Management of undifferentiated embryonal sarcoma of the liver in children: a case series and management review.
BACKGROUND
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare neoplasm, and the third-most common paediatric hepatic malignancy. However, no treatment guidelines exist. No randomised, controlled trials support specific combinations of therapy.


OBJECTIVE
To compare presentation and management of UESL with other series, review the literature, and formulate treatment guidelines.


METHODS
A retrospective chart review of all hepatic malignancies was conducted from 1996 to 2007 and 5 children with UESL were identified. Management and outcomes were documented. The literature regarding treatment modalities up to September 2012 was reviewed.


RESULTS
Over a period of 11 years, 5 patients presented. All underwent surgery and 4 received chemotherapy. One received radiotherapy at relapse. Three are disease-free with follow-up of 58 - 184 months. One died after relapse, as did the patient whose family declined chemotherapy.


CONCLUSIONS
The improved outcomes are consistent with the international experience and are probably related to combined treatment modalities and advances in supportive care. Pre-operative percutaneous biopsy provides no benefit if the lesion is resectable because it may not prove to be diagnostic, and may cause recurrence in the biopsy tract. If resectable, the recommended treatment is primary excision and adjuvant chemotherapy, with radiotherapy in selected cases. If unresectable, open biopsy is necessary to document histology, and neo- adjuvant chemotherapy is given prior to resection. If deemed unresectable, liver transplantation is considered.
Division of Paediatric Haematology and Oncology, Charlotte Maxeke Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa. loveland@wol.co.za.
J A
JA
Geel
J A
JA
Loveland
G J
GJ
Pitcher
P
P
Beale
J
J
Kotzen
J E
JE
Poole
D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D009373:Neoplasms, Germ Cell and Embryonal / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D013019:South Africa / Q000453:epidemiology; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed