Novel Insights Large Estrogen and Cortisol Co-Secreting Adrenocortical Tumor in a 17-Year-Old Female: A Case Report...

Adrenocortical tumors (ACTs) are rare pediatric tumors which include both benign adrenocortical adenomas (ACAs) and malignant adrenocortical carcinomas (ACCs). The most common presentations of an ACT in children are virilization and/or Cushing's syndrome resulting from tumoral secretion of androgens and cortisol, respectively. Adrenocortical tumors that express high levels of estrogen are extremely rare, and adrenal tumors that co-secrete more than one class of adrenal hormones raise concern for malignancy.

We present a case of a 17-year-old female presenting with weight gain, secondary amenorrhea, and acute abdominal pain. She was found to have a large (9.4 cm) right adrenal tumor co-secreting both cortisol and estrogen, which was successfully surgically removed by minimally invasive laparoscopy. Her perioperative course was complicated by severe refractory hypertension requiring multiple antihypertensive medications, and venous thromboses associated with hypercoagulability. Genetic testing revealed no germline driver mutation. Classification of the adrenal tumor as either benign or malignant was discordant when determined by the adult Weiss vs. the pediatric AFIP/Wieneke criteria in this older adolescent.

This prismatic case of a rare estrogen-cortisol secreting tumor raises discussion regarding the complexities in the clinical presentation and uncertainties in prognosis of adrenal tumors in adolescents. A literature review showcases 19 case reports published between 1948 and 2025 of estrogen-secreting pediatric ACTs highlighting their rare prevalence. This case stresses the need for a multidisciplinary approach to diagnosis, treatment, and long-term management of this entity in children and adolescents.