Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience

Abstract

Treatment for HEH does not follow a standardized algorithm. From clinical experience, it is assumed that pediatric patients with HEH will fare as well as other common pediatric liver tumors post-OLT. The UNOS dataset was examined for patients with pediatric OLT between 1987 and 2007. Patients were grouped into non-tumors, HB, HCC, HEH, and rare liver tumors. COD analysis was calculated using Fisher's exact test. Patient, allograft, and recurrence-free survival were compared using Kaplan-Meier curves and log-rank tests. A total of 366 patients with pediatric OLT were identified with primary liver tumors (HB - 237, HCC - 58, HEH - 35, other - 36). HEH patient survival (five yr: 60.6%) was poorer than non-tumor OLTpatient survival (five yr: 84.4%). Survival was worse when compared to HB (five yr: 72%) and rare liver tumors (five yr: 78.9%), but better than HCC (five yr: 53.5%). Allograft survival in HEH (five yr: 50.1%) lies between HB (five yr: 63.6%) and HCC (five yr: 42.8%). COD analysis demonstrates recurrence as a major cause in HB and HCC, but not for HEH or other liver tumors. The data suggest that patient survival may not be as high as previously believed and further investigation is warranted.

Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma

“…The widespread application of OLT for children diagnosed with hepatic malignancies has been well established. In a recent review of the United Network for Organ Sharing database, 366 children were identified who underwent OLT for the indication of liver neoplasm between 1987 and 2007 . The most common indication was hepatoblastoma, which was followed by hepatocellular carcinoma and hepatic epithelioid hemangioendothelioma.…”

Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents

“…Liver transplantation has been proposed as the treatment of choice for those with unresectable liver disease (5‐year overall survival rates ranging from 55 to 83%) . However, recurrent allograft disease was observed in 24–33% of patients, and survival postliver transplant appears to be relatively unfavorable for children with EHE . In our series, one of four children transplanted died from transplant complications.…”

Successful management of multi-focal hepatic infantile hemangioendothelioma using TACE/surgery followed by maintenance metronomic therapy

“…Zenge et al opined that resection be reserved for patients with life-threatening complications or suspected malignant tumours,8 while Samuel and Spitz recommended primary resection in all patients with symptoms with readily resectable solitary tumours 9. Though liver transplantation has also been recommended as a life saving option for severely patients with symptoms with multifocal IHE, new data suggest that 5-year survival (60.6%) may not be as high as previously believed10 and whenever possible, alternative treatment options should be considered 11. Steroids and IFN form the backbone of pharmacologic therapy.…”