Efficacy of neoadjuvant therapy and surgical rescue for locally advanced hepatoblastomas: 10 year single-center experience and literature review

Table 1 Patients features and outcomes

Age (yr)	PRE-TEXT pre-QT	Segments involved at diagnosis	AFP at diagnosis (ng/mL)	Histology	Chemotherapy(No. of cycles)	POST-TEXT at surgery (localization)	Surgery	Postoperative events	Follow-up (mo)	Current status
3	III, P1 (right branch)	I, V, VI, VII, VIII	18597.37	Embryonal	Neoadjuvant PLA (4 cycles) + adjuvant (2c)	II (V, VI, VII, VIII)	Right hepatectomy	Subphrenic abscess (drainage)	18	CR
4	II, P1 (left branch)	IVb, V, VIII	551.21	Epithelial mesenchymal mixed	Neoadjuvant PLA (4c) + adjuvant (2c)	I (IVb)	Segmentectomy IVb	Uneventful	19	CR
8	I	II, III	473856.00	Epithelial fetal	Neoadjuvant PLADO (4c) + adjuv (2c)	I (II, III)	Left hepatectomy	Uneventful	56	CR
13	I	VIII	14277.00	Epithelial fetal	Neoadjuvant PLA (4c) + adjuv (2c)	I (VIII)	Right hepatectomy	Uneventful	28	CR
15	I	VI	401800.00	Epithelial mesenchymal mixed	Adjuvant PLADO (4c)	I (VI)	Tumorectomy	Uneventful	106	CR
25	II, P1 (right branch)	V, VI, VII, VIII	83100.50	Embryonal fetal epithelial	Neoadjuvant PLADO (4c) + adjuv (2c)	II, P0 (VII, VIII)	Bisegmentectomy VII, VIII	Uneventful	13	Lung metastasis
31	I	VI	379.00	Epithelial	Adjuvant PLADO (4c)	I (VI)	Segmentectomy VI	Uneventful	50	CR

PLA: Cisplatin; DO: Doxorubicin; P1: Involvement of either the left or the right branch of the portal vein; CR: Complete remission.

Table 2 Review of current literature

Study	NACH/N (n)	Gender (M/F)	Age (mo)	CH regimen	CH morbidity	PR CH	Complete resection	Postoperative events	OS	DFS
Pritchard et al[8], 2000 SIOPEL-1	138/154	97/ 57	16.5	PLADO (cis+dox)	2% death 6% myelotoxicity < 2% others	82%	92%	5 deaths 8% infections 3% bleeding 9% others	75%	66%
Katzenstein et al[18], 2002	33/33	21/12	22	Car-vin-5FU	60% myelotoxicity	82%	58%	-	57%	48%
Perilongo et al[14], 2004 SIOPEL-2	135/135	81/54	16-25	Cis Car-dox-cis	Neutropenia 43%-81% Infections 40%-76% Transfusion 19%-76%	90% (SR HB) 78% (HR HB) 90% (SR-HR)	97% (SR HB) 67% (HR HB) 100% (SR-HR)	-	91% (SR HB) 53% (HR HB) 86% (SR-HR)	89% (SR HB) 48% (HR HB) 89% (SR-HR)
Towu et al[16], 2004	54/56	34/22	12	22 PLADO 14 SIOPEL-2 17 SIOPEL-3	-	92%	74%	1 death 22% (bile leakage, collections, others)	75%	-
Zsíros et al[15], 2010 SIOPEL-3	150/151	90/61	21	Cis/car-dox	1 death 76% neutropenia 51% infections 33% renal toxicity	78.70%	76.20%	4 deaths	69%1	65%
Lautz et al[19], 2010	14/14	7/7	8	Cis-vin-5FU Others	-	61%	85%	1 Iscq cholangio 1 portal thrombosis	88%	77%
Hishiki et al[17], 2011	185/212	132/80	17	Cis-pir Iof-car-pir-eto	90% neutropenia 10% infections < 10% others	65%	63%	-	81%	62.4%

¹3-year overall survival. NACH: Number of patients with neoadjuvant chemotherapy; N: Total number of patients; CH: Chemotherapy; PR: Partial response; OS: 5-year overall survival; DFS: Disease-free survival; cis: Cisplatin; dox: Doxorubicin; car: Carboplatin; pir: Pirarubicin; iof: Ifosfamide; eto: Etoposide; vin: Vincristine; 5FU: 5-fluorouracil; SR HB: Standard risk hepatoblastoma; HR HB: High risk hepatoblastoma; SR-HR: Standard risk hepatoblastoma treated as high risk hepatoblastoma.