Clinical characteristics of adrenocortical tumors in children.

BACKGROUND: Adrenocortical tumors (ACTs) are rare in children. Because of the rarity and various manifestations of ACTs, patients of ACTs are not easily diagnosed. Some patients were misdiagnosed before surgery.

OBJECTIVE: Identify the clinical, laboratorial, imaging and histopathological characteristics of adrenocortical tumors in children. Compare adrenalcortical adenoma with carcinoma.

METHODS: A retrospective review of 34 identified patients who were younger than 15 years old with histologic confirmation of adrenocortical carcinoma (ACC) or adenomas from 1991 to 2010.

RESULTS: In these 34 patients, 19 were adrenocortical adenoma (ACA) and 15 were ACC. The median age at diagnosis was 3.33 years (range, 0-16 years), and 70.6% of the patients were younger than five years. Girls slightly predominated over boys (1.4:1). For endocrine abnormality, 14 patients had isolated precocious puberty, five patients had isolated Cushing syndrome, 10 patients had precocious puberty plus Cushing syndrome, and five patients did not have any symptoms. The most frequent findings in laboratory tests were disturbance of the normal circadian rhythm of cortisol secretion (93.8%), followed by elevated serum level of testosterone (89.7%). Only 3.8% of ultrasound diagnosis and 12.1% of computed tomography (CT) diagnosis were consistent with pathologic diagnosis.

CONCLUSION: Different from those in adult, the most frequent presentation in children with ACTs is peripheral precocious puberty with or without Cushing syndrome, and isolated Cushing syndrome. Few present with non-functional local mass. Laboratory tests usually reveal the discordantly elevated serum levels of sexual corticosteroid hormones, change of diurnal rhythm of cortisol or increase of morning cortisol. The differentiation of malignant from benign tumor cannot merely depend on imaging. Final diagnosis relies on comprehensive evaluation of clinical manifestations, laboratory data, imaging and pathology.