PSAT005 The Third Twin: A Rare Presentation of Adrenocortical Carcinoma

Abstract

Abstract Introduction Adrenocortical carcinoma (ACC) is rare, even more so during pregnancy. It is frequently aggressive and carries an extremely poor prognosis. Although it is limited to < 2% of adrenal incidentaloma (AI), it remains a significant concern. Here we present a case of ACC that was diagnosed postpartum 4 years after discovering an AI. Case presentation A 30-year-old female G3P4 presented to the emergency department 4-weeks postpartum with intermittent fever and decreased appetite following an uncomplicated vaginal delivery of healthy twin boys. On physical examination, she had a palpable left-sided abdominal mass. She had a known left 2.2×2.2×2 cm adrenal mass that was discovered on Magnetic resonance imaging 4 years prior. At that the time, she had a comprehensive biochemical evaluation with 1 mg overnight dexamethasone suppression test, PM salivary cortisol, 24-hour urine free cortisol, DHEAS, total testosterone, 24-hour urine dopamine, 24-hour urine epinephrine, 24-hour urine norepinephrine, plasma metanephrine, normetanephrine, renin, and aldosterone. This testing was all unrevealing and she was diagnosed with non-functional AI. She did not undergo follow-up repeat imaging. Computed tomography of the abdomen and pelvis obtained on presentation revealed 19.3×15.6×12.7 cm left retroperitoneal heterogeneous soft tissue mass with a Hounsfield Unit (HU) of > 10. Metastatic workup was negative and repeat biochemical testing remained inconsistent with a functional lesion. She underwent radical left nephrectomy and adrenalectomy. Surgical pathology confirmed the diagnosis of ACC confined within the capsule. The surgery was complicated by a pancreatic leak and short-term total parental nutrition. Fortunately, she was doing well at her 4-month postoperative follow-up. Discussion ACC has an extremely poor prognosis, especially in pregnancy and in the postpartum period. A previous study has shown a 50% and 0% survival rate at 1 and 8-year follow-up for pregnant patients, respectively [1]. AI is a common finding radiologically and on autopsy with a prevalence of 1-9%. It is mostly benign nonetheless; ACC can occur in 1.9% of cases. Although a published review article about AI in New England Journal of Medicine recommends no further testing for adrenal masses < 4 cm and HU <10, the Italian Association of Clinical Endocrinologists (AME) recommends repeat imaging 12 months after initial diagnosis to reconfirm diagnosis of a benign adrenal mass [2]. Unfortunately, this patient did not undergo repeat imaging and was subsequently diagnosed with an impressive ACC. Hence, this case highlights the importance of radiologic monitoring of AI.