Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the adrenal cortex often with unexpected biological behavior. It can occur at any age, with two peaks of incidence: in the first and between fifth and seventh decades of life. Although ACC are mostly hormonally active, precursors and metabolites may be also produced by dedifferentiated and immature malignant cells.

Case report: We report a rare and challenging case of adrenocortical carcinoma (ACC) in a 40-year-old male with no previous pathological history, presenting with severe abdominal pain, significant weight loss, and hypercortisolism. The patient was admitted to the Endocrinology Clinic for evaluation of an adrenal mass, initially suspected to be an adrenal hematoma of approximately 6 cm based on abdominal CT findings. Clinical examination revealed pale skin, minimal adipose tissue, and a large, hard, painful mass in the right flank. Hormonal evaluation confirmed hypercortisolism, indicated by increased cortisol levels (CLU at 590 mg/dl), a lack of suppression with 1 mg dexamethasone, and only partial suppression with a high-dose dexamethasone test. Notably, DHEAS levels were markedly elevated (>1000 mg/dl), along with increased 17-OH-Progesterone, whereas urinary metanephrines remained normal. The patient also exhibited hypogonadotropic hypogonadism, likely secondary to hypercortisolism. Further imaging with an abdominal CT scan revealed a large retroperitoneal mass centered at the right adrenal lodge, measuring 149/118/168 mm. This mass had heterogeneous components, showing no clear demarcation from adjacent vital structures including the liver, kidney, and inferior vena cava, and exerting a mass effect. Multiple nodular lesions in the right hepatic lobe suggested metastases. A biopsy from the adrenal mass confirmed the diagnosis of adrenocortical carcinoma with a high Ki67 proliferation index of 70-80%. Given the tumor’s extensive size, mass effect, and evidence of hepatic metastases, surgical intervention was deemed unfeasible – stage IV. The patient was commenced on Mitotane and protocol-based chemotherapy.

Conclusions: This case of adrenocortical carcinoma exemplifies the unique and complex nature of this malignancy, particularly in its long-term evolution without overt clinical signs of hypercortisolism. Despite the absence of initial symptoms typically associated with hypercortisolism, the patient’s condition rapidly deteriorated, underlining the unpredictable and aggressive course of ACC. The long asymptomatic progression of ACC in this patient raises important questions about the underlying mechanisms of tumor growth and hormonal activity, suggesting a potential area for further investigation in the field of endocrine oncology.