PSAT005 The Third Twin: A Rare Presentation of Adrenocortical Carcinoma
Authors: Abdulwahid Tiba , Selk Karen 
Published: November 2022

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 &lt; 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 &gt; 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 &lt; 4 cm and HU &lt;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.

References
[1] Abiven-Lepage G, Coste J, Tissier F, et al. Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis. Eur J Endocrinol. 2010;163(5): 793-800. doi: 10.1530/EJE-10-0412
[2] Terzolo M, Stigliano A, Chiodini I, et al. AME position statement on adrenal incidentaloma. Eur J Endocrinol. 2011;164(6): 851-870. doi: 10.1530/EJE-10-1147
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