Two Autopsy Cases of Advanced Adrenocortical Carcinoma: The Utility of Immunohistochemical Panel for Pathological Diagnosis

Abstract

Adrenocortical carcinomas are extremely rare aggressive tumors arising from the adrenal cortex. Histopathologically, differential diagnosis between adrenocortical carcinoma and pheochromocytoma is often difficult. We report two autopsy cases of adrenocortical carcinoma. The first case was a 20-year old man. He had abdominal discomfort and increasing swelling of the lower legs. Computer tomography (CT) detected a tumor of 16 cm in diameter at the right retroperitoneum space and multiple liver nodules. Although he was treated with chemotherapy, he died of the cardiovascular failure accompanying inferior vena cava tumor embolism after 4 days of initial chemotherapy. The second case was a 57-year old woman. She felt easy fatigability. Abdominal ultrasonography and CT detected a tumor of 20 cm in diameter at the left retroperitoneum space and multiple liver nodules. She was also treated with chemotherapy and mitotane. However, she died 13 months after the initial diagnosis. Autopsies were conducted in both cases. Histologically, both tumors were composed of polygonal shaped cells with marked atypia. Immunohistochemically, both showed positive for synaptophysin, carletinin, inhibin, melan A and steroidogenic factor 1 and negative for chromogranin, suggesting that both tumors were adrenal cortex origin. Moreover, both cases were categorized into malignancy according to Weiss criteria. Thus, we diagnosed both cases were aderenocortical carcinoma. We report here two autopsy cases of aggressive advanced adrenocortical carcinoma, emphasizing the utility of the immunohistochemical panel.