Functional adrenocortical carcinoma with adrenohepatic fusion: A case report

Abstract

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 μg/dL; and total serum testosterone, 1489.01 ng/mL. Ultrasonography and computed tomography initially revealed a hepatic tumor measuring 100 × 111 × 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 × 10 × 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.