Symptom Management in a Patient with Adrenocortical Carcinoma – Targeting Glucocorticoid and Androgen Excess

Outcomes 1. Utilizing a case report, participants will be able to familiarize themselves with the epidemiology and clinical course of adrenocortical carcinoma and learn how to best employ the strengths of a multi-disciplinary palliative care team to provide optimal management of symptoms and complex medical decision making. 2. Utilizing a case-report, participants will be able to identify the many distressing symptoms related to hormone excess associated with adrenocortical carcinoma and with the collaboration of endocrinology experts, be able to employ several treatment options to manage difficult, often refractory symptoms. Key Message Adrenocortical carcinoma is a rare and aggressive malignancy that can present with symptoms of glucocorticoid and/or androgen excess which can drastically affect a patient's quality of life. Palliative care providers should work alongside endocrinology experts to provide targeted symptom relief aiming to improve quality of life using medications targeting hormone synthesis. Abstract Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy often diagnosed after a patient develops symptoms of hypercortisolism and/or hypervirilization (1). A 68-year-old woman was diagnosed with metastatic poorly differentiated ACC after presenting with symptoms associated with glucocorticoid and androgen excess, including lethargy, hyperglycemia, muscle weakness, hirsutism, abdominal pain, depression, and insomnia. The American Association of Clinical Endocrinology recommends all patients with metastatic ACC be evaluated by a palliative care team given it is rarely curable, carries a median survival of 3 to 9 months, and comes with a heavy symptom burden (1,2). However, palliative care providers may be unfamiliar with therapies used to treat symptoms driven by hormonal excess, which are often managed using medications that limit production and increase breakdown of glucocorticoids and androgens (3). Common therapies to treat hormonal excess in ACC include adrenal enzyme inhibitors like ketoconazole, metyrapone, and low-dose etomidate. Ketoconazole is most commonly used, and requires close monitoring for drug interactions, liver toxicity, and development of adrenal insufficiency. Mitotane is an adrenocorticolytic agent that can reduce hypercortisolism and is also often part of an anti-neoplastic approach for those with unresectable advanced disease. Mitotane requires therapeutic monitoring and has considerable adverse effects, including liver toxicity. For hyperandrogenemia and estrogen excess spironolactone, aromatase inhibitors, and selective-estrogen-receptor-modulators can be used. Our patient's symptoms related to hypercortisolism and androgen excess were co-managed by endocrinology and palliative care with ketoconazole and mitotane. Palliative care also recommended appropriate analgesics, anti-emetics, and sleep aids to treat refractory symptoms. Members of the palliative care interdisciplinary team provided additional support for the patient's depressed mood and existential distress and assisted with conversations about goals of care. Patients with ACC benefit from close collaboration between endocrinologists and palliative medicine specialists to ensure optimal management of symptoms and complex medical decision making. Keywords Disease specific managementEmergencies / Refractory Symptom Management