Adrenocortical Carcinoma a Rare Cancer

Adrenocortical carcinoma occurs in fewer than two cases per million per year and shows bimodal age distribution with peaks in early childhood and the fourth to fifth decade. About sixty percent secrete cortisol, androgens, mineralocorticoids, or estrogens producing Cushing syndrome, virilization, hypertension, or feminization, while the remainder are non functioning and discovered as large abdominal masses.

Diagnosis combines hormonal workup with twenty four hour urinary cortisol, dehydroepiandrosterone sulfate, sex steroids, and renin aldosterone ratio together with cross sectional imaging that shows large heterogeneous mass with high attenuation on unenhanced computed tomography, irregular margins, and calcifications. ENSAT staging classifies tumor extension and is the strongest prognostic factor.

Complete margin negative surgical resection is the only curative therapy and adjuvant mitotane reduces recurrence in high risk cases. Advanced disease receives mitotane plus etoposide doxorubicin cisplatin combination or radiotherapy for locoregional control. Overall five year survival ranges from sixty percent in stage one to less than twenty percent in stage four with active investigation of immunotherapy and targeted approaches.