The aim of this study is to describe childhood cancer incidence and survival in Castilla y León (Spain) for the period 2003 to 2014 and to explore differences between rural and urban areas.We made a cohort study in the childhood population of our region for the period of years referred before. Age-adjusted incidence rates to the world standard population (ASRw) were calculated by direct method, and their comparisons were made using incidence rate rations. Survival proportions were calculated by Kaplan-Meier method and their comparisons with log-rank test. The median childhood population less than 15 years old was 296,776 children. A total of 615 cases were recorded from the population-based Childhood Cancer Registry, including all malignant and benign tumors of the central nervous system.Age-standardized incidence rates for all cancers were 176.6 per million. Leukemia incidence rates were highest in rural areas (51.08/million) than in urban areas (33.65/million; P = .018), and by age groups; these differences only remained at age 0 to 4 years with higher rural leukemia incidence (67.13/million) than in urban areas (39.32/million; P = .05). There were no statistically significant differences between rural and urban areas for lymphomas, central nervous system, and all other malignant solid tumors grouped. The 5-year overall survival rate for all patients was 84%, similar to other developed countries, with greater survival in rural areas (88%) compared with urban areas (80%; P = .033). The analysis by tumor groups showed a greater survival rate in rural areas for all the groups, although these differences only reached statistical significance in the group of leukemias, with a survival rate of 90% for rural areas compared with 76% for urban areas (P = .01). Analyzing survival rate by age groups in leukemias only significant survival differences at 10 to 14 years were encountered.We found a higher incidence of leukemia in girls, mainly in rural areas, and also a better survival rate in children diagnosed with leukemia belonging to this population area. Future studies that analyze these facts in similar populations can help us clarify what genetic, epigenetic and environmental factors influence our population and are responsible for these findings.

Acrocephaly is a common neonatal craniofacial malformation. Saethre-Chotzen syndrome (SCS) is one of the acrocephaly related syndromes less frequently described in the literature. A female newborn term was admitted to our Neonatal Unit to study craniofacial dysmorphia without family history of interest. Pregnancy, childbirth and the neonatal period were uneventful. She had exotropia, short anterior-posterior cranial diameter, flat occiput and wide normotensive anterior fontanelle (beginning at the nose root, continuing through the sagittal suture with the posterior fontanelle) without syndactyly. The scanner imaging confirmed an acrocephaly with fusion of bilateral coronal sutures. We initially suspected a cranyosinostosis due to a Crouzon syndrome or SCS. After differential diagnosis and genetic study the patient was diagnosed as having SCS due to a de novo TWIST1 gene mutation. The craniofacial dysmorphias were corrected early by neurosurgical with good results. This case shows a new example of the phenotypic and genotypic variability of these TWIST1 gene mutations.

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