ORadj = 3.4; 95% CI 2.7-4.4) and drinking initiation before age 14 (ORadj = 6.9; 95% CI 4.4-10.8). Among survivors, symptoms of depression, anxiety or somatization, fair or poor self-assessed health, activity limitations and anxiety about cancer were associated with heavy drinking. Cognitively compromising treatment, brain tumors and older age at diagnosis were protective. CONCLUSIONS: Adult survivors of childhood cancer show only a modest reduction in alcohol consumption compared to peers despite their more vulnerable health status. Distress and poorer health are associated with survivor heavy drinking. Screening for alcohol consumption should be instituted in long-term follow-up care and interventions among survivors and siblings should be established to reduce risk for early drinking.     

Childhood cancer in Sudan: 1999-2007

There is paucity of information on childhood cancer from Sudan with the last studies published more than 20 years ago. This study aims to provide a current picture of childhood cancer in Sudan. Data was obtained from the hospital registry for the period May 1999 to June 2007 on all paediatric patients presenting to the Institute of Nuclear Medicine and Oncology, University of Gezira, Wad Madani, Sudan. There were 322 children with cancer during this time period with a male:female ratio of 1.6:1. Lymphomas (111, 35%), leukaemia (83, 26%) and Wilms' tumour (43, 13%) were the three most common groups of tumours. Thirty percent of all lymphomas were Burkitt's lymphoma; 3.4% of all childhood cancer cases were nasopharyngeal carcinomas.     

Cause-specific mortality and second cancer incidence after non-Hodgkin lymphoma: a report from the Childhood Cancer Survivor Study

Second primary malignancies and premature death are a concern for patients surviving treatment for childhood lymphomas. We assessed mortality and second malignant neoplasms (SMNs) among 1082 5-year survivors of non-Hodgkin lymphoma (NHL) in the Childhood Cancer Survivor Study, a multi-institutional North American retrospective cohort study of cancer survivors diagnosed from 1970 to 1986. Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) were calculated using US population rates. Relative risks for death and solid tumor SMNs were calculated based on demographic, clinical, and treatment characteristics using Poisson regression models. There were 87 observed deaths (SMR = 4.2; 95% CI, 1.8-4.1) with elevated rates of death from solid tumors, leukemia, cardiac disease, and pneumonia. Risk for death remained elevated beyond 20 years after NHL. Risk factors for death from causes other than NHL included female sex (rate ratio [RR] = 3.4) and cardiac radiation therapy exposure (RR = 1.9). There were 27 solid tumor SMNs (SIR = 3.9; 95% CI, 2.6-5.7) with 3% cumulative incidence between 5 and 20 years after NHL diagnosis. Risk factors were female sex (RR = 3.1), mediastinal NHL disease (RR = 5.2), and breast irradiation (RR = 4.3). Survivors of childhood NHL, particularly those treated with chest RT, are at continued increased risk of early mortality and solid tumor SMNs.     

Clinical significance of signet ring cell rectal carcinoma

Background and aims Signet ring cell carcinoma of the rectum (SCCR) is a rare type of rectal carcinoma. This study examined the clinical significance of SCCR.Patients and methods From our medical records we retrospectively identified 61 SCCR patients and compared their clinical data and outcomes to those of 144 consecutive patients with non-SCCR mucinous rectal adenocarcinomas (NSMR) and 2,414 consecutive patients with nonmucinous rectal adenocarcinomas (NMR).Results The incidence of SCCR was 1.39% of rectal cancers. Mean patient age at onset of SCCR (48.1years, range 15–80) was significantly lower than that for NSMR (57.4 years, 9–88) and NMR (62.6 years, 12–94). The proportion of late stage (TNM III+IV) tumors was significantly higher in SCCR (90%) than in NSMR (69%) and NMR (48%). There were more tumors located in the lower rectum in SCCR (46%) than in NSMR (34%) and NMR (29%). SCCR tumors were significantly larger (5.68±3.84 cm) than NSMR (4.27±1.78 cm) and NMR tumors (3.76±1.71 cm). A higher percentage of patients with SCCR (42.6%) received abdominoperineal resection for treatment. In tumors with TNM stage IV the rate of tumor spread via the hematogenous route was significantly lower in SCCR (18.5%) than in NSMR (43.5%) and in NMR (69%). The rate of tumor spread via seeding to the peritoneum was lower in SCCR (22.2%) than in NSMR (43.5%) but higher than in NMR (2.7%). The rate of tumor spread via the lymphatic route was higher in SCCR (44.4%) than in NSMR (26.1%) and significantly higher than in NMR (12.3%). The 1-, 2-, and 5-year overall SCCR survival rates were 73.9%, 36.3%, and 23.3% respectively, which were significantly poorer than those of NSMR and NMR. For the 28 stage III and R0 SCCR tumors the 1-, 2-, and 5-year disease-free survival rates of SCCR were 84.0%, 44.2%, and 30.3%, respectively, which are comparable with general data of stage III rectal cancer in the world.Conclusion Diffuse infiltration of signet ring cells enhances the tendency of mucinous carcinomas of the rectum in more local extension and easier lymphatic spreading but not at peritoneal seeding. Although SCCR had the poorest prognosis, this outcome may be due to the advanced tumor stage rather than histology itself.     

Quality of diabetes management in children and adolescents in Denmark.

AIM: To describe the management of children and adolescents with Type 1 diabetes mellitus in Denmark. METHODS: Quality indicators with standards of childhood diabetes management were chosen based on international and national guidelines. Data originated from the nation-wide Danish Registry for Childhood Diabetes and two questionnaires: one questionnaire was sent to all children with diabetes (response rate 78%, n=1335) and the other was sent to the 19 centres in Denmark treating these children (response rate 100%). Simultaneously, the children were asked to take a blood sample for central HbA1c-analysis (normal range 4.3-5.8, mean 5.1%). RESULTS: Most children were managed at centres which complied with the standards for the process indicators for good diabetes management, but not with the standards for most structure and outcome indicators. Only one third of the children reached the treatment target for HbA1c. Their mean HbA1c-level increased gradually from ages 4-14 without significant difference between genders. The youngest children had the lowest HbA1c (mean 8.2%) and the lowest rate of severe hypoglycaemic events (4.6 events per 100 patient years). The subgroup of children without any hypoglycaemic events had the significantly lowest mean HbA1c-level (8.6%, P=0.028). CONCLUSIONS: The Danish Registry for Childhood Diabetes provided useful data for quality improvement, but had to be supplemented with data from questionnaires on the structure and process indicators. Outcome of paediatric diabetes management in Denmark was unsatisfactory. Centres need feedback on ways to improve care to lower the children's risk of developing severe diabetes complications.     

Risk of cancer in patients with scleroderma: a population based cohort study

BACKGROUND: Previous studies have suggested an increased risk of cancer among patients with scleroderma. OBJECTIVE: To study a population based cohort of patients with scleroderma in South Australia. METHODS: Subjects with scleroderma were identified from the South Australian Scleroderma Registry established in 1993. All subjects on the scleroderma registry were linked to the South Australian Cancer Registry to identify all cases of cancer until 31 December 2000. Standardised incidence ratios (SIRs) for cancer for subjects with scleroderma were determined using the age- and sex-specific rates for South Australia. RESULTS: In 441 patients with scleroderma, 90 cases of cancer were identified, 47 of which developed after inclusion on the scleroderma registry. The SIRs for all cancers among these patients were significantly increased (SIR=1.99; 95% confidence interval (95% CI) 1.46 to 2.65) compared with the cancer incidence rates for South Australia. The SIRs for lung cancer (SIR=5.9; 95% CI 3.05 to 10.31) were also significantly increased. The SIRs for all cancers among the subgroups with diffuse scleroderma (SIR=2.73; 95% CI 1.31 to 5.02) and limited scleroderma (SIR=1.85; 95% CI 1.23 to 2.68) were significantly increased. CONCLUSIONS: This population based cohort study provides evidence that scleroderma is associated with cancer, and in particular, lung cancer. In addition, both diffuse and limited forms of scleroderma are associated with a similarly increased risk of cancer.     

Mortality from primary liver cancer in Switzerland from 1975 to 1994

BACKGROUND/AIMS: The aim of the present study was to analyze the mortality from primary liver cancer in Switzerland over a 20 year period and compare our results with the mortality data from Germany, France, Italy and Austria. METHODS: Absolute and age-standardized mortality rates for primary liver cancer from 1975 to 1994 were obtained from the Swiss Federal Office of Statistics. The corresponding figures (1980-1994) for Germany, France, Italy and Austria were extracted from the World Health Organization mortality database. RESULTS: The average age standardized mortality rate from primary liver cancer in Swiss men increased by 33% over the last twenty years from 3.9 to 5.2/100,000 people, whereas it remained unchanged on a much lower level in women (around 1.1/100,000). A similar increase was observed in men from France (91%), Italy (44%) and Germany (52%), whereas in Austria (5%) the increase was much less pronounced. CONCLUSION: The rising mortality from primary liver cancer in Switzerland is restricted to Swiss men. The changes in Switzerland are very similar to those in France, Italy and Germany. The reason for this increase remains unknown, but could be related to an increase in HCV-related primary liver cancer. Population based studies analyzing the aetiology of the underlying liver disease associated with HCCs detected are required to address this issue.     

A population-based study of childhood myelodysplastic syndrome in British Columbia, Canada

Myelodysplastic syndrome (MDS) is considered to be very rare in children. However, the only two published population-based studies reported widely divergent incidence figures. To further explore the epidemiology of childhood MDS and to evaluate the accuracy of cancer registry and treatment trial data, we conducted a population-based study of children aged 0-14 years in British Columbia (BC), Canada, between 1982 and 1996. MDS was diagnosed in 31 cases corresponding to an annual incidence of 3.2 per million children or 6% of all leukaemias, compared with an incidence of 6.0/million for acute myeloid leukaemia (AML), and of 0.5/million for chronic myeloid leukaemia. There was a non-significant (P = 0.19) trend toward an increase in MDS incidence with time, the increase was partly explained by an increasing number of patients with Down syndrome. Associated abnormalities were found in 48% of the MDS cases with Down syndrome as the most common (seven cases). Only one third of the MDS cases were correctly registered in the Cancer Registry and less than half of the eligible MDS patients were enrolled on a cooperative group study. Data on MDS from treatment-based studies and cancer registries were inaccurate and seemed to significantly underestimate the incidence of MDS in children.     

Descriptive epidemiology of thyroid cancer in the Swiss Canton of Vaud

Although substantial decreases have been recorded, age-standardized mortality rates from thyroid cancer in Switzerland are still the highest in Europe in men (0.9/100,000), together with those from Austria, and the third highest (1.0/100,000) in women. Detailed analysis of 308 new cases registered between 1974 and 1987 in the Swiss Canton of Vaud revealed an overall incidence rate of 1.36/100,000 men (world standard) in 1974-1980 and of 1.74/100,000 in 1981-1987. Corresponding values for women were 4.28 and 4.51, respectively. Thus, women constituted the majority of all cases (76%). Papillary carcinoma was the most frequent histological type (53%) followed by follicular (27%), undifferentiated (5%) and medullary (2%); other morphologies and clinical tumours accounted for 13% of the whole series. In both sexes, most of the apparent increase over the calendar period was restricted to the papillary type. Overall 5- and 10-year survival rates were 71% and 57%. When various factors were introduced in a Cox proportional-hazard model, young age at diagnosis (hazard rate for greater than or equal to 65 years vs less than 45 = 14.7; 95% confidence interval = 7.5-29.1) and good histological differentiation (hazard rate for papillary and follicular vs undifferentiated = 0.4) emerged as strong favourable and independent prognostic factors. The reduced hazard rate for women, other factors being equal, was of borderline significance (0.7, 95% confidence interval = 0.5-1.0), whereas no significant difference was observed between follicular and papillary carcinomas, and calendar periods of diagnosis.     

Epidemiology of Type I diabetes mellitus in Switzerland: steep rise in incidence in under 5 year old children in the past decade

Aims/hypothesis. In this nationwide prospective study we wanted to verify the trend of increasing diabetes incidence data from our earlier retrospective analysis of the military registry of Swiss men. Subjects and methods. The data collection of newly diagnosed children in Switzerland at an age younger than 15 years started in 1991. The countrywide survey used a small questionnaire which was sent back to the study centre. The questionnaire was anonymous and contained: hospital of diagnosis, initials, sex, birth date, date of diagnosis, residence, country of citizenship, and responsible physician. General data on the population were taken from publications of the Swiss Federal Statistical Office. Results. A total of 941 children below the age of 15 years with newly diagnosed Type I (insulin-dependent) diabetes mellitus were collected (434 girls, 507 boys). The incidence in children aged 0 to 14 years rose significantly between 1991 and 1999 with a yearly average increase of 5.1 %. In the age group 0 to 4 years a more than four-fold increase in incidence from 2.4/100 000 per year to 10.5/100 000 per year (p = 0.0002) was recorded, whereas the age-specific incidence in the 5 to 9-year-old and 10 to 14-year-old children did not change during the data collection period. The incidence was significantly higher in boys than in girls, whereas no difference was found between rural and urban populations. Conclusion/interpretation. The incidence of Type I diabetes is rising in children living in Switzerland but only the youngest age group of under 5 years of age is affected showing a large annual average increase of 23.8 %. [Diabetologia (2001) 44: 286–289] Received: 7 September 2000 and in revised form: 25 October 2000     

Haematopoietic stem cell transplantation in Switzerland. Report from the Swiss Transplant Working Group Blood and Marrow Transplantation (STABMT) Registry 1997-2003

In 1997, the Swiss Transplant Working Group Blood and Marrow Transplantation (STABMT) initiated a mandatory national registry for all haematopoietic stem cell transplants (HSCT) in Switzerland. As of 2003, information was collected of 2010 patients with a first HSCT (577 allogeneic (29%) and 1433 autologous (71%) HSCT) and 616 additional re-transplants. This included 1167 male and 843 female patients with a median age of 42.4 years (range 0.2-76.6 years). Main indications were leukaemias (592; 29%) lymphoproliferative disorders (1,061; 53%), solid tumours (295; 15%) and non-malignant disorders (62; 3%). At the time of analysis 1,263 patients were alive (63%), 747 had died (37%). Probability of survival, transplant related mortality or relapse at 5 years was 52%, 21%, 36% for allogeneic and 54%, 5%, 60% for autologous HSCT. Outcome depended on indication, donor type, stem cell source and age of patient. HSCT is an established therapy in Switzerland. These data describe current practice and outcome.     

Incidence of malignancies among patients with type I Gaucher disease from a single referral clinic

BACKGROUND: It has been implied that the incidence of malignant disorders is increased in patients with non-neuronopathic (type I) Gaucher disease. The purpose of the study was to ascertain numbers of cancers in type I Gaucher disease since this is of considerable concern to patients and physicians. METHODS: Records of 505 patients with type I Gaucher disease seen at a large referral clinic since 1990 were culled in December 2004 to ascertain diagnosis of a cancer identified during follow-up. Age-matched data from the Israeli National Cancer Registry (INCR) database were used for comparison. FINDINGS: Patients diagnosed with cancer before 1990 were not included. Of the remaining 500 patients, 227 (45.4%) were male, mean age=38.7 years; and 273 (54.6%) were female, mean age=37.0 years (SD=21.0 years for both). Twenty patients (4.0%) had developed a cancer through December 31, 2003: 6 were male and 14 were female. The most common were three cases each of lymphoma and myelodysplastic syndrome and two cases of multiple myeloma. There was no statistically significant excess of cancer rate among patients relative to age-matched rates reported in national Jewish Israeli and Ashkenazi Jewish Israeli registry records. CONCLUSIONS: There appears to be no excess risk for hematological or other cancers among patients with type I Gaucher disease relative to the overall Jewish population matched for age. This study confirms recent international studies of patients with Gaucher disease for no excess risk for all cancers but multiple myeloma where these latter studies implicate a significantly higher incidence.     

Longitudinal trends of stomach cancer and esophageal cancer in Yangzhong County: a high-incidence rural area of China

OBJECTIVE: This study aims to investigate the recent trends in incidence rates of stomach cancer and esophageal cancer in a high-incidence rural area of China. METHODS: All new cancer occurrences registered between 1991 and 2003 in the Yangzhong Cancer Registry were reviewed. Yearly age-specific and age-adjusted incidence rates were calculated for males and for females. Longitudinal trends of cancer incidence were estimated by the estimated annual percentage change method. RESULTS: In total 12 691 incident cancer cases were registered, with 7159 males (56.41%) and 5532 females (43.59%). Adjusting to the world standard population, the incidence rate of all cancers decreased significantly across the period 1991-2003 from 357.02 to 283.21 per 10 person-years. For males this rate decreased from 447.22 to 346.72 per 10 person-years, and for females the incidence rate decreased from 284.36 to 225.73 per 10 person-years. The major cancers in Yangzhong County were stomach cancer and esophageal cancer, accounting for more than 70% of all cancer occurrences. During the past 13 years, the incidence rates of stomach cancer decreased greatly from 231.92 to 145.26 per 10 person-years in males and from 114.16 to 74.59 per 10 person-years in females. The estimated annual percentage changes of stomach cancer incidence were -2.96% [95% confidence interval (CI), -2.99% to -2.92%] in males and -2.86% (95% CI, -2.89% to -2.82%) in females. Incidence rates for esophageal cancer decreased slightly from 121.48 to 93.84 per 10 person-years in males with an estimated annual percentage change of -1.39% (95% CI, -1.42% to -1.36%), and from 99.74 to 73.73 per 10 person-years in females at an annual change of -2.18% (95% CI, -2.22% to -2.14%). CONCLUSION: Findings from this study showed that, although there is a decreasing trend of stomach cancer and esophageal cancer in this high-incidence area, the rates remain high. Future effort should be directed toward identifying factors behind the high rates and those contributing to the decreasing trend.