Thelansis

 Pediatric Adrenocortical Cancer (ACC) is a rare neoplasm that affects the adrenal glands in children. The adrenal glands, located above the kidneys, produce hormones that regulate various physiological processes, including blood pressure, heart rate, and metabolism. Clinical presentation of pediatric ACC includes weight gain, hypertension, and changes in skin pigmentation. The genomic landscape of pediatric ACC is characterised by copy-neutral loss of heterozygosity on chromosomes 11 and 17, which is associated with germline TP53 pathogenic variants, insulin-like growth factor-2 overexpression, and somatic mutations in ATRX and CTNNB1. Li-Fraumeni Syndrome, an autosomal dominant familial cancer syndrome, is most commonly associated with pediatric ACC. Pediatric ACCs are more likely to be functional, often presenting with excess androgen production. The 5-year survival rate for pediatric ACC ranges from 30-70%, depending on disease presentation. The bimodal age distribution of pedi...