Others
Growth hormone is another option that has been described, however it should only be used in proven growth hormone deficiency such as idiopathic short stature. Children with a constitutional delay have not been shown to benefit from growth hormone therapy. Although serum growth hormone levels are low in constitutional delay of puberty, they increase after treatment with sex hormones and in those cases, growth hormone is not suggested to accelerate growth.Subnormal vitamin A intake is one of the etiological factors in delayed pubertal maturation. Supplementation of both vitamin A and iron to normal constitutionally delayed children with subnormal vitamin A intake is as efficacious as hormonal therapy in the induction of growth and puberty.More therapies are being developed to target the more discreet modulators of the HPG axis including kisspeptin and neurokinin B.In cases of severe delayed puberty secondary to hypogonadism, evaluation by a psychologist or psychiatrist, as well as counseling and a supportive environment are an important supplemental therapy for the child. Transition from pediatric to adult care is also vital as many children are lost during transition of care. Outlook
Constitutional delay of growth and puberty is a variation of normal development with no long-term health consequences, however it can have lasting psychological effects. Adolescent boys with delayed puberty have a higher level of anxiety and depression relative to their peers.
Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%.It gets its name because the majority of the cells in the tumor are derived from neuroectoderm, but have not developed and differentiated in the way a normal neuron would, and so the cells appear "primitive".PNET belongs to the Ewing family of tumors. Genetics
Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis. The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 Large T- antigen, other than p53. Diagnosis
Classification
It is classified into two types, based on location in the body: peripheral PNET and CNS PNET. Peripheral PNET
The peripheral PNET (pPNET) is now thought to be virtually identical to Ewing sarcoma:
"Current evidence indicates that both Ewings sarcoma and PNET have a similar neural phenotype and, because they share an identical chromosome translocation, they should be viewed as the same tumor, differing only in their degree of neural differentiation.