Ramandeep S. Arora

Age–incidence patterns of primary CNS tumors in children, adolescents, and adults in England

Around 25% of all tumors in those 0-14 years of age and 9% in those 15-24 years of age involve the CNS. They are the most common cause of cancer-related deaths in both age groups. In adults 25-84 years of age, the proportion of CNS tumors is 2%; 5-year overall survival is 10%-15%; and survivors have considerable morbidity. Comprehensive up-to-date population-based incidence data on these tumors are lacking. We present incidence rates for primary CNS tumors based on data derived from the high-quality national cancer registration system in England. A total of 54,336 CNS tumors of malignant, benign, and uncertain behavior were registered across the whole of England from 1995 through 2003. The age-standardized rates for all ages (0-84 years) was 9.21 per 100,000 person-years. This is higher than previously reported for England because it includes nonmalignant CNS tumors and hence gives a more accurate picture of burden of disease. The age-standardized rates for those 0-14 years of age, 15-24 years of age, and 25-84 years of age were 3.56, 3.26, and 14.57 per 100,000 person-years, respectively. In this article, we describe the changing patterns in the epidemiology of primary CNS tumors in these three age groups with respect to sex, tumor behavior, and histology using the current WHO classification. This information will provide a reference for future studies nationally and internationally and make comparisons relevant and meaningful.

Comparative incidence patterns and trends of gonadal and extragonadal germ cell tumors in England, 1979 to 2003

It is believed that gonadal and extragonadal germ cell tumors (GCTs) arise from primordial germ cells and may have similar etiopathogenesis. Unlike testicular GCTs, there has been limited comprehensive population‐based analysis of ovarian and extragonadal GCTs.

Survival outcome of childhood acute lymphoblastic leukemia in India: a resource-limited perspective of more than 40 years.

The outcome of childhood acute lymphoblastic leukemia in India has been inferior to more than 80% cure rates in developed nations. This study was done to analyze the outcome of acute lymphoblastic leukemia in India over 4 decades. There has been a gradual improvement in survival rates of up to >70% in some centers along with a decline in relapse and mortality. However, these results cannot be generalized to the entire nation. There is a crying need to address treatment abandonment, take quality improvement, educational and financial initiatives; cooperative research into risk factors and disease biology, and the implementation of risk stratification along with the assessment of response to therapy.

Are reported increases in incidence of primary CNS tumours real? An analysis of longitudinal trends in England, 1979–2003

Reported increases in the incidence of CNS tumours in the developed world in the 1970s to 1990s have been a cause for concern and debate. It still remains to be adequately answered whether these increases are true or an artefact of changes in diagnostic and registration practices. Using high-quality national cancer registration data, we have analysed incidence trends for each major histological subgroup of CNS tumour (2000 World Health Organisation (WHO) classification) registered in those aged 0-84 years for the whole of England during the period 1979 through 2003. 134,509 primary CNS tumours of malignant, benign and uncertain behaviour located in the brain, meninges, spinal cord, cranial nerves, other parts of the central nervous system and in the pituitary and pineal glands were registered. In summary, we present the single largest nationwide study on the longitudinal incidence trends of CNS tumours. The increase in incidence observed in the 1970s and 1980s was mainly in the young and the elderly and has now plateaued and may even be decreasing. There is however variation in trends by histology. The incidence of some histological sub-groups has continued to increase until the most recent period of analysis. Much of the initial increase can be attributed to the emergence of much more widely available neuroimaging, while the most recent incidence changes for specific sub-groups of CNS tumours appear to be due to greater diagnostic specificity leading to a shift in registered categories. However, the trends for high-grade astrocytomas and other gliomas need further observation and investigation.

The contrasting age-incidence patterns of bone tumours in teenagers and young adults: Implications for aetiology

Bone tumours comprise 0.2% of cancers overall but 5.7% in 15–24 year olds. To explore the relationship with adolescence we have analysed age‐incidence patterns of bone tumours in a large national dataset. Data on incident cases of bone tumours in 0–84 year olds in England, 1979–2003, were extracted from national cancer registration data. Incidence rates per million person‐years by 5‐year age‐group, sex, morphology and primary site were calculated and adjusted to the world standard population. Nine thousand one hundred forty‐six cases were identified giving an overall age‐standardized rate of 7.19 per million person‐years. The distribution by morphology was: osteosarcoma, 34.2%; chondrosarcoma, 27.2%; Ewing sarcoma, 19.3%; other, 19.4%. The distribution varied by age. Ewing sarcoma was most common in 0–9 year olds, osteosarcoma in 10–29 year olds and chondrosarcoma in 30–84 year olds. 29.2% of all tumours occurred in 0–24 year olds. Highest incidence of osteosarcoma and Ewing sarcoma in females was in 10–14 year olds. In males, peak incidence occurred at 15–19 years and exceeded that in females. Chondrosarcoma incidence steadily increased with age. The proportions of Ewing sarcomas occurring in respective bones were consistent with those of the adult skeleton by weight. In osteosarcoma tumours of long bones of lower limb were markedly over‐represented in the adolescent peak, being six times more than at any other site. Variation in incidence patterns with age and site suggests pubertal bone growth to be a key factor in osteosarcoma while different biological pathways could be relevant for Ewing sarcoma.

Improving Care for Children With Cancer in Low- and Middle-Income Countries-A SIOP PODC Initiative

The Paediatric Oncology in Developing Countries (PODC) committee of International Society of Paediatric Oncology (SIOP) has 10 working groups that provide a forum for individuals to engage, network, and implement improvements in the care of children with cancer in low‐ and middle‐income countries. The development of adapted guidelines (medulloblastoma, retinoblastoma, Wilms tumor, neuroblastoma, retinoblastoma, Burkitt lymphoma, supportive care), advocacy and awareness (on hospital detention and essential drugs), education and training, and global mapping (nutritional practice, abandonment rates, and twinning collaborations) have been the initial areas of focus, and the impact of some of these activities is evident, for example, in the SIOP Africa PODC Collaborative Wilms tumor project.

Relationship between height at diagnosis and bone tumours in young people: a meta-analysis

ObjectiveSome evidence exists that patients with osteosarcoma and Ewing sarcoma are taller than the general population. However, previous studies are under-powered, lack comprehensive data and show inconsistencies.MethodsRelevant studies linking osteosarcoma and Ewing sarcoma with height at diagnosis were identified in two major online databases, Medline (1950 to 2009) and Embase (1980 to 2009). Outcomes in individual studies were reported as standard deviation (SD) scores or percentages of study population with height at diagnosis above the median of the reference population. We performed separate random-effects meta-analyses for each outcome and tumour type.Results14 studies examined the height of patients with osteosarcoma or Ewing sarcoma. Meta-analyses on SD scores found patients with osteosarcoma were 0.260 SD (95% CI: 0.088–0.432) taller than the reference population (five studies). A meta-analysis on percentages found 62% (95% CI: 57%–67%) of patients were estimated to have a height above the median (six studies). Patients with Ewing sarcoma were 0.096 SD (95% CI 0.004–0.188) taller (four studies). Only one study reported the percentage of Ewing sarcoma patients with height above the median.ConclusionThe average height of patients with osteosarcoma, but not Ewing sarcoma, was significantly above the average height of the reference population by 2–3 centimetres. The observed differences indicate the involvement of pubertal longitudinal bone growth in osteosarcoma development while different biological pathways could be relevant for Ewing sarcoma.

Determinants of Treatment Abandonment in Childhood Cancer: Results from a Global Survey

Background Understanding and addressing treatment abandonment (TxA) is crucial for bridging the pediatric cancer survival gap between high-income (HIC) and low-and middle-income countries (LMC). In childhood cancer, TxA is defined as failure to start or complete curative cancer therapy and known to be a complex phenomenon. With rising interest on causes and consequences of TxA in LMC, this study aimed to establish the lay-of-the-land regarding determinants of TxA globally, perform and promote comparative research, and raise awareness on this subject. Methods Physicians (medical oncologists, surgeons, and radiation therapists), nurses, social workers, and psychologists involved in care of children with cancer were approached through an online survey February-May 2012. Queries addressed social, economic, and treatment-related determinants of TxA. Free-text comments were collected. Descriptive and qualitative analyses were performed. Appraisal of overall frequency, burden, and predictors of TxA has been reported separately. Results 581 responses from 101 countries were obtained (contact rate = 26%, cooperation rate = 70%). Most respondents were physicians (86%), practicing pediatric hematology/oncology (86%) for >10 years (54%). Providers from LMC considered social/economic factors (families’ low socioeconomic status, low education, and long travel time), as most influential in increasing risk of TxA. Treatment-related considerations such as preference for complementary and alternative medicine and concerns about treatment adverse effects and toxicity, were perceived to play an important role in both LMC and HIC. Perceived prognosis seemed to mediate the role of other determinants such as diagnosis and treatment phase on TxA risk. For example, high-risk of TxA was most frequently reported when prognosis clearly worsened (i.e. lack of response to therapy, relapse), or conversely when the patient appeared improved (i.e. induction completed, mass removed), as well as before aggressive/mutilating surgery. Provider responses allowed development of an expanded conceptual model of determinants of TxA; one which illustrates established and emerging individual, family, center, and context specific factors to be considered in order to tackle this problem. Emerging factors included vulnerability, family dynamics, perceptions, center capacity, public awareness, and governmental healthcare financing, among others. Conclusion TxA is a complex and multifactorial phenomenon. With increased recognition of the role of TxA on global pediatric cancer outcomes, factors beyond social/economic status and beliefs have emerged. Our results provide insights regarding the role of established determinants of TxA in different geographical and economic contexts, allow probing of key determinants by deliberating their mechanisms, and allow building an expanded conceptual model of established and emerging determinants TxA.

Cancer at ages 15-29 years: the contrasting incidence in India and England

There has been a steady increase in published research from Europe and North America on the epidemiology of cancers in young people. There are limited data from the developing world. We contrast the incidence of cancer at ages 15–29 years in India and England.

Assessing the impact of paediatric oncology publications using three citation databases

Despite some reported limitations, Web of Science has been the standard source to assess the impact of individual articles, and consequently journals. By analysing the citations to articles published in the field of paediatric oncology, we demonstrate that Scopus and Google Scholar, the two new citation databases, retrieve more citations than Web of Science. The strength of Scopus lies in identifying non‐English literature from Western and Eastern Europe, while Google Scholar is proficient at identifying English and non‐English literature from Africa, Asia and Central and South America. These findings have implications for researchers, journals and health libraries. Pediatr Blood Cancer. 2010;56:152–153.