119increased bone collagen breakdown. In addition, cortisol-mediated suppression
of hypothalamo–pituitary–gonadal axis and alterations in calcium–vitamin D
homeostasis (decreased calcium absorption and hypercalciuria) also contrib-
utes to poor linear growth.
Why is growth failure more common in children with Cushing ’ s disease as com-
pared to Cushing ’ s syndrome due to adrenocortical tumors?
Growth retardation is more common in children with Cushing’s disease as com-
pared to those with adrenocortical tumors. This is because of long lag time prior
to diagnosis of Cushing’s disease in children. Majority of adrenocortical tumors
in children are malignant and have short lag time prior to diagnosis. In addition,
concurrent androgen excess in children with adrenocortical carcinoma may
result in normal/accelerated growth velocity.
How does hypercortisolemia affect pubertal development?
Puberty is commonly delayed in children with CS due to the inhibitory
effect of cortisol on hypothalamo–pituitary–gonadal axis. However, patients
with concurrent androgen excess due to androgen and cortisol co-secreting
adrenal tumors may present with heterosexual precocity in girls and iso-
sexual precocity in boys (gonadotropin-independent precocious puberty).
Children with Cushing’s disease may also have increased adrenal androgen
secretion and may have premature pubarche; however, gonadarche is
delayed.- What are the characteristics of adrenocortical tumors in children?
Adrenocortical tumors are rare in children and account for only 0.3–0.4 % of all
neoplasms in childhood, except in southern Brazil where the incidence is high.
Majority of adrenocortical tumors in children are functional (90 %) and malig-
nant (88 %). Among the functional tumors, 61 % have features of androgen
excess, 33 % have both androgen and cortisol excess, and 6 % of patients have
isolated cortisol excess. Cortisol-secreting adrenal tumors are common during
infancy and early childhood with a peak incidence at 4.5 years. Androgen-
secreting adrenocortical tumors may present with heterosexual precocity in
girls and isosexual precocity in boys.- What are the disorders associated with adrenocortical tumors in children?
Li–Fraumeni, Beckwith–Wiedemann, isolated hemihypertrophy syndromes,
and, rarely, MEN 1 are associated with adrenocortical tumors in children.4 Childhood Cushing’s Syndrome