Many studies have shown that UESL does not have a specific immunophenotype.
Individual markers are often not helpful in differentiating UESL from other liver tumors.
The extensive panels of immunohistochemical markers are helpful in diagnosing UESL.
The concept of mesenchymal origin in UESL is generally accepted, despite its evidently obscure histogenesis.
The main differential diagnoses for UESL in the pediatric population are MH, hepatoblastoma, and embryonal rhabdosarcoma of the biliary tree.
Distinguishing UESL from hepatoblastoma is usually not difficult on the basis of its characteristic histologic appearance and immunophenotype.
Patients with UESL cannot be cured using modalities that exclude surgery.
UESL is an aggressive malignancy that is prone to local recurrence and, less often, distant metastatic spread.
Because there were no standard treatment guidelines, the Italian (GCI) and German (CWS) Soft Tissue Sarcoma Cooperative Study Groups independently treated children with UESL according to the childhood rhabdomyosarcoma guidelines.
An article on UESL from a developing country details the successful treatment of 3 patients with cisplatinum, bleomycin and etoposide,  a course of treatment which may have been chosen for its comparatively low cost, despite the incidence of pulmonary fibrosis which limits use of bleomycin in better-resourced centres.
Oxaliplatin is a newer platinum agent with a more favourable toxicity profile, which is being considered as a possible agent against recurrent hepatoblastoma, and may thus also offer hope in the treatment of UESL.
28] While radiotherapy is not part of the standard treatment protocol for other hepatic malignancies (because the effective tumour dose exceeds hepatic tolerance), a role for it may evolve in the treatment of UESL as soft-tissue sarcomas are typically radiosensitive.