HBL is the most common malignant
liver neoplasm in children. The etiology of HBL is largely unknown but there are certain syndromes, such as
Beckwith-Wiedemann syndrome, that have been clearly associated with an increased incidence of this
malignancy. EBS, also known as
prune belly syndrome, is a congenital anomaly characterized by lax abdominal musculature,
bilateral cryptorchidism requiring, in some cases,
hemodialysis due to significant kidney and urinary tract dysfunctions. Despite an improvement on the survival rates of patients with advanced-stage HBL, the presence of concomitant
end-stage renal disease that occurs in patients with EBS constitutes a therapeutic challenge for the clinician not only due to the use of nephrotoxic
chemotherapy but also due to the potential need for multi-organ transplant. We report case of a 2-year-old male patient with EBS diagnosed with stage IV, metastatic HBL successfully treated with multi-agent
chemotherapy while on dialysis whom then underwent a simultaneous liver-kidney transplant followed by
adjuvant chemotherapy. Ultimately, the patient achieved
cancer remission with normalization of his renal function. Our report emphasizes that patients with HBL in the setting of EBS will not only require careful kidney function monitoring while receiving
chemotherapy, but they might also need to undergo multi-
organ transplantation in order to achieve adequate
cancer control and also normalization of their kidney function. Awareness of this unusual association calls for further investigation to potentially establish a genetic association between these two disease processes.