Adrenocortical Carcinoma: An Exceptional Survival Span Across a Tailored Management

Abstract

Adrenocortical carcinoma (ACC) represents a very rare (an incidence of 1–2 cases per million) and aggressive malignancy (an overall 5-year survival of 15%). Less than 15–20% of cases involve a single metastatic disease. In this presentation, we documented a most exceptional survival span in a case of ACC, between the female patient’s age of 50 and the current age of 66. The subject underwent an open right adrenalectomy three times (at first diagnosis, after recurrence 2 years later, and, most recently, 15 years after the initial diagnosis amid a multi-visceral resection). She initially displayed Cushing syndrome, which remitted post-surgery. While the first pathological report established an adrenocortical adenoma, after relapse, low-grade ACC was confirmed and the first tumor mass was re-assessed as ACC based on a similar pathological profile. Following the second surgery and ACC recognition, she started mitotane therapy for 5 years (which she tolerated well, although she developed primary adrenal insufficiency). During this period, a single metastatic disease was detected at the pulmonary level, and she underwent a successful thoracic surgical resection of a 2.5 cm metastasis in the left inferior lobe (2 years after the second adrenalectomy). After this 5-year course of mitotane, the disease was stable, and a 4-year withdrawal period was followed by a relapse with local adrenal recurrence and growth of several lung nodules. Mitotane was re-started, and the en bloc resection included the right adrenal tumor (of 12 cm), right kidney, three liver segments, retro-hepatic inferior vena cava, and (partial) left renal vein. The reconstruction of the inferior vena cava required a Dacron graft of 12 cm in addition to a synthetic graft, which was placed between the left renal vein and the first graft. During surgery and in the following weeks, she presented with several episodes of acute adrenal insufficiency despite continued prompt replacement. A peak ACTH of 369 (Normal: 7.2–63.3) pg/mL was reached at one point. Renal function remained stable. In the meantime, she continued levothyroxine replacement (50 to 75 µg/day) for mitotane-induced primary hypothyroidism. Postoperative, low-grade ACC was confirmed with a Ki67 of 15%, as previously found. She re-started mitotane in month 3 and completely recovered following 9 months, while an abdominal CT scan confirmed no adrenal remnants. Further close surveillance was mandatory. A particular aspect involves the patient’s options (personalized management), as she declined chemotherapy, immunotherapy or radiotherapy at any point in life and delayed the third adrenalectomy during the COVID-19 pandemic and early post-pandemic years. This case highlights an exceptional survival span across two decades with an ailment with a generally poor prognosis. Another particular aspect involves a complex multi-segmental resection, which requires a highly skilled surgical team; only a limited number of cases have previously been published, and, to the best of our knowledge, there is no previous data with respect to a third intervention after 15 years of surviving with ACC.