Undifferentiated carcinoma of the liver in a 3-year-old girl treated by neoadjuvant chemotherapy and complete resection

Abstract Introduction Undifferentiated carcinoma (UC) of the liver has only been reported in three adults in the English language literature and is so rare it has never been reported in a child. Our management is presented to improve knowledge of its treatment. Case presentation A 3-year-old previously well Japanese girl was referred for further assessment/management of an abdominal mass. On examination an obvious right hypocostal mass was visible extending across the midline. Diagnostic imaging identified a 12.5 cm mass on the ventral surface of the liver containing multiple cystic lesions extending along Glisson’s capsule with invasion to the portal vein. Open biopsy eventually led to a diagnosis of poorly differentiated or UC of the liver with embryonal features. Resection of hepatic segments 4b and 5 after a remarkable initial response to cisplatin/doxorubicin that shrank the tumor substantially, separating it from Glisson’s capsule enabled total excision. Surgery was successful and tolerated well with unremarkable postoperative recovery. Unfortunately, ascites due to peritoneal carcinomatosis developed 4 months postoperatively and she died 5 months later. Conclusion The initial impressive response to neoadjuvant chemotherapy and successful surgery was unexpectedly fortuitous but inadequate for controlling such an aggressive malignancy. Our case demonstrates the value of neoadjuvant chemotherapy.