Proton therapy following induction chemotherapy for pediatric and adolescent nasopharyngeal carcinoma.

Purpose: In children treated for nasopharyngeal carcinoma, proton therapy and postchemotherapy target volumes can reduce the radiation dose to developing tissue in the brain and the skull base region. We analyzed outcomes in children with nasopharyngeal carcinoma treated with induction chemotherapy followed by moderate-dose proton therapy.

Methods/materials: Seventeen patients with nonmetastatic nonkeratinizing undifferentiated/poorly differentiated nasopharyngeal carcinoma underwent double-scattered proton therapy between 2011 and 2017.

Acromegaly, growth hormone and cancer risk.

Acromegaly is an endocrine disorder characterized by sustained hypersecretion of growth hormone (GH) with concomitant elevation of insulin-like growth factor I (IGF-I) associated with premature mortality from cardiopulmonary diseases and certain malignancies. In particular, there is a two-fold increased risk of developing colorectal cancer. Possible mechanisms underlying this association include elevated levels of circulating GH and IGF-I, but several other plausible processes may be relevant.

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.

Reassessment of growth hormone status is required at final height in children treated with growth hormone replacement after radiation therapy.

The most appropriate way to manage GH replacement in the transition period to adulthood in children treated with GH for GH deficiency (GHD) is controversial. The Growth Hormone Research Society suggests that the retesting of GH status at final height (FH) is unnecessary in the presence of severe organic GHD, and cranial irradiation falls into this etiological category. This recommendation has never been validated.

Improvements in final height over 25 years in growth hormone (GH)-deficient childhood survivors of brain tumors receiving GH replacement.

Final height (FH) outcome is important in survivors of childhood brain tumors. GH replacement is indicated in those found to be GH deficient (GHD). More recently, GnRH analogs (GnRHa) have been introduced to delay early or rapidly progressing puberty to allow more time for linear growth.

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.