Childhood Cancer Epidemiology

Acute Myeloid Leukemia (AML)

Brain and Spinal Cord Tumours

Osteosarcoma

Austin MT, Hamilton E, Zebda D, et al.
Health disparities and impact on outcomes in children with primary central nervous system solid tumors.
J Neurosurg Pediatr. 2016; 18(5):585-593 [PubMed] Related Publications

OBJECTIVE Health disparities in access to care, early detection, and survival exist among adult patients with cancer. However, there have been few reports assessing how health disparities impact pediatric patients with malignancies. The objective in this study was to examine the impact of racial/ethnic and social factors on disease presentation and outcome for children with primary CNS solid tumors. METHODS The authors examined all children (age ≤ 18 years) in whom CNS solid tumors were diagnosed and who were enrolled in the Texas Cancer Registry between 1995 and 2009 (n = 2421). Geocoded information was used to calculate the driving distance between a patient's home and the nearest pediatric cancer treatment center. Socioeconomic status (SES) was determined using the Agency for Healthcare Research and Quality formula and 2007-2011 US Census block group data. Logistic regression was used to determine factors associated with advanced-stage disease. Survival probability and hazard ratios were calculated using life table methods and Cox regression. RESULTS Children with advanced-stage CNS solid tumors were more likely to be < 1 year old, Hispanic, and in the lowest SES quartile (all p < 0.05). The adjusted odds ratios of presenting with advanced-stage disease were higher in children < 1 year old compared with children > 10 years old (OR 1.71, 95% CI 1.06-2.75), and in Hispanic patients compared with non-Hispanic white patients (OR 1.56, 95% CI 1.19-2.04). Distance to treatment and SES did not impact disease stage at presentation in the adjusted analysis. Furthermore, 1- and 5-year survival probability were worst in children 1-10 years old, Hispanic patients, non-Hispanic black patients, and those in the lowest SES quartile (p < 0.05). In the adjusted survival model, only advanced disease and malignant behavior were predictive of mortality. CONCLUSIONS Racial/ethnic disparities are associated with advanced-stage disease presentation for children with CNS solid tumors. Disease stage at presentation and tumor behavior are the most important predictors of survival.

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Dermody S, Walls A, Harley EH
Pediatric thyroid cancer: An update from the SEER database 2007-2012.
Int J Pediatr Otorhinolaryngol. 2016; 89:121-6 [PubMed] Related Publications

OBJECTIVE: To update the medical literature regarding the incidence, disease specific survival, and treatment modalities utilized in pediatric patients diagnosed with thyroid carcinomas.
STUDY DESIGN: Cross Sectional Analysis of a National Database.
STUDY SETTING: SEER Database.
METHODS: The National Cancer Institute's Surveillance Epidemiology and End Results (SEER) Database was queried for all cases of pediatric thyroid cancer between the years 2007 and 2012. Patients ages 0-19 were grouped by histological subtypes and demographic data, overall incidence rate, and disease specific survival after surgery and surgery with radiation therapy. Fifteen-Year Disease Specific Survival Curves were generated and treatment modalities were compared to assess for statistical differences at each yearly interval.
RESULTS: A total of 1723 pediatric patients were identified and the average age-adjusted rate of malignancy was determined to be 0.59 per 100,000 patients. The incidence of pediatric thyroid cancer was approximately 4.4:1 when comparing females to males, respectively. Papillary subtype was the most common (n = 1014, 58.8%), followed by follicular variant subtype (n = 397, 23%), follicular subtype (n = 173, 10.1%) and medullary subtype (n = 139, 8.1%). As pediatric patients reached fifteen to nineteen years of age, the incidence of papillary and follicular variant subtypes increased. Analysis of medullary thyroid cancer data revealed that incidence was highest in the zero to four age group and declined at later years. Pediatric patients presenting with metastatic medullary thyroid carcinoma maintained significantly poorer fifteen-year disease specific survival when compared to other histologic subtypes (p < 0.05). Intervention with surgery and radiation therapy provided significant benefit across all histologic subtypes when evaluating disease specific survival at fifteen-years past the initial diagnoses (p < 0.05).
CONCLUSIONS: Pediatric thyroid carcinoma remains an uncommon diagnosis despite an annual increase in incidence of approximately one percent since the development of the SEER database. Overall, pediatric thyroid carcinomas demonstrate an excellent prognosis if identified early and appropriate management is available. Caucasian female patients have higher incidence of carcinoma diagnoses when compared to males. Medullary histologic subtype, especially when metastatic at initial diagnoses, demonstrates statistically poorer outcomes when compared to other subtypes.

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Thyroid Cancer

Ohira T, Takahashi H, Yasumura S, et al.
Comparison of childhood thyroid cancer prevalence among 3 areas based on external radiation dose after the Fukushima Daiichi nuclear power plant accident: The Fukushima health management survey.
Medicine (Baltimore). 2016; 95(35):e4472 [PubMed] Free Access to Full Article Related Publications

The 2011 Great East Japan Earthquake led to a subsequent nuclear accident at the Fukushima Daiichi Nuclear Power Plant. In its wake, we sought to examine the association between external radiation dose and thyroid cancer in Fukushima Prefecture. We applied a cross-sectional study design with 300,476 participants aged 18 years and younger who underwent thyroid examinations between October 2011 and June 2015. Areas within Fukushima Prefecture were divided into three groups based on individual external doses (≥1% of 5 mSv, <99% of 1 mSv/y, and the other). The odds ratios (ORs) and 95% confidence intervals of thyroid cancer for all areas, with the lowest dose area as reference, were calculated using logistic regression models adjusted for age and sex. Furthermore, the ORs of thyroid cancer for individual external doses of 1 mSv or more and 2 mSv or more, with the external dose less than 1 mSv as reference, were calculated. Prevalence of thyroid cancer for the location groups were 48/100,000 for the highest dose area, 36/100,000 for the middle dose area, and 41/100,000 for the lowest dose area. Compared with the lowest dose area, age-, and sex-adjusted ORs (95% confidence intervals) for the highest-dose and middle-dose areas were 1.49 (0.36-6.23) and 1.00 (0.67-1.50), respectively. The duration between accident and thyroid examination was not associated with thyroid cancer prevalence. There were no significant associations between individual external doses and prevalence of thyroid cancer. External radiation dose was not associated with thyroid cancer prevalence among Fukushima children within the first 4 years after the nuclear accident.

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Thyroid Cancer

Siddiqui EU, Kazi SG, Habib MI, et al.
Pattern of relapse in paediatric acute lymphoblastic leukaemia in a tertiary care unit.
J Pak Med Assoc. 2016; 66(8):961-7 [PubMed] Related Publications

OBJECTIVE: To determine the frequency, site and time to relapse from diagnosis, and to see the relationship of relapse with important prognostic factors.
METHODS: The prospective descriptive observational study was conducted at the National Institute of Child Health, Karachi, June 2005 to May 2007, and comprised newly-diagnosed cases of acute lymphoblastic leukaemia. Bone marrow aspiration was done on reappearance of blast cells in peripheral smear and cerebrospinal fluid. Detailed report was done each time when intra-thecal chemotherapy was given or there were signs and symptoms suggestive of central nervous system relapse. SPSS 12 was used for data analysis.
RESULTS: Of the 60 patients enrolled, 4(6.6%) expired and 1(1.7%) was lost to follow-up. Of the 55(91.6%) who comprised the study sample, 35(58%) were males and 25(42%) females. Mean age of relapse was 6.8±3.27 years. Mean time to relapse from diagnosis was 1.3±0.54 years; 12(20%) patients suffered relapse, and of them 5(14%) were boys. Central nervous system relapse in 8(67%) patients was the most common site, with 3(25%) bone-marrow relapses. Out of 12 patient with relapses, 9(75%) had white blood cell count less than 50,000/cm.
CONCLUSIONS: Relapse in acute lymphoblastic leukaemia was common, although treatment modalities are improving day by day.

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Brain and Spinal Cord Tumours

Lin HC, Chao YH, Wu KH, et al.
Increased risk of herpes zoster in children with cancer: A nationwide population-based cohort study.
Medicine (Baltimore). 2016; 95(30):e4037 [PubMed] Free Access to Full Article Related Publications

Herpes zoster is rare in healthy children, but immunocompromised persons have an increased risk of herpes zoster and severe diseases. Considering the very limited information on herpes zoster in children with cancer, we performed a nationwide population-based cohort study to estimate the incidence of herpes zoster in children with cancer and to explore the association between the 2 diseases.Data were obtained from the National Health Research Institutes Database in Taiwan. A total of 4432 children with newly diagnosed cancer between 2000 and 2007 were identified as the cancer cohort, and 17,653 children without cancer frequency-matched by sex and age at entry were considered the noncancer cohort. The association between herpes zoster and childhood cancer was determined.Children with cancer had a higher risk of herpes zoster. The incidence rate of herpes zoster was higher in the cancer cohort than in the noncancer cohort (20.7 vs 2.4 per 10,000 person-years; IRR = 8.6; 95% CI = 4.8-15.6). The cumulative incidence was significantly higher in the cancer cohort (P < 0.0001). Leukemia, lymphoma, and solid tumor were all associated with the increased risk, and leukemia had the highest magnitude of strength of association.This nationwide population-based cohort study demonstrated that children with cancer were associated with an increased risk of herpes zoster. In addition to early antiviral treatment, vaccination with heat-treated zoster vaccine or adjuvanted subunit vaccine could be an appropriate policy to decrease the incidence in children with cancer.

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Marcos-Gragera R, Galceran J, Martos C, et al.
Incidence and survival time trends for Spanish children and adolescents with leukaemia from 1983 to 2007.
Clin Transl Oncol. 2017; 19(3):301-316 [PubMed] Related Publications

OBJECTIVE: We have analysed incidence and survival trends of children and adolescents with leukaemia registered in Spanish population-based cancer registries during the period 1983-2007.
METHODS: Childhood and adolescent leukaemia cases were drawn from the 11 Spanish population-based cancer registries. For survival, registries with data for the period 1991-2005 and follow-up until 31-12-2010 were included. Overall incidence trends were evaluated using joinpoint analysis. Observed survival rates were estimated using Kaplan-Meier, and trends were tested using the log-rank test.
RESULTS: Based on 2606 cases (2274 children and 332 adolescents), the overall age-adjusted incidence rate (ASRw) of leukaemia was 47.9 cases per million child-years in children and 23.8 in adolescents. The ASRw of leukaemia increased with an annual percentage change of 9.6 % (95 % CI: 2.2-17.6) until 1990 followed by a stabilisation of rates. In adolescents, incidence did not increase. Five-year survival increased from 66 % in 1991-1995 to 76 % in 2001-2005. By age, survival was dramatically lower in infants (0) and adolescents (15-19) than in the other age groups and no improvement was observed. In both children and adolescents, differences in 5-year survival rates among major subgroups of leukaemias were significant.
CONCLUSIONS: The increasing incidence trends observed in childhood leukaemias during the study period were confined to the beginning of the period. Remarkable improvements in survival have been observed in Spanish children with leukaemias. However, this improvement was not observed in infants and adolescents.

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Leukemia - Molecular Biology

Childhood Leukaemia

Chan V, Pole JD, Keightley M, et al.
Children and youth with non-traumatic brain injury: a population based perspective.
BMC Neurol. 2016; 16:110 [PubMed] Free Access to Full Article Related Publications

BACKGROUND: Children and youth with non-traumatic brain injury (nTBI) are often overlooked in regard to the need for post-injury health services. This study provided population-based data on their burden on healthcare services, including data by subtypes of nTBI, to provide the foundation for future research to inform resource allocation and healthcare planning for this population.
METHODS: A retrospective cohort study design was used. Children and youth with nTBI in population-based healthcare data were identified using International Classification of Diseases Version 10 codes. The rate of nTBI episodes of care, demographic and clinical characteristics, and discharge destinations from acute care and by type of nTBI were identified.
RESULTS: The rate of pediatric nTBI episodes of care was 82.3 per 100,000 (N = 17,977); the average stay in acute care was 13.4 days (SD = 25.6 days) and 35% were in intensive care units. Approximately 15% were transferred to another inpatient setting and 6% died in acute care. By subtypes of nTBI, the highest rates were among those with a diagnosis of toxic effect of substances (22.7 per 100,000), brain tumours (18.4 per 100,000), and meningitis (15.4 per 100,000). Clinical characteristics and discharge destinations from the acute care setting varied by subtype of nTBI; the proportion of patients that spent at least one day in intensive care units and the proportion discharged home ranged from 25.9% to 58.2% and from 50.6% to 76.4%, respectively.
CONCLUSIONS: Children and youth with nTBI currently put an increased demand on the healthcare system. Active surveillance of and in-depth research on nTBI, including subtypes of nTBI, is needed to ensure that timely, appropriate, and targeted care is available for this pediatric population.

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Childhood Brain Tumours

Childhood Brain Tumors

Michel LA, Donckier J, Rosière A, et al.
Post-Chernobyl incidence of papillary thyroid cancer among Belgian children less than 15 years of age in April 1986: a 30-year surgical experience.
Acta Chir Belg. 2016; 116(2):101-13 [PubMed] Related Publications

OBJECTIVE: We raised the question of a possible relationship in Belgium between the occurrence of papillary thyroid carcinoma (PTC) and age of children (<15 years) at the time of the Chernobyl nuclear plant accident in April 1986.
SETTING: Referral university centre for endocrine surgery.
MATERIAL AND METHODS: Thirty-year prospective study of the experience of a surgical team with PTC since the Chernobyl accident, taken out of 2349 patients operated on for any thyroid lesions from April 1986 to April 2015, comparing the incidence of PTC by age groups.
MAIN OUTCOME MEASUREMENT: Comparison of PTC incidence in patients >15 years (group A) and children <15 years (group B) in April 1986.
RESULTS: Out of a total of 2349 patients having undergone thyroid surgery for all types of lesions during 30 year after Chernobyl and born before April 1986, 2164 were >15 years of age at the time of the nuclear accident (group A) and 175 developed PTC (8.1%) compared to 36 PTC (19.5%) that occurred in 185 children <15 years of age (group B) in April 1986 (p < 0.001).
CONCLUSIONS: Radiation exposure affected residents of countries (including Belgium) well beyond Ukraine and Belarus. This was demonstrated by a 1990 meteorological report. Over 30 years, there has been a persistent higher incidence of PTC among Belgian children below the age of 15 years at the time of the Chernobyl accident. This relationship with age has even been strengthened by the implementation of more sophisticated immunohistochemical biomarkers diagnostic technology since April 2011.

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Thyroid Cancer

Singer AW, Selvin S, Block G, et al.
Maternal prenatal intake of one-carbon metabolism nutrients and risk of childhood leukemia.
Cancer Causes Control. 2016; 27(7):929-40 [PubMed] Article available free on PMC after 01/07/2017 Related Publications

PURPOSE: Folate, vitamins B12 and B6, riboflavin, and methionine are critical nutrients for the one-carbon metabolism cycle involved in DNA synthesis and epigenetic processes. We examined the association between maternal intake of these nutrients before pregnancy and risk of childhood acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) in a matched case-control study.
METHODS: Maternal dietary intake and vitamin supplement use in the year before pregnancy was assessed by food frequency questionnaire for 681 ALL cases, 103 AML cases, and 1076 controls. Principal component analysis was used to construct a variable representing combined nutrient intake, and conditional logistic regression estimated the odds ratio (OR) and 95% confidence interval (CI) for the association of ALL and AML with the principal component and each nutrient.
RESULTS: Higher maternal intake of one-carbon metabolism nutrients from food and supplements combined was associated with reduced risk of ALL (OR for one-unit change in the principal component = 0.91, CI 0.84-0.99) and possibly AML (OR for the principal component = 0.83, CI 0.66-1.04). When analyzed separately, intake of supplements high in these nutrients was associated with a reduced risk of ALL in children of Hispanic women only.
CONCLUSIONS: In conclusion, these data suggest that higher maternal intake of one-carbon metabolism nutrients may reduce risk of childhood leukemia.

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Schoenaker MH, Suarez F, Szczepanski T, et al.
Treatment of acute leukemia in children with ataxia telangiectasia (A-T).
Eur J Med Genet. 2016; 59(12):641-646 [PubMed] Related Publications

Early onset ataxia telangiectasia (A-T) is a neurodegenerative DNA-instability disorder, which presents early in childhood. Hallmarks of A-T are progressive ataxia and a dramatic increased risk of developing malignancies (25%), especially of hematological origin. In children these malignancies mainly concern aggressive Non-Hodgkin lymphoma, acute leukemias and Hodgkin lymphoma. Of the acute leukemias, T-cell lymphoblastic leukemia (T-ALL) is by far the most common. Since patients with A-T experience increased toxicity to radio- and chemotherapeutic treatment, the optimal treatment strategy of acute leukemia remains subject of debate. Review of literature of treatment of T-ALL in patients with A-T (n = 18) showed that many patients are not diagnosed with A-T at time of presentation of T-ALL. This implicates that physicians must be aware of symptoms of A-T in young patients presenting with T-ALL. Complete remission rates are high following upfront modified as well as unmodified treatment strategies. Treatment of ALL in children with A-T is feasible and should be performed. Definitive treatment strategy must be determined by shared decision making with patient, caretakers and medical team. Future prospective studies are needed to elucidate optimal treatment strategy.

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Ataxia-Telangiectasia

Ataxia Telangiectasia

da Conceição Nunes J, de Araujo GV, Viana MT, Sarinho ES
Association of atopic diseases and parvovirus B19 with acute lymphoblastic leukemia in childhood and adolescence in the northeast of Brazil.
Int J Clin Oncol. 2016; 21(5):989-995 [PubMed] Related Publications

BACKGROUND: Several factors related to the immune system, such as a history of allergies and virus infections, may be associated with acute lymphoblastic leukemia (ALL). The purpose of this study was to analyze whether the presence of atopic diseases and previous infection with parvovirus B19 and Epstein-Barr virus (EBV) are associated with the development of ALL.
METHODS: This case-control study was performed in two tertiary hospitals located in northeastern Brazil. The study population included 60 patients who were diagnosed with non-T-cell ALL using myelogram and immunophenotyping and 120 patients in the control group. Atopy was evaluated via a parent questionnaire and medical records. Total immunoglobulin (Ig)E and IgG levels of parvovirus B19 and EBV were measured in the serum. Logistic regression was performed to assess the association between variables and odds of ALL.
RESULTS: We identified a significant inverse association between rhinitis, urticaria and elevated IgE serum levels with ALL. A history of parvovirus B19 infection showed a significant association with this type of cancer [OR (95 % CI) 2.00 (1.94-4.26); P = 0.050]. In logistic regression, the presence of atopy was a protective factor [OR (95 % CI) 0.57 (0.38-0.83); P = 0.004], and the presence of IgG for parvovirus B19 was an important risk factor for ALL [OR (95 % CI) 2.20 (1.02-4.76); P = 0.043].
CONCLUSIONS: These results suggest that atopic diseases and elevated total IgE levels are associated with a potential protective effect on the development of ALL. Previous infection with parvovirus B19 contributed to ALL susceptibility.

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Tournaire M, Epelboin S, Devouche E, et al.
Adverse health effects in children of women exposed in utero to diethylstilbestrol (DES).
Therapie. 2016; 71(4):395-404 [PubMed] Related Publications

OBJECTIVE: Exposure to diethylstilbestrol (DES) in utero is associated with adverse health effects, including genital anomalies in women and men, and cancers in women. Animal studies showed birth defects and tumors in the offspring of DES exposed mice, revealing transgenerational transmission of DES effects. In humans, birth defects, such as hypospadias were observed in children of prenatally exposed women. The aim of this research was to further assess the health effects in children of prenatally exposed women.
METHODS: In a retrospective cohort study, the reports of women exposed to DES in utero on their 4409 children were compared with those of unexposed women on their 6203 children. Comparisons used odd ratios (OR) between children of exposed and unexposed women and standardized incidence rate (SIR) with the general population. These cohorts were recruited on a voluntary basis to answer questionnaires.
RESULTS: There was a global increase of defects in children born to exposed women when compared with those born to unexposed (OR 2.29, 95% CI: 1.80-2.79, P<0.001) and with the general population (SIR 2.39, 95% CI: 2.11-2.68). Increased defects were observed in male genital tract, esophagus, lip or palate, musculoskeletal and circulatory systems. For female genital tract anomalies, there was no significant increase. However, this cohort being relatively young, further follow-up is needed. An increase of cerebral palsy was revealed. The incidence of cancers was not increased, in particular for breast, uterus and ovary.
CONCLUSION: Our results confirmed a transgenerational transmission of defects in male genital tract. With caution due to possible bias associated with this method, our data suggest an increase of defects for esophagus, lip or palate, musculoskeletal and circulatory system in children of exposed women.

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France

Archer NP, Napier TS, Villanacci JF
Fluoride exposure in public drinking water and childhood and adolescent osteosarcoma in Texas.
Cancer Causes Control. 2016; 27(7):863-8 [PubMed] Related Publications

PURPOSE: The purpose of this study was to examine the association between fluoride levels in public drinking water and childhood and adolescent osteosarcoma in Texas; to date, studies examining this relationship have been equivocal. Using areas with high and low naturally occurring fluoride, as well as areas with optimal fluoridation, we examined a wide range of fluoride levels in public drinking water.
METHODS: This was a population-based case-control study, with both cases and controls obtained from the Texas Cancer Registry. Eligible cases were Texas children and adolescents <20 years old diagnosed with osteosarcoma between 1996 and 2006. Controls were sampled from children and adolescents diagnosed with either central nervous system (CNS) tumors or leukemia during the same time frame. Using geocoded patient addresses at the time of diagnosis, we estimated patients' drinking water fluoride exposure levels based on the fluoride levels of their residence's public water system (PWS). Unconditional logistic regression models were used to assess the association between osteosarcoma and public drinking water fluoride level, adjusting for several demographic risk factors.
RESULTS: Three hundred and eight osteosarcoma cases, 598 leukemia controls, and 604 CNS tumor controls met selection criteria and were assigned a corresponding PWS fluoride level. PWS fluoride level was not associated with osteosarcoma, either in a univariable analysis or after adjusting for age, sex, race, and poverty index. Stratified analyses by sex were conducted; no association between PWS fluoride level and osteosarcoma was observed among either males or females.
CONCLUSIONS: No relationship was found between fluoride levels in public drinking water and childhood/adolescent osteosarcoma in Texas.

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Bone Cancers

Osteosarcoma

Teppo E, Penttinen J, Myöhänen O, et al.
Single-centre study reports a 84% five-year overall survival rate for paediatric solid tumours.
Acta Paediatr. 2016; 105(8):952-8 [PubMed] Related Publications

AIM: We investigated the characteristics and outcome of paediatric patients with solid tumours diagnosed and treated at the Tampere University Hospital, one of the five tertiary referral centres in Finland, for children and adolescents with malignancies.
METHODS: This retrospective cohort study collected data from hospital medical records on survival, diagnosis, age, sex, tumour size and stage at diagnosis. We also observed the disease recurrence and use of autologous haematopoietic stem cell transplantation. Data analyses were carried out with the Kaplan-Meier method, various nonparametric and parametric tests, and Cox regression modelling.
RESULTS: Between 1987 and May 2015, 424 children (59% boys), with a median age of 6.4 (IQR 2.5-11.8) years at diagnosis, were diagnosed and followed up for a median of 7.5 (range 0-27.9) years. Central nervous system (CNS) tumours were the most common (38%), followed by lymphomas (19%), soft tissue sarcomas (10%), renal tumours (9%) and neuroblastomas (9%). The five-year overall survival rate of all solid tumour patients was 84% (95% CI, 81-88%), 82% (95% CI, 76-89%) for CNS and 85% (95% CI, 80-90%) for non-CNS tumours. Advanced tumour stage at diagnosis predicted a poor prognosis.
CONCLUSION: The treatment results in our study are comparable with those previously published. A comprehensive local database allows for a timely follow-up of the characteristics and quality of treatment of childhood malignancies.

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Brain Stem Glioma - Childhood

Di Cataldo A, Agodi A, Balaguer J, et al.
Metastatic neuroblastoma in infants: are survival rates excellent only within the stringent framework of clinical trials?
Clin Transl Oncol. 2017; 19(1):76-83 [PubMed] Related Publications

INTRODUCTION: SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population.
MATERIALS AND METHODS: Italian and Spanish metastatic INES patients' data are reported. SPSS 20.0 was used for statistical analysis.
RESULTS: Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis.
CONCLUSIONS: The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data.

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MYCN (n-myc)

Vaassen P, Rosenbaum T
Nevus Anemicus As an Additional Diagnostic Marker of Neurofibromatosis Type 1 in Childhood.
Neuropediatrics. 2016; 47(3):190-3 [PubMed] Related Publications

Diagnosis of neurofibromatosis type 1 (NF1) can be established when at least two out of seven defined clinical findings are present. However, a definite clinical diagnosis may be challenging, especially in young children. Therefore, we tried to identify additional clinical signs suggestive of NF1. We observed that nevi anemici (NA) occur with increased frequency in NF1 patients. To establish NA as an additional diagnostic criterion for NF1 we evaluated their exact frequency in children potentially affected by NF1. During a 6-month period we examined 100 NF1 patients and documented patients' age and sex as well as presence, location, and characteristic features of NA. We were able to show that NA are present in 28% of NF1 patients, which is well above the 5% prevalence of NA in the general population. It is not known why NA appear with increased frequency in NF1. We hypothesize that an imbalance between α- and β-adrenergic receptors, resulting in increased α-adrenergic vasoconstriction might be the underlying cause of NF1-associated NA. Based on our own observations and previously published studies, we propose that NA in children with suspected NF1 might facilitate definite diagnosis and improve clinical management.

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Skin Cancer

Krishnatry R, Zhukova N, Guerreiro Stucklin AS, et al.
Clinical and treatment factors determining long-term outcomes for adult survivors of childhood low-grade glioma: A population-based study.
Cancer. 2016; 122(8):1261-9 [PubMed] Related Publications

BACKGROUND: The determinants of outcomes for adult survivors of pediatric low-grade glioma (PLGG) are largely unknown.
METHODS: This study collected population-based follow-up information for all PLGG patients diagnosed in Ontario, Canada from 1985 to 2012 (n = 1202) and determined factors affecting survival. The impact of upfront radiation treatment on overall survival (OS) was determined for a cohort of Ontario patients and an independent reference cohort from the Surveillance, Epidemiology, and End Results database.
RESULTS: At a median follow-up of 12.73 years (range, 0.02-33 years), only 93 deaths (7.7%) were recorded, and the 20-year OS rate was 90.1% ± 1.1%. Children with neurofibromatosis type 1 had excellent survival and no tumor-related deaths during adulthood. Adverse risk factors included pleomorphic xanthoastrocytoma (P < .001) and a thalamic location (P < .001). For patients with unresectable tumors surviving more than 5 years after the diagnosis, upfront radiotherapy was associated with an approximately 3-fold increased risk of overall late deaths (hazard ratio [HR], 3.3; 95% confidence interval [CI], 1.6-6.6; P = .001) and an approximately 4-fold increased risk of tumor-related deaths (HR, 4.4; 95% CI, 1.3-14.6; P = .013). In a multivariate analysis, radiotherapy was the most significant factor associated with late all-cause deaths (HR, 3.0; 95% CI, 1.3-7.0; P = .012) and tumor-related deaths (HR, 4.4; 95% CI, 1.3-14.6; P = 0.014). A similar association between radiotherapy and late deaths was observed in the independent reference cohort (P < .001). In contrast to early deaths, late mortality was associated not with PLGG progression but rather with tumor transformation and non-oncological causes.
CONCLUSIONS: The course of PLGG is associated with excellent long-term survival, but this is hampered by increased delayed mortality in patients receiving upfront radiotherapy. These observations should be considered when treatment options are being weighed for these patients.

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Childhood Brain Tumours

Childhood Brain Tumors

Bjerregaard LG, Aarestrup J, Gamborg M, et al.
Childhood height, adult height, and the risk of prostate cancer.
Cancer Causes Control. 2016; 27(4):561-7 [PubMed] Article available free on PMC after 01/07/2017 Related Publications

PURPOSE: We previously showed that childhood height is positively associated with prostate cancer risk. It is, however, unknown whether childhood height exerts its effects independently of or through adult height. We investigated whether and to what extent childhood height has a direct effect on the risk of prostate cancer apart from adult height.
METHODS: We included 5,871 men with height measured at ages 7 and 13 years in the Copenhagen School Health Records Register who also had adult (50-65 years) height measured in the Danish Diet, Cancer and Health study. Prostate cancer status was obtained through linkage to the Danish Cancer Registry. Direct and total effects of childhood height on prostate cancer risk were estimated from Cox regressions.
RESULTS: From 1996 to 2012, 429 prostate cancers occurred. Child and adult heights were positively and significantly associated with prostate cancer risk. When adjusted for adult height, height at age 7 years was no longer significantly associated with the risk of prostate cancer. Height at 13 years was significantly and positively associated with prostate cancer risk even when adult height was adjusted for; per height z-score the hazard ratio was 1.15 [95% confidence interval (CI) 1.01-1.32].
CONCLUSIONS: The effect of height at 13 years on the risk of prostate cancer was not entirely mediated through adult height, suggesting that child height and adult height may be associated with prostate cancer through different pathways.

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Prostate Cancer

García-Pérez J, Morales-Piga A, Gómez J, et al.
Association between residential proximity to environmental pollution sources and childhood renal tumors.
Environ Res. 2016; 147:405-14 [PubMed] Related Publications

BACKGROUND: Few risk factors for childhood renal tumors are well established. While a small fraction of cases might be attributable to susceptibility genes and congenital anomalies, the role of environmental factors needs to be assessed.
OBJECTIVES: To explore the possible association between residential proximity to environmental pollution sources (industrial and urban areas, and agricultural crops) and childhood renal cancer, taking into account industrial groups and toxic substances released.
METHODS: We conducted a population-based case-control study of childhood renal cancer in Spain, including 213 incident cases gathered from the Spanish Registry of Childhood Tumors (period 1996-2011), and 1278 controls individually matched by year of birth, sex, and region of residence. Distances were computed from the respective subject's residences to the 1271 industries, the 30 urban areas with ≥75,000 inhabitants, and the agricultural crops located in the study area. Using logistic regression, odds ratios (ORs) and 95% confidence intervals (95%CIs) for categories of distance to pollution sources were calculated, with adjustment for matching variables and socioeconomic confounders.
RESULTS: Excess risk (OR; 95%CI) of childhood renal tumors was observed for children living near (≤2.5km) industrial installations as a whole (1.97; 1.13-3.42) - particularly glass and mineral fibers (2.69; 1.19-6.08), galvanization (2.66; 1.14-6.22), hazardous waste (2.59; 1.25-5.37), ceramic (2.35; 1.06-5.21), surface treatment of metals (2.25; 1.24-4.08), organic chemical industry (2.22; 1.15-4.26), food and beverage sector (2.19; 1.18-4.07), urban and waste-water treatment plants (2.14; 1.07-4.30), and production and processing of metals (1.98; 1.03-3.82) -, and in the proximity of agricultural crops (3.16; 1.54-8.89 for children with percentage of crop surface ≥24.35% in a 1-km buffer around their residences).
CONCLUSIONS: Our study provides some epidemiological evidence that living near certain industrial areas and agricultural crops may be a risk factor for childhood renal cancer.

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Kidney Cancer

Simony SB, Lund LW, Erdmann F, et al.
Effect of socioeconomic position on survival after childhood cancer in Denmark.
Acta Oncol. 2016; 55(6):742-50 [PubMed] Related Publications

Background One fifth of all deaths among children in Europe are accounted for by cancer. If this is to be reduced there is a need for studies on not only biology and treatment approaches but also on how social factors influence cure rates. We investigated how various socioeconomic characteristics were associated with survival after childhood cancer. Material and methods In a nationwide cohort of 3797 children diagnosed with cancer [hematological cancer, central nervous system (CNS) tumors, non-CNS solid tumors] before age 20 between 1990 and 2009 we identified parents and siblings and obtained individual level parental socioeconomic variables and vital status through 2012 by linkage to population-based registries. Hazard ratios (HR) and 95% confidence intervals (CI) for dying were estimated using multivariate Cox proportional hazard models. Results For all children with cancer combined, survival was slightly but not statistically significantly better the higher the education of the mother or the father, and with maternal income. Significantly better survival was observed when parents were living together compared to living alone and worse survival when the child had siblings compared to none. Young (<20) or older (≥40) maternal age showed non-significant associations, but based on small numbers. For hematological cancers, no significant associations were observed. For CNS tumors, better survival was seen with parents living together (HR 0.70, CI 0.51-0.97). For non-CNS solid tumors, survival was better with high education of the mother (HR 0.66, CI 0.44-0.99) compared to basic and worse for children with one (HR 1.45, CI 1.11-1.89) or two or more siblings (HR 1.29, CI 0.93-1.79) (p for trend 0.02) compared to none. Conclusion The impact of socioeconomic characteristics on childhood cancer survival, despite equal access to protocolled and free-of-charge treatment, warrants further and more direct studies of underlying mechanisms in order to target these as a means to improve survival rates.

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Agulnik A, Forbes PW, Stenquist N, et al.
Validation of a Pediatric Early Warning Score in Hospitalized Pediatric Oncology and Hematopoietic Stem Cell Transplant Patients.
Pediatr Crit Care Med. 2016; 17(4):e146-53 [PubMed] Related Publications

OBJECTIVES: To evaluate the correlation of a Pediatric Early Warning Score with unplanned transfer to the PICU in hospitalized oncology and hematopoietic stem cell transplant patients.
DESIGN: We performed a retrospective matched case-control study, comparing the highest documented Pediatric Early Warning Score within 24 hours prior to unplanned PICU transfers in hospitalized pediatric oncology and hematopoietic stem cell transplant patients between September 2011 and December 2013. Controls were patients who remained on the inpatient unit and were matched 2:1 using age, condition (oncology vs hematopoietic stem cell transplant), and length of hospital stay. Pediatric Early Warning Scores were documented by nursing staff at least every 4 hours as part of routine care. Need for transfer was determined by a PICU physician called to evaluate the patient.
SETTING: A large tertiary/quaternary free-standing academic children's hospital.
PATIENTS: One hundred ten hospitalized pediatric oncology patients (42 oncology, 68 hematopoietic stem cell transplant) requiring unplanned PICU transfer and 220 matched controls.
INTERVENTIONS: None.
MEASUREMENTS AND MAIN RESULTS: Using the highest score in the 24 hours prior to transfer for cases and a matched time period for controls, the Pediatric Early Warning Score was highly correlated with the need for PICU transfer overall (area under the receiver operating characteristic = 0.96), and in the oncology and hematopoietic stem cell transplant groups individually (area under the receiver operating characteristic = 0.95 and 0.96, respectively). The difference in Pediatric Early Warning Score results between the cases and controls was noted as early as 24 hours prior to PICU admission. Seventeen patients died (15.4%). Patients with higher Pediatric Early Warning Scores prior to transfer had increased PICU mortality (p = 0.028) and length of stay (p = 0.004).
CONCLUSIONS: We demonstrate that our institution's Pediatric Early Warning Score is highly correlated with the need for unplanned PICU transfer in hospitalized oncology and hematopoietic stem cell transplant patients. Furthermore, we found an association between higher scores and PICU mortality. This is the first validation of a Pediatric Early Warning Score specific to the pediatric oncology and hematopoietic stem cell transplant populations, and supports the use of Pediatric Early Warning Scores as a method of early identification of clinical deterioration in this high-risk population.

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Child CJ, Zimmermann AG, Jia N, et al.
Assessment of Primary Cancer Incidence in Growth Hormone-Treated Children: Comparison of a Multinational Prospective Observational Study with Population Databases.
Horm Res Paediatr. 2016; 85(3):198-206 [PubMed] Related Publications

BACKGROUND/AIMS: Although results of the majority of clinical studies have shown no association between growth hormone (GH) treatment in childhood and risk of primary cancer, concerns remain regarding the potential influence of GH therapy on neoplastic cell growth. This study evaluated the incidence of primary malignancies in a large observational study of paediatric GH treatment.
METHODS: Primary cancer incidence was assessed in a cohort of 19,054 GH-treated children without a reported prestudy history of malignancy in the observational Genetics and Neuroendocrinology of Short Stature International Study (GeNeSIS). The standardised incidence ratio (SIR) for primary cancer in GH-treated children was determined by comparing cancer incidence in the GeNeSIS study population with incidence rates for country-, age-, and sex-matched cohorts of the general population.
RESULTS: During a mean follow-up of 3.4 years in GeNeSIS (64,705 person-years), 13 incident potential primary cancers were identified in GH-treated patients. The SIR (95% confidence interval) for all observed cancers was 1.02 (0.54-1.75), and the crude incidence was 20.1 (10.7-34.4) cases per 100,000 person-years.
CONCLUSION: Acknowledging the relatively short follow-up in our study, GH-treated children without a history of previous malignancy did not have a higher risk of all-site primary cancer during the study when compared to general-population cancer registries.

Jastaniah W, Elimam N, Abdalla K, et al.
Comparison of clinical trial versus non-clinical trial treatment outcomes of childhood acute lymphoblastic leukemia using comparable regimens.
Hematology. 2016; 21(3):175-81 [PubMed] Related Publications

OBJECTIVES: Treatment regimens tested in major clinical trials, conducted by cooperative groups, are often adapted as standard of care by cancer centers with the hope to replicate the treatment outcomes reported in these landmark studies. It is therefore postulated that applying clinical trial regimens in a non-clinical trial setting yield similar outcomes. The aim of the present study was to explore this hypothesis in the context of childhood acute lymphoblastic leukemia (ALL) in our institution.
METHODS: We retrospectively evaluated 224 consecutive pediatric ALL cases treated between January 2001 and December 2007. Standard-risk (SR) patients were treated on CCG-1991 (regimen OD) while high-risk (HR) patients were treated on CCG-1961 (regimen D). Results were compared with those of the equivalent regimen in the original clinical trials. Statistical analysis was carried using chi-square or Fisher's exact test, Kaplan-Meier and log-rank tests.
RESULTS: Comparison of treatment outcomes revealed that SR patients had inferior 5-year overall survival (OS) of (89.0 ± 2.9 vs. 96.0% ± 0.9%); event-free survival of (82.3 ± 3.5 vs. 88.7% ± 1.4%); and relapse rate of (15.8 vs. 9.3% (P = 0.034)) compared to patients treated in the clinical trial. However, no statistically significant difference in treatment outcomes was observed between HR patients.
CONCLUSIONS: Despite using comparable regimens, suboptimal outcomes were noted in SR patients implying that similar treatments do not necessarily yield similar outcomes. This underscores the need to evaluate outcomes of adapted regimens to identify areas that need further improvement in centers not enrolling patients on prospective collaborative clinical trials.

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Berrington de Gonzalez A, Salotti JA, McHugh K, et al.
Relationship between paediatric CT scans and subsequent risk of leukaemia and brain tumours: assessment of the impact of underlying conditions.
Br J Cancer. 2016; 114(4):388-94 [PubMed] Article available free on PMC after 01/07/2017 Related Publications

BACKGROUND: We previously reported evidence of a dose-response relationship between ionising-radiation exposure from paediatric computed tomography (CT) scans and the risk of leukaemia and brain tumours in a large UK cohort. Underlying unreported conditions could have introduced bias into these findings.
METHODS: We collected and reviewed additional clinical information from radiology information systems (RIS) databases, underlying cause of death and pathology reports. We conducted sensitivity analyses excluding participants with cancer-predisposing conditions or previous unreported cancers and compared the dose-response analyses with our original results.
RESULTS: We obtained information from the RIS and death certificates for about 40% of the cohort (n∼180 000) and found cancer-predisposing conditions in 4 out of 74 leukaemia/myelodysplastic syndrome (MDS) cases and 13 out of 135 brain tumour cases. As these conditions were unrelated to CT exposure, exclusion of these participants did not alter the dose-response relationships. We found evidence of previous unreported cancers in 2 leukaemia/MDS cases, 7 brain tumour cases and 232 in non-cases. These previous cancers were related to increased number of CTs. Exclusion of these cancers reduced the excess relative risk per mGy by 15% from 0.036 to 0.033 for leukaemia/MDS (P-trend=0.02) and by 30% from 0.023 to 0.016 (P-trend<0.0001) for brain tumours. When we included pathology reports we had additional clinical information for 90% of the cases. Additional exclusions from these reports further reduced the risk estimates, but this sensitivity analysis may have underestimated risks as reports were only available for cases.
CONCLUSIONS: Although there was evidence of some bias in our original risk estimates, re-analysis of the cohort with additional clinical data still showed an increased cancer risk after low-dose radiation exposure from CT scans in young patients.

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Childhood Brain Tumours

Childhood Brain Tumors

Leukemia - Molecular Biology

Childhood Leukaemia

Lau CS, Mahendraraj K, Ward A, Chamberlain RS
Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).
Pediatr Neurosurg. 2016; 51(3):127-36 [PubMed] Related Publications

BACKGROUND/AIMS: Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine factors impacting prognosis and survival.
METHODS: Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011).
RESULTS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005).
CONCLUSIONS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.

Berger M, Lanino E, Cesaro S, et al.
Feasibility and Outcome of Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant High-Dose Cyclophosphamide for Children and Adolescents with Hematologic Malignancies: An AIEOP-GITMO Retrospective Multicenter Study.
Biol Blood Marrow Transplant. 2016; 22(5):902-9 [PubMed] Related Publications

Post-transplant high-dose cyclophosphamide (PTCy) is a novel approach to prevent graft-versus-host disease (GVHD) and rejection in patients given haploidentical hematopoietic stem cell transplantation (HSCT). Thirty-three patients with high-risk hematologic malignancies and lacking a match-related or -unrelated donor were treated with PTCy haploidentical HSCT in 5 Italian AIEOP centers. Nineteen patients had a nonmyeloablative preparative regimen (57%), and 14 patients received a full myeloablative conditioning regimen (43%). No patients received serotherapy; GVHD prophylaxis was based on PTCy (50 mg/kg on days +3 and +4) combined with mycophenolate plus tacrolimus or cyclosporine A. Neutrophil and platelet engraftment was achieved on days +17 (range, 14 to 37) and +27 (range, 16 to 71). One patient had autologous reconstitution for anti-HLA antibodies. Acute GVHD grades II to IV and III to IV and chronic GVHD developed in 22% (95% CI, 11 to 42), 3% (95% CI, 0 to 21), and 4% (95% CI, 0 to 27) of cases, respectively. The 1-year overall survival rate was 72% (95% CI, 56 to 88), progression-free survival rate was 61% (95% CI, 43 to 80), cumulative incidence of relapse was 24% (95% CI, 13 to 44), and transplant-related mortality was 9% (95% CI, 3 to 26). The univariate analysis for risk of relapse incidence showed how 3 significant variables, mother as donor (P = .02), donor gender as female (P = .04), and patient gender as female (P = .02), were significantly associated with a lower risk of relapse. Disease progression was the main cause of death. PTCy is a safe procedure also for children and adolescents who have already received several lines of chemotherapy. Among the different diseases, a trend for better 1-year rates of overall survival was obtained for nonacute leukemia patients.

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Cyclophosphamide

Haematological Malignancies & Realted Disorders

Jain S, Kapoor G, Bajpai R
ABVD-Based Therapy for Hodgkin Lymphoma in Children and Adolescents: Lessons Learnt in a Tertiary Care Oncology Center in a Developing Country.
Pediatr Blood Cancer. 2016; 63(6):1024-30 [PubMed] Related Publications

BACKGROUND: As Hodgkin lymphoma (HL) is a highly curable malignancy, most current pediatric trials focus on strategies aimed at reducing late effects of therapy. We report our results with doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD) therapy.
PROCEDURE: We retrospectively analyzed 17 years (1996-2013) data of patients ≤18 years of age with HL. All patients received ABVD chemotherapy and involved field radiotherapy (IFRT) was reserved for those with bulky disease or partial response. The analysis was carried out to assess overall survival (OS) and freedom from treatment failure (FFTF) and factors predicting the events.
RESULTS: Of 167 eligible patients, 72 (43.1%) had B symptoms, 28 (16.7%) had bulky disease, 31 (18.6%) had >4 lymph node regions, and 53 (31.8%) had advanced disease (stages III and IV). In all, 87% patients received six cycles of ABVD and IFRT was administered to 51 (30.5%) patients. The 5-year OS and FFTF were 95.9% and 79%, respectively, and were similar in patients treated with or without IFRT. On multivariable analysis, advanced disease (stages III and IV), involvement of >4 lymph node regions, and serum lactate dehydrogenase (LDH) ≥500 IU/l at diagnosis were statistically significant factors for FFTF (P = 0.03, 0.003, 0.048, respectively).
CONCLUSIONS: The excellent survival of HL patients in the setting of a developing country reported in this retrospective analysis warrants treatment reduction, especially for early-stage patients. The use of risk- and response-based stratification incorporating disease stage, involved lymph node regions, and serum LDH, along with fluorodeoxyglucose-positron emission tomography-based response, may guide development of effective and less toxic protocols.

Related:

Childhood Hodgkin's Lymphoma

Hodgkin Lymphoma - Molecular Biology

Vinblastine

Al-Jamal RT, Cassoux N, Desjardins L, et al.
The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group.
Ophthalmology. 2016; 123(4):898-907 [PubMed] Related Publications

PURPOSE: To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI.
DESIGN: Retrospective, multicenter observational study.
PARTICIPANTS: Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults.
METHODS: Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression.
MAIN OUTCOME MEASURES: Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality.
RESULTS: Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival.
CONCLUSIONS: This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.

Related:

Melanoma

Ocular Melanoma

IntraOcular Melanoma

Ostroumova E, Hatch M, Brenner A, et al.
Non-thyroid cancer incidence in Belarusian residents exposed to Chernobyl fallout in childhood and adolescence: Standardized Incidence Ratio analysis, 1997-2011.
Environ Res. 2016; 147:44-9 [PubMed] Article available free on PMC after 01/05/2017 Related Publications

BACKGROUND: While an increased risk of thyroid cancer from post-Chernobyl exposure to Iodine-131 (I-131) in children and adolescents has been well-documented, risks of other cancers or leukemia as a result of residence in radioactively contaminated areas remain uncertain.
METHODS: We studied non-thyroid cancer incidence in a cohort of about 12,000 individuals from Belarus exposed under age of 18 years to Chernobyl fallout (median age at the time of Chernobyl accident of 7.9 years). During 15 years of follow-up from1997 through 2011, 54 incident cancers excluding thyroid were identified in the study cohort with 142,968 person-years at risk. We performed Standardized Incidence Ratio (SIR) analysis of all solid cancers excluding thyroid (n=42), of leukemia (n=6) and of lymphoma (n=6).
RESULTS: We found no significant increase in the incidence of non-thyroid solid cancer (SIR=0.83, 95% Confidence Interval [CI]: 0.61; 1.11), lymphoma (SIR=0.66, 95% CI: 0.26; 1.33) or leukemia (SIR=1.78, 95% CI: 0.71; 3.61) in the study cohort as compared with the sex-, age- and calendar-time-specific national rates. These findings may in part reflect the relatively young age of study subjects (median attained age of 33.4 years), and long latency for some radiation-related solid cancers.
CONCLUSIONS: We found no evidence of statistically significant increases in solid cancer, lymphoma and leukemia incidence 25 years after childhood exposure in the study cohort; however, it is important to continue follow-up non-thyroid cancers in individuals exposed to low-level radiation at radiosensitive ages.

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