Adrenocortical carcinoma during pregnancy

Adrenocortical carcinoma (ACC) is a rare malignant neoplasm. Hypercortisolism and inhibition of gonadotropin secretion usually result in menstrual disorders and secondary amenorrhea. The coincidence of ACC and pregnancy is therefore extremely rare. The signs of hypercortisolism are commonly seen in otherwise healthy pregnancies, which decreases the doctor's vigilance. We present the diagnostic challenges and current treatment recommendations according to European guidelines from the European Society of Endocrinology (ESE) and European Network for the Study of Adrenal Tumors (ENS@T) 2018 and Polish guidelines 2024.

We conducted an extensive search via MEDLINE using the phrases "Adrenocortical carcinoma", "ACC", and "Pregnancy" without temporal or language restrictions. Only cases with ACC diagnosed during pregnancy were taken into consideration. Ten papers were found, with 12 described cases. We analyzed the management and outcome both for the mother and the child. We also included a case of a woman treated in our department. A 29-year-old woman in the 20th/21st gestation week (GW) presented to us with Cushing's syndrome symptoms and androgenization. Laboratory tests showed low plasma adrenocorticotropic hormone (ACTH), high cortisol, testosterone, dehydroepiandrosterone sulfate (DHEA-SO4), androstenedione, 24-hour urinary free cortisol (UFC), and hypokalemia. In the abdominal magnetic resonance imaging (MRI) there was a mass in the left adrenal gland. An open surgery was performed in the 21st GW with no perioperative complications. The pathology report established the diagnosis of ACC. The tumor board along with the patient decided to defer the adjuvant therapy until the 32nd GW to increase the odds for the fetus to survive. In the 31st GW an urgent caesarian section was performed due to risk of fetal hypoxia. Computed tomography (CT) scan after the delivery showed local recurrence in the tumor bed. The patient was qualified to mitotane therapy and underwent tumor bed radiotherapy followed by chemotherapy, but the treatment did not stop the progression of the disease. She passed away 14 months after the diagnosis.

It is critical to remember about the possibility of ACC occurrence during pregnancy, as well as to know about the differences in hormonal tests in pregnant women such as higher free plasma cortisol, ACTH, UFC, and high rate of false-positive results of low-dose dexamethasone suppression test (LDDST) in comparison to non-pregnant women. Therapeutical options are scarce and pose an ethical dilemma.