A 21 year old female with a new diagnosis of hypertension, eight months post-partum following an uncomplicated pregnancy. Symptomatology included headache, palpitations, increased appetite, bruising and hirsutism. Imaging identified a large left adrenal mass (6.5cm) and multiple lung nodules. Hypertension was managed with phenoxybenzamine and propranolol initially for possible phaeochromocytoma. Endocrine investigations showed normal aldosterone:renin ratio, normal plasma and urine metanephrines and normetanephrines, normal DHEA and androstenedione. However, morning cortisol was elevated, with elevated 24-hour urinary free cortisol:creatinine ratio, and elevated midnight cortisol with a suppressed midnight ACTH. MIBG scan showed no uptake, whereas PET scan demonstrated a metabolically active left adrenal mass and innumerable metabolically active pulmonary metastases.
Video-assisted thoracosocopic surgery for biopsy of lung metastases confirmed metastatic adrenocortical carcinoma (Ki67 60%, IGF2 positive, IGF-1R positive, ER negative, PR positive). In the absence of symptoms or signs of Cushing’s syndrome in pregnancy, this 21 year old female had a rapidly progressive cortisol-secreting adrenocortical carcinoma (ACC) post-partum.
Blood pressure control was achieved with spironolactone and nifedipine with weaning of alpha blockade. Ketoconazole was initiated then changed to metyrapone due to worsening acne and hirsutism. The patient was planned for palliative chemotherapy with etoposide, doxorubicin and cisplatin with mitotane. However, the patient passed away within two months of her initial hospital admission.
There are 24 cases described in the literature of ACC arising in pregnancy or post-partum. According to one study, ACC arising in pregnancy and post-partum are associated with worse overall survival than ACC diagnosed in women who have not been pregnant (1). A proposed mechanism for this difference is the stimulation of hormone receptors on adrenal tumours during pregnancy.