Bone mineral density in childhood survivors of acute lymphoblastic leukemia treated without cranial irradiation.

Adult survivors of childhood acute lymphoblastic leukemia (ALL) whose treatment included cranial irradiation (XRT) have reduced bone mineral density (BMD). Fifty-three survivors of ALL (aged 6-17 yr; 22 males and 31 females), who had completed their treatment without XRT, at least 1 yr previously, and 187 (5-19 yr; 86 males and 101 females) healthy controls were examined with dual energy x-ray absorptiometry of the total body and L1-L4 vertebrae and peripheral quantitative computer tomography at the distal and midradial sites. The total body and lumbar spine BMDs did not differ between the ALL survivors and controls.

Rhabdomyosarcoma in Nijmegen breakage syndrome: strong association with perianal primary site.

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disorder resulting from mutations in the NBS1 gene, which encodes for the DNA double strand break repair protein nibrin. NBS is clinically characterized by microcephaly, dysmorphic features, immunodeficiency, and increased susceptibility to malignancy, mainly of lymphoid origin. Here, we describe a 7-year-old girl with NBS who is homozygous for the NBS1 698del4 mutation.

Puberty in children with cancer.

Cancer may impinge on puberty either directly through a mass lesion effect on the reproductive axis or indirectly through hormones secreted by tumours, for example human chorionic gonadotrophin, or weight loss, or the actual presence of a chronic disease process per se. The more frequent pubertal problems faced by children with cancer are due to the impact of treatment either on the central nervous system, the hypothalamic-pituitary axis or the gonad; in this review, we concentrate on these complications and their potential management.