Leptomeningeal metastasis from Adrenocortical carcinoma: a case report

Adrenocortical carcinoma (ACC) is an uncommon endocrine malignancy with limited treatment options. While overall 5-year survival rate in patients with ACC is 35%, the disease is often rapidly progressive with long-term survival in only 5% of patients. Although tumor stage, grade and excess hormonal activity predict unfavorable prognosis, additional biomarkers are needed to identify patients with aggressive disease.

A 23-year-old woman presented with rapidly progressing signs and symptoms of Cushing’s syndrome, with associated abdominal pain and fullness. Evaluation revealed a large left adrenal mass which had developed over 8 months. En bloc surgical resection was performed by an endocrine surgeon, and pathology revealed adrenocortical carcinoma with Ki67 of 60%. Despite adjuvant treatment with mitotane and etoposide-doxorubicin-carboplatin chemotherapy, the patient had rapid disease progression with metastatic spread to liver, lung, bone, brain and leptomeningies and she died eleven...