Case study

A 55-year-old male with metastatic ACC with multiple recurrences

• The patient underwent first adrenalectomy in 2004, but experienced multiple recurrences of increasing frequency, and had several further rounds of surgery.
• Patient was eventually deemed unfit for any more surgery after an MDT meeting, due to shorter disease-free intervals.
• Commenced palliative mitotane therapy and gradually titrated to the highest tolerated dose (1,000 mg twice daily [BD]). Mitotane levels were not checked as the patient could not afford the self-paid tests.
• Patient initially responded well, but later showed disease progression (2019).
• Due to symptoms associated with tumour progression, patient was offered further debulking surgery, which he declined. Therefore, mitotane treatment was continued, reserving chemotherapy as next option in the event of further disease progression.
• Patient noted skin pigmentation (Figure 1a) and symptoms of lethargy, with subsequent biochemistry showing hyperkalaemia, hyponatraemia and elevated adrenocorticotropic hormone (ACTH) (2021). These findings reflect insufficient steroid replacement despite usual dose of hydrocortisone replacement.
• Hormonal replacement therapy was adjusted (hydrocortisone 10 mg BD to 20 mg BD). Fludrocortisone 25 µg daily was added (2021), and further increased to 50 µg daily (2022). Skin pigmentation resolved (Figure 1b).
• In 2023, patient developed neurological symptoms initially suspected to be mitotane toxicity-related. Subsequent evaluation confirmed malignancy-related cerebral venous thrombosis as concluded by the neurology team.