Functional adrenocortical carcinoma with adrenohepatic fusion: A case report

Introduction: Adrenocortical carcinoma (ACC) is rare, aggressive, and metastasizes mainly to the lungs, liver, and regional lymph nodes. This is the first reported case of a pediatric patient with adrenohepatic fusion (AHF) and functional ACC that solely invaded the liver. Case presentation: The patient was a 2 year and 9 month old female, with a 3-month history of weight gain, signs of virilization, Cushing's syndrome, hypertension, and a palpable mass in the right hypochondrium and epigastrium. Blood tests revealed the following: hemoglobin, 16.3 g/ dL; cortisol, 25.3 mu g/dL; and total serum testosterone, 1489.01 ng/mL. Ultrasonography and computed tomography initially revealed a hepatic tumor measuring 100 x 111 x 106 mm, with a volume of 615 mL. The patient was diagnosed with functional ACC. Curative resection (R0 resection) was performed, involving partial resection of the right hepatic lobe. The resected tumor was 12 x 10 x 7 cm and 650 g, with evidence of invasion into the liver capsule, which was consistent with ACC stage III (T4N0M0). Management included mitotane, cisplatin, etoposide, and doxorubicin, and favorable progress was noted at the 2-year and 3-month follow-up. Conclusion: In cases with AHF, a sufficient portion of the right hepatic lobe must be resected to ensure complete removal of the tumor.