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Sm_mary There is a well-known raised risk of leukaemia in children with neurofibromatosis type 1 (NF-1).We carried out the first detailed population-based study of leukaemia and non-Hodgkin lymphoma (NHL) associated with NF-I in order to estimate the risk and elucidate the relationship between these conditions. Over the 17 year study period there were five cases of chronic myelomonocytic leukaemia (CMML) in patients with NF-1 (relative risk 221; 95% CI 71-514), 12 cases of acute lymphoblastic leukaemia (ALL) (relative nrsk 5.4; 95% CI 2.8-9.4) and five cases of NHL (relative risk 10.0; 95% CI 3.3-23.4). Marrow cytogenetics could be reviewed for seven patients. Specific abnormalities found were monosomy 21 in a child with CMML and 7p+, 17p-in a child with ALL. No abnormalities were reported of 17q, which includes the NFI gene. CMML occurred predominantly in boys, who also had a family history of NF-1. ALL and NHL were more often found in children with no previous family history.

Clinical, morphologic, and cytogenetic features were examined in a group of 68 children with myelodysplasia (MDS) referred to a single institution between 1971–1991. The morphologic French-American-British (FAB) system of classification proved of limited value in this group of patients because 50% of the cases were categorized as chronic myelomonocytic leukemia and three patients with eosinophilia and MDS were unclassifiable. Cytogenetic analysis was performed in 63 cases and clonal abnormalities were detected in 55%; the most common chromosome involved was number 7. Modification of the FAB system to incorporate additional diagnostic features such as pretreatment fetal hemoglobin (Hb F) and cytogenetics allowed incorporation of the categories of juvenile chronic myeloid leukemia (JCML) and infantile monosomy 7 syndrome (IMo7). The resulting groups of patients had highly significant differences in survival (P = .00009). The overall 5-year survival for the patients was 31.9% (95% CI 21.7 to 44.1) and factors influencing prognosis included: modified FAB type, platelet count, Hb F level, and cytogenetic complexity. We developed a scoring system (“FPC”) where each of the following findings at diagnosis scored one point: HbF greater than 10%, platelets < or = 40 x 10(9)/L, and complex karyotypic changes (two or more clonal structural/numerical abnormalities), which produced groups with highly significant differences, patients with a score of 0 having a 5-year survival of 61.6% (CI 33% to 84%), whereas those with a score of two or three all died within 4 years of diagnosis. The revised classification and scoring system may prove helpful in making treatment choices in pediatric MDS and now needs to be tested prospectively in large scale population-based studies.

SummaryBackgroundGlobal inequalities in access to health care are reflected in differences in cancer survival. The CONCORD programme was designed to assess worldwide differences and trends in population-based cancer survival. In this population-based study, we aimed to estimate survival inequalities globally for several subtypes of childhood leukaemia.MethodsCancer registries participating in CONCORD were asked to submit tumour registrations for all children aged 0–14 years who were diagnosed with leukaemia between Jan 1, 1995, and Dec 31, 2009, and followed up until Dec 31, 2009. Haematological malignancies were defined by morphology codes in the International Classification of Diseases for Oncology, third revision. We excluded data from registries from which the data were judged to be less reliable, or included only lymphomas, and data from countries in which data for fewer than ten children were available for analysis. We also excluded records because of a missing date of birth, diagnosis, or last known vital status. We estimated 5-year net survival (ie, the probability of surviving at least 5 years after diagnosis, after controlling for deaths from other causes [background mortality]) for children by calendar period of diagnosis (1995–99, 2000–04, and 2005–09), sex, and age at diagnosis (<1, 1–4, 5–9, and 10–14 years, inclusive) using appropriate life tables. We estimated age-standardised net survival for international comparison of survival trends for precursor-cell acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML).FindingsWe analysed data from 89 828 children from 198 registries in 53 countries. During 1995–99, 5-year age-standardised net survival for all lymphoid leukaemias combined ranged from 10·6% (95% CI 3·1–18·2) in the Chinese registries to 86·8% (81·6–92·0) in Austria. International differences in 5-year survival for childhood leukaemia were still large as recently as 2005–09, when age-standardised survival for lymphoid leukaemias ranged from 52·4% (95% CI 42·8–61·9) in Cali, Colombia, to 91·6% (89·5–93·6) in the German registries, and for AML ranged from 33·3% (18·9–47·7) in Bulgaria to 78·2% (72·0–84·3) in German registries. Survival from precursor-cell ALL was very close to that of all lymphoid leukaemias combined, with similar variation. In most countries, survival from AML improved more than survival from ALL between 2000–04 and 2005–09. Survival for each type of leukaemia varied markedly with age: survival was highest for children aged 1–4 and 5–9 years, and lowest for infants (younger than 1 year). There was no systematic difference in survival between boys and girls.InterpretationGlobal inequalities in survival from childhood leukaemia have narrowed with time but remain very wide for both ALL and AML. These results provide useful information for health policy makers on the effectiveness of health-care systems and for cancer policy makers to reduce inequalities in childhood cancer survival.FundingCanadian Partnership Against Cancer, Cancer Focus Northern Ireland, Cancer In...

S_.mary A review was carried out of the published literature on survival rates for cancer in relation to patterns of organisation of medical care, specifically treatment at specialist centres or at hospitals treating larger numbers of patients and treatment by protocol, usually within the context of a clinical trial. Centralised referral or entry to trials was frequently associated with a higher survival rate, particularly for the less common cancers, and was never found to be associated with a lower survival rate. Few (Chouillet et al., 1994). Analyses of survival in relation to organisational factors have yet to appear from either of these studies.Finally, for 1984 onwards two electronic databases, Medline and Embase, were searched on the Silver Platter Cancer CD. Tables I-XI contain 32 references included on Cancer CD and already known to the author before searching began. Of these, 14 (44%) could be retrieved by a search of medical subject headings (MESH) containing at least one of the terms survival, outcome, prognosis, mortality and at least one of hospitals, protocols, cancer care facilities, referral, health services. Four additional references not previously known were found in this search, making a total of 18 publications, and these accounted for 2.8% of the total of 649 references so indexed. Extending the search to other fields or by additional keywords increased the total number of references retrieved by a much greater proportion than it did the number of relevant publications found. This is a considerably worse result than that found by Silagy (1993)