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Calinescu et al. Hepatoma Res 2021;7:54 https://dx.doi.org/10.20517/2394-5079.2021.25 Page 3 of 9

Table 1. Treatment protocols for paediatric undifferentiated embryonal sarcoma of the liver (UESL)
Treatment protocol for UESL and bile ducts Rhabdomyosarcoma according to EPSSG RMS 2005
Low risk (subgroup A) IRS I Age < 10 years and size < 5 cm Post-op VA 8 cycles (22 weeks)
Standard risk IRS I Age > 10 years or size > 5 cm Post-op IVA 4 cycles and VA 5 cycles
(subgroup B)
Standard risk IRS II or III Age < 10 years and size < 5 cm IVA 3 cycles
(subgroup D) N0 Surgical assessment
IVA 1 cycle
(Re-do) Surgery +/- Radiotherapy
IVA 5 cycles
High risk IRS II, III unfavourable size or age or N1 IVA +/- Do 3 cycles
Surgical assessment
IVA +/- Do 1 cycles
(Re-do) Surgery +/- Radiotherapy
IVA 5 cycles
Very high risk Metastatic IVADo 3 cycles
Surgical assessment
IVADo 1 cycle
(Re-do) Surgery +/- Radiotherapy
IVA 5 cycles
24 weeks maintenance with vinorelbine and cyclophosphamide
CWS-2002 P
Low risk IRS I Age < 10 years and size < 5 cm Post-op VA 4 cycles
2
Standard risk IRS I and/or Age > 10 years and size > 5 Post-op I VA 4 cycles
(subgroup B) cm and N0 VA 2 5 cycles
2
Standard risk IRS II or III and N0 I VA 4 cycles
(subgroup C) Surgery
2
I VA 5 cycles No radiotherapy
Or radiotherapy with 5 cycles VA 2
2
Standard risk IRS II or III and N0 and ≤ 5 cm and < 10 I VA 4 cycles
(subgroup D) years Surgery +/- Radiotherapy
2
I VA 5 cycles
2nd line treatment according to response
2
High risk (subgroup E, I VA 4 cycles
F, G) Surgery +/- Radiotherapy
2
I VA 5 cycles
2nd line treatment according to response
2 2 2 2
Very high risk IRS II, III and N1 I VAd-I VA-I VAd-I VAd
Surgery after 4th cycle
Radiotherapy
2
2
2
I VA-I VAd-3 cycles I VA
2nd line treatment according to response
ARST 0332
Low risk arm B Tm diameter ≤ 5cm, macroscopic Adjuvant radiotherapy
margins
Intermediate risk arm C Tm diameter > 5 cm Adjuvant chemotherapy 2 cycles Adriamycin/Ifosfamide and 2 cycles
Ifosfamide radiotherapy
Intermediate risk arm D Unresectable tumour or high grade Neoadjuvant chemotherapy
tumour > 5 cm 2 cycles Adriamycin/Ifosfamide and 2 cycles Ifosfamide
Neoadjuvant radiotherapy
UESL: Undifferentiated embryonal sarcoma of the liver; IRS: intergroup rhabdomyosarcoma study; IVA: ifosfamide, vincristine, actinomycin D;
IVADo: ifosfamide, vincristine, actinomycin D, doxorubicin; VA: vincristine, actinomycin D; Source: clinicaltrial.gov (id: NCT00379457).
option in pediatric liver malignancies [17,18] .

As with other liver tumors, in the case of tumor rupture or spontaneous bleeding and if the patient is
unstable, arterial embolization or, if feasible, complete upfront resection might be performed .
[9]