Adulthood hepatoblastoma

Tulay Akman, Mehmet Asi Oktan, Ilkay Tugba Unek, Tarkan Unek, Ilhan Oztop, Tugba Yavuzsen, Ahmet Ugur Yilmaz, Ozgul Sagol
2015 The Turkish Journal of Gastroenterology  
Hepatoblastoma is one of the most common malignant hepatic tumors to be diagnosed in children. On the other hand, it is rare and associated with poor prognosis in adults (1). Here, we present a case of an adult who was diagnosed with hepatoblastoma, and has been followed up for 5 years, during which time she remained alive. An 18-year-old female patient was admitted to our clinic following detection of hepatocellular carcinoma in her liver biopsy. Alpha-fetoprotein (AFP) was 4646 ng/mL and
more » ... 4646 ng/mL and other tumor markers and laboratory parameters were within the normal ranges. In a thoraco-abdominal computerized tomography examination, a 4.5x5.5 cm lesion was detected in the liver (Figure 1) . A hepatic left segmentectomy was then performed and histopathological examination revealed an epithelial hepatoblastoma (fetal embryonic) (Figure 2 ). After the operation, 6 cycles of cisplatin and adriamycin chemotherapy were administered. Within the first year of follow-up, a recurrent lesion was detected in the liver. The patient underwent mass excision and the subsequently received 6 cycles of vincristine, cisplatin and 5-FU chemotherapy following the operation. In the second year of follow-up, a recurrent lesion in the liver and a thrombus in the inferior vena cava were detected. A decision was made to perform liver transplantation. Due to the lack of a donor, however, mass excision, diaphragm resection, inferior vena cava resection and vein grafting were performed instead. Six cycles of iphosphamide, cisplatin and etoposide chemotherapy were administered to the patient after surgery. The patient, who is within the fifth year of her first diagnosis, continues to be followedup in the post-operative period. Informed consent was taken from the patient about this publication. Among hepatoblastoma cases, 95% are aged below 5 years (2). It presents very rarely during adolescence and early adulthood (1). Therapeutic regimens are based on chemotherapy and total excision of the tumor (3). While anthracycline-containing regimens have been used preferentially as chemotherapeutic agents, most recent studies have highlighted that cisplatin-based regimens without anthracycline were as efficient as those including anthracycline (4). In patients with resectable tumors, 5-year survival is approximately 80%
doi:10.5152/tjg.2015.6544 pmid:25698280 fatcat:upwe6g7bujcw7fc2emtwcaax3u