Endocrine Abstracts

JOINT8

Background: Malignant adrenocortical carcinoma (ACC) is a rare, aggressive endocrine malignancy with a poor prognosis, even with therapeutic intervention. Tumor size and metastatic spread significantly influence the disease’s clinical course. Despite advancements in diagnostic and treatment approaches, managing ACC remains challenging, necessitating a multidisciplinary strategy.

Case Presentation: We report the case of a patient presenting initially with nonspecific abdominal pain managed with symptomatic treatment. Persistent symptoms led to further imaging, revealing a large right adrenal mass measuring 105 × 107 × 71 mm, accompanied by pulmonary, lymph node, and retroperitoneal metastases. Clinical examination showed hypercorticism, hyperandrogenism, and signs of virilization. Laboratory investigations confirmed elevated urinary free cortisol (8 times normal), testosterone (0.53 ng/ml), and SDHEA (84.82 µg/dl), alongside hypokalemia (2.5 mmol/l), which required correction. Negative plasma methoxylated derivatives excluded pheochromocytoma. Pathological confirmation of malignant ACC was obtained via biopsy. Despite initiating a chemotherapy protocol, the patient succumbed shortly after admission to the oncology unit.

Discussion: ACC’s clinical presentation often varies, ranging from asymptomatic incidentalomas to severe hormonal dysregulation and metastatic disease. In this case, the constellation of virilization and hypercortisolism highlights the functional nature of the tumor. Imaging and biochemical workup were pivotal in rapid diagnosis—unfortunately, the advanced stage at presentation limited therapeutic options. Current recommendations emphasize the importance of early detection through vigilant monitoring of incidental adrenal masses and hormonal assessment. Treatment modalities include surgery for localized disease, systemic therapies such as mitotane, and palliative measures for advanced cases. The poor prognosis in advanced ACC underscores the need for novel therapeutic approaches and inclusion in clinical trials.

Conclusion: This case highlights the aggressive nature of ACC and the importance of a comprehensive, multidisciplinary approach in management. The rapid progression from diagnosis to mortality underlines the critical need for earlier detection and innovative therapeutic strategies to improve patient outcomes. Future research should focus on early biomarkers, targeted therapies, and optimizing systemic treatments to better address this devastating malignancy.