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Adrenocortical tumors (ACT) develop from clonal transformation of an adrenal cortex cell and commonly displays signs and symptoms related to increased adrenocortical hormone production. However, in about 10% of pediatric ACT cases, these signs and symptoms are not present. Non-functioning ACT is generally not discovered until the tumor mass is sizable and patients complain of abdominal pain or discomfort from pressure applied to the abdominal organs.
Signs and symptoms associated with adrenocortical tumors vary significantly. The signs and symptoms of adrenal gland tumors may include, but are not limited to, early onset of pseudopuberty, such as secondary hair growth, increase in genital size, and voice changes, as well increased weight, high blood pressure, low potassium, palpitations, anxiety, and hyperglycemia. Signs and symptoms can often be defined by adrenocortical hormonal syndromes that are very often associated with ACTs (Ribeiro R et al. 2010; Rodriguez-Galindo C et al. 2005).
Hormonal syndromes associated with ACT are:
Signs and symptoms of virilization are the most common presenting endocrine features (>80% of patients) (Michalkiewica et al. 2004). Clinical manifestations of ACT can be present before or at birth. An acute abdomen due to spontaneous tumor rupture is rarely the presenting clinical manifestation of ACT. To avoid delaying the diagnosis of ACT, any child less than 4 years with pubarche should be considered to have ACT. Cushing syndrome should be considered highly indicative of ACT in children younger than 10 years.