A Rare Case of Androgen- and Cortisol- Secreting Adrenal Adenoma in Childhood

Introduction: Adrenal tumors are rare in children, with neuroblastoma being the most common neoplasia. In this age group, a significant concern is the higher pre-test probability of malignancy. Adrenal adenomas are uncommon, with nonfunctioning adenomas accounting for only 0.5% of all adrenal incidentalomas. Androgen- and cortisol-cosecreting adenomas are exceptionally rare, with no case report in childhood to date. Case Presentation: We present a case of a 16-year-old female adolescent referred to the Endocrinology Department due to severe hirsutism and secondary amenorrhea. The patient exhibited obesity (BMI 32.98 kg/m2; +1.97 SD). Further workup revealed elevated levels of total testosterone (5.13 nmol/L [reference range: 0.61–1.77]), free testosterone (5.65 pmol/L [reference range: 0.06–0.58]), and a lack of serum cortisol suppression after overnight 1-mg dexamethasone suppression test (612 nmol/L [reference range: <50]). Adrenal imaging identified a solid, heterogeneous lesion in the right adrenal gland measuring 4.6 cm, with 26 Hounsfield units on CT and with loss of signal intensity on out-phase MRI. The patient underwent laparoscopic adrenalectomy, and histopathologic examination revealed an adrenocortical adenoma (Weiss Score 1; weight 55 g). Postoperatively, the patient developed symptoms consistent with glucocorticoid withdrawal syndrome. Conclusion: In contrast to adrenocortical carcinomas, androgen- and cortisol-cosecreting adenomas have a more prolonged clinical course. However, similar to carcinomas, large size and structural heterogeneity are characteristic features. Although benign, early diagnosis is crucial to prevent complications associated with prolonged exposure to hyperandrogenism and hypercortisolism during puberty.