Page 28 - Read Online

Calinescu et al. Hepatoma Res 2021;7:54 https://dx.doi.org/10.20517/2394-5079.2021.25 Page 7 of 9

Table 3. Reported criteria for unresectable UESL
Unresectable UESL
1. Involvement of both liver lobes, including caudate lobe
2. Large tumor size precluding safe surgical resection
3. Distant metastasis

UESL: Undifferentiated embryonal sarcoma of the liver.

Table 4. Possible determinants for the long-term outcome of UESL
Possible determinants for the long-term outcome of UESL
1. Tumor size
2. Tumor staging (including age)
3. The role of neoadjuvant chemotherapy and (neo)adjuvant radiotherapy
4. Positive microscopic resection margins
5. Preoperative tumor rupture
6. Degree of tumor necrosis after neoadjuvant chemotherapy
7. Metastatic disease
8. Tumor recurrence
9. Transplantation
UESL: Undifferentiated embryonal sarcoma of the liver.

Liver failure was described in a three-patient case series causing one patient’s death, but data concerning
tumor location and surgical excision is scarce . One post-resection liver failure needed a rescue liver
[39]
transplantation in another series .
[9]
One in 17 patients was reported to die after a right lobectomy in an early report of 2002, due to surgical
complications .
[22]
SURVIVAL
Five-year survival rate was less than 40% in the early 1980’s , with current survival rates higher than
[11]
80% [11,14,28] . It seems that diagnosis errors with inadequate surgical management lead to lower survival
rates , again, supporting the evidence that more generally accepted standardized protocols are needed.
[40]

Studies focus mainly on the chemotherapy regimens when making correlations with survival rates. The role
of other determinants for the long-term outcome summarized in Table 4 needs further clarifications [9,12,41] .

CONCLUSIONS
Over the last decades, the outcome of pediatric UESL has improved. To allow for optimal management of
UESL, and thus for accurate outcome analysis, systematic, preoperative neoadjuvant chemotherapy should
be further defined, as compared to upfront surgery. A new risk stratification grouping system for UESL
including the PRETEXT staging, age of patient, and size of the mass might be considered in a prospective
study. Further, the role of positive microscopic margins needs to be defined.

DECLARATIONS
Authors’ contributions
Made substantial contributions to conception and design of the study, performed data analysis and
interpretation, finally approved the version to be published and agreed to be accountable for all aspects of
the work: Calinescu AM, Wildhaber BE, Guérin F