Adrenocortical neoplasms in children: Radiologic-pathologic correlation

Primary neoplasms of the adrenal cortex are rare in children and differ sig nificantly in epidemiology, clinical characteristics, and biologic features from their counterparts in adults. In children, the inclusive term adrenoc ortical neoplasm is applied because adrenal adenoma and adrenal carcinoma m ay be difficult to distinguish histopathologically. Pediatric adrenocortica l neoplasms typically occur before 5 years of age, affect young girls more commonly than boys, and are associated with hemihypertrophy and Beckwith-Wi edemann and Li-Fraumeni syndromes. Most children with an adrenocortical neo plasm present with signs and symptoms of endocrine abnormality, including v irilization and Gushing syndrome. Cross-sectional imaging studies typically demonstrate a large, circumscribed, predominantly solid suprarenal mass wi th variable heterogeneity due to hemorrhage and necrosis. Calcification is not uncommon. Local invasion and metastases to the lungs, liver, and region al lymph nodes may be present at diagnosis. When friable tumor thrombus ext ends into the inferior vena cava, it poses a high risk of pulmonary emboliz ation, The finding Of increased retroperitoneal fat due to hypercortisolism on computed tomographic and magnetic resonance images of children with an adrenal mass favors the diagnosis of adrenocortical neoplasm, Surgical rese ction is the mainstay of therapy, with chemotherapy used for patients with metastases or persistent elevated hormone levels following surgery. Patient s younger than 5 years with aggressive adrenocortical neoplasms fare better than older children.