Improved outcome for children with hepatoblastoma

Wilkinson

American Journal of Transplantation

et al. 2002

The purpose of our study was to evaluate the outcome of children who underwent liver transplantation as treatment for unresectable hepatoblastoma. We prospectively collected data on 311 consecutive liver transplants performed at Children's Medical Center of Dallas between October 1984 and November 2000. There were nine recipients (five boys, four girls) with a diagnosis of unresectable hepatoblastoma. Postoperative survival of those currently alive ranged from 6 months to 16 years (mean 6.4 years, median 7.7 years). All recipients received preoperative chemotherapy; 67% received postoperative chemotherapy. Mean AFP level prior to transplantation was 1 448 000 ng/mL. Mean age at diagnosis was 0.81 years. Mean age at transplantation was 1.87 years. Only two patients experienced acute cellular rejection in the postoperative period. There was a total of three deaths and one recurrence. The only instance in which AFP levels did not decrease to low or undetectable levels post-transplantation was in the patient with recurrent tumor. Liver transplantation has an established role in the treatment of hepatoblastoma. It accounted for 3% of pediatric liver transplants, and provided the only opportunity for survival in otherwise incurable patients. Early diagnosis and treatment were found to be associated with better results. Response to chemotherapy may be an important factor influencing survival. Rising AFP levels after transplantation are associated with recurrence.

Section: Discussionmentioning

confidence: 99%

Treatment of Unresectable Hepatoblastoma with Liver Transplantation in the Pediatric Population

Wilkinson

American Journal of Transplantation

et al. 2002

“…Prior to the use of preoperative chemotherapy, about half of newly diagnosed hepatoblastomas were considered resectable [15]. In more recent reports only about 30% have been considered resectable at diagnosis, since tumors that are likely to result in significant surgical morbidity with resection are now considered unresectable [45,48]. Chemotherapy has been proven to be effective both in an adjuvant as well as neoadjuvant setting.…”

Section: Treatment Surgerymentioning

confidence: 99%

“…Chemotherapy has been proven to be effective both in an adjuvant as well as neoadjuvant setting. The use of neoadjuvant chemotherapy has resulted in most nonmetastatic hepatoblastomas being resectable [45,48].…”

Section: Treatment Surgerymentioning

confidence: 99%

“…The key to reducing complications and providing a successful resection is careful, meticulous dissection of the portal vein, hepatic artery, and hepatic ducts and their branches to assure that the remaining lobes have adequate blood supply and bile drainage. The most common intraoperative complication is hemorrhage [15,45]. The risk of bleeding is increased with extended hepatectomy or tumor proximity to the IVC or hepatic vessels [45].…”

Section: Treatment Surgerymentioning

confidence: 99%

See 1 more Smart Citation

Childhood Cancers: Hepatoblastoma

2000

Hepatoblastoma is the most common primary liver tumor in children, accounting for just over 1% of pediatric cancers. The etiology is unknown, but it has been associated with Beckwith-Weidemann syndrome, familial adenomatosis polypi, and low birth weight. The primary treatment is surgical resection, however, chemotherapy plays an important role by increasing the number of tumors that are resectable. The prognosis for patients with resectable tumors is fairly good, however, the outcome for those with nonresectable or recurrent disease is poor. Texas-Houston Medical School, 1515 Holcombe Blvd., Houston, Texas 77030, USA. Telephone: 713-745-0157; Fax 713-792-0608; e-mail: cherzog@ mdanderson.org Received June 21, 2000; accepted for publication September 6, 2000. ©AlphaMed Press 1083-7159/2000 INTRODUCTIONHepatoblastoma is the most common malignant tumor of the liver in children. Surgery remains the primary means of curative therapy, but the role of chemotherapy in both the adjuvant and neoadjuvant setting has become increasingly important over the past three decades. New insight has also been gained into the molecular biology of hepatoblastoma.

“…However, the presence of microscopic residual disease at the resection margin is not a major adverse determinant of survival. 4,21,22 None of the 11 patients with positive margins in SIOPEL-1 underwent a second resection and all but one was treated by postoperative chemotherapy alone. None developed recurrent tumour and all the survivors were in complete remission 5 years later.…”

Section: Liver Transplantationmentioning

confidence: 99%

The Role of Liver Transplantation in the Management of Paediatric Liver Tumours

Stringer

2007

annals

In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy. Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver. A 5-year survival of 70% can be achieved in such cases. In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation. An exception is those children in whom the hepatocellular carcinoma is detected during surveillance of chronic liver diseasethey typically have smaller tumours and frequently have a good prognosis after liver transplantation. The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited. Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.