Endocrine Abstracts

Introduction: Adrenocortical carcinoma is a rare tumour, with an annual incidence of 1 to 2 case per million. Hypercortisolism constitutes the predominant clinical manifestation in patients with hormone excess, presenting in 50% to 80% of all adrenocortical carcinomas. Non-functioning tumors are even rarer, comprising only 20% of adrenal carcinomas in the largest reported series. Conversely, Addison’s disease is also an infrequent disorder, with estimated incidence rates in Europe ranging from 4.4 to 6.2 new cases per million per year. Currently, autoimmunity stands as the prevailing etiology of Addison’s disease in Europe, accounting for 75-96% of all cases. Herein, we present an unprecedented case involving a woman diagnosed with both adrenocortical carcinoma and Addison’s disease.

Case report: A 45-year-old woman with a medical history marked by multiple allergies and autoimmune disorders, including vitiligo, autoimmune vasculitis, and myasthenia gravis underwent thymoma surgery in 2013. An incidental finding on CT revealed a 52 x 44 mm right adrenal adenoma with suspected malignancy. Functionality studies demonstrated normal 24-hour urine metanephrines and slightly elevated 24-hour urine cortisol. Subsequently, a right adrenalectomy was performed, yielding biopsy results indicative of carcinoma staged as T2N0M0. No oncological treatments were administered during ten years of follow-up, with no evidence of recurrence or hormonal abnormalities. After a decade without issues, the patient was referred to our clinic due to significant asthenia and weight loss. Physical examination unveiled marked cutaneous and mucosal hyperpigmentation, accompanied by a propensity toward hypotension. In light of suspected adrenal insufficiency, blood tests and a CT scan were conducted. The CT scan revealed no abnormalities in the contralateral adrenal gland, but analysis confirmed primary adrenal insufficiency, reflected in decreased cortisol levels (1.86 µg/dl, reference range: 4.8-20.00 µg/dl) and elevated ACTH (1835 pg/ml, reference range: 3.6-60.5 pg/ml). Aldosterone and DHEA levels were also diminished. Positive 21-OHAbs (3.35, reference range: 0.00-1.00) confirmed autoimmune adrenal insufficiency. Treatment commenced with hydrocortisone 20 mg/24h and fludrocortisone 50 mg/24h, resulting in notable improvement in asthenia and hyperpigmentation, enabling the patient to resume her usual activities.

Conclusions: Addison’s disease should be consistently contemplated in the differential diagnosis of adrenal insufficiency. Prompt initiation of corticosteroid therapy is imperative in the management of adrenal insufficiency.