Endocrine Abstracts

Background: Adrenocortical carcinoma (ACC) is a rare malignancy with varied clinical presentations. While sporadic cases are common, familial predisposition plays a significant role in some cases, particularly in younger patients. We present a case of aggressive ACC in a young adult with strong familial cancer susceptibility.

Case Presentation: A 29-year-old male presented with a 4-month history of pleuritic chest pain, dyspnea, right upper quadrant abdominal mass, and 15kg weight loss without any features of hormonal excess. Initial imaging revealed a 17x14.3cm mass between the right kidney and liver, with extensive pulmonary and hepatic metastases. Ultrasound-guided biopsy confirmed adrenocortical neoplasm with oncocytic features, necrosis, and high mitotic activity. Immunohistochemistry showed diffuse positivity for Melan A and Synaptophysin, consistent with ACC. Family history revealed familial predisposition to cancer including adrenocortical, breast, and uterine cancers.

Management: Adrenal MDT commented that tumor was inoperable, and palliative chemotherapy was recommended. However, the patient developed complications including sepsis and acute kidney injury. Repeat CT abdomen showed portal empyema and renal & IVC thrombosis. An overnight dexamethasone suppression test done during initial response to antibiotics revealed hypercortisolemia. Despite initial response to antibiotics, the patient’s condition deteriorated rapidly and scummed before cortisol-lowering therapy could be initiated.

Discussion: This case highlights the aggressive nature of ACC in young adults and the importance of considering genetic predisposition. ACC runs in families 50% of the time. Genetic testing is recommended for all close relatives of people with ACC.

Conclusion: This case emphasizes the importance of early recognition of ACC, particularly in young patients with family history of cancer. Diagnosis in this rare case was delayed due to nonspecific presentation. The aggressive course and rapid deterioration highlight the need for prompt diagnosis and treatment.