Endocrine Abstracts

Background: Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy with an annual incidence of 1–2 per million and poor prognosis in advanced stages. Surgical resection is the only potentially curative treatment, but the benefit of debulking surgery in metastatic disease remains uncertain. ACC can also occur in hereditary cancer predisposition syndromes such as Li-Fraumeni, which are associated with central nervous system tumours including medulloblastoma.

Case presentation: A 39-year-old woman with a childhood history of medulloblastoma treated with surgery and radiotherapy presented with severe ACTH-independent Cushing’s syndrome and androgen co-secretion. Imaging revealed a large right adrenal mass invading adjacent structures with pulmonary, pleural, bony, and nodal metastases. Cytology confirmed metastatic ACC. She was stabilised with metyrapone and hydrocortisone, then underwent open adrenalectomy with nephrectomy for debulking. Histology showed high-grade ACC (Ki67 30%, stage IV, R2 resection). Postoperatively, she developed malignant pleural effusions, pulmonary emboli, and functional decline. Restaging scans demonstrated rapid progression, systemic therapy was not appropriate, and she was transitioned to best supportive care.

Discussion: This case highlights the limited role of debulking surgery in advanced ACC, where morbidity may outweigh potential benefit in aggressive disease. The dual occurrence of medulloblastoma and ACC suggests possible germline predisposition, consistent with syndromes such as Li-Fraumeni. Genetic evaluation should be considered in such patients.

Conclusion: In advanced ACC, multidisciplinary decision-making is essential before undertaking debulking surgery. A prior history of CNS malignancy should prompt consideration of hereditary cancer predisposition syndromes.