Elsevier

Journal of Pediatric Surgery

Volume 47, Issue 12, December 2012, Pages 2194-2198
Journal of Pediatric Surgery

PAPS paper
Surgical strategies for unresectable hepatoblastomas

https://doi.org/10.1016/j.jpedsurg.2012.09.006Get rights and content

Abstract

Background

The aim of this study was to assess the surgical strategies for unresectable hepatoblastomas at the initial diagnosis based on the experience of two institutions.

Methods

The PRETEXT (Pretreatment evaluation of tumor extent) and POST-TEXT (Post treatment extent of disease) staging, surgical treatments, and clinical outcomes were retrospectively analyzed for 12 cases with PRETEXT III or IV and M(−) of 29 hepatoblastomas treated based on the JPLT-2 (The Japanese Study Group for Pediatric Liver Tumor-2) protocol at two institutions between 1998 and 2011.

Results

Two of the 9 cases with PRETEXT III status were downstaged to POST-TEXT II. One of the 3 cases with PRETEXT IV showed downstaging to POST-TEXT III. Four of the 7 cases with P2 or V3 (indicated for liver transplantation) in the PRETEXT staging system showed P2 or V3 in POST-TEXT staging after 2 cycles of CITA (JPLT-2 standard regimen), and one case showed P2 or V3 in POST-TEXT staging at the initial operation and underwent primary liver transplantation. The initial surgical treatments were 1 lobectomy, 2 segmentectomies, 6 trisegmentectomies, 2 mesohepatectomies, and 1 primary liver transplantation. Both patients who underwent mesohepatectomies had bile leakage, and 1 of 5 trisegmentectomies had an acute obstruction of the right hepatic vein. Two patients underwent rescue living donor liver transplantation. Both of these patients showed P2 or V3 positive findings in POST-TEXT staging after 2 cycles of CITA.

Conclusions

POST-TEXT staging and P and V factors should be evaluated after 2 cycles of CITA for unresectable hepatoblastomas detected at the initial diagnosis. The patients should be referred to the transplantation center if the POST-TEXT IV, P2, or V3 is positive at that time. Liver resection by trisegmentectomy is recommended in view of the incidence of surgical complications. Careful treatment, such as back-up transplantation, should thus be considered for liver resection in the cases with POST-TEXT IV, P2, or V3 status after initial 2 cycles of CITA.

Section snippets

Patients and methods

All 29 patients treated for hepatoblastoma at the two institutions (Kyushu University Hospital and Kyoto Prefectural University of Medicine Hospital) from 1998 through 2011 were retrospectively reviewed. This study was performed according to the Ethical Guidelines for Clinical Research published by the Ministry of Health, Labor, and Welfare of Japan on July 30, 2003 (revised 2008) and complies with the Helsinki Declaration of 1964 (revised 2008).

The tumors were evaluated using state-of-the-art

Results

Table 1 shows a summary of the 12 cases with PRETEXT III or IV and M (−) hepatoblastomas treated based on the JPLT-2 regimen. Ten cases were PRETEXT III, and 2 cases were PRETEXT IV. Three cases underwent liver transplantation. One case had a primary liver transplantation due to POST-TEXT IV at surgery, while the other 2 cases underwent rescue liver transplantations. Two of the 10 cases with PRETEXT III showed a downstaging to POST-TEXT II at the initial operation. Five cases underwent

Discussion

The current Children's Oncology Group (COG) hepatoblastoma protocol (AHEP0731), determines tumor extent from computed tomography (CT) imaging using the PRETEXT system [10], which is now referred to as POST-TEXT for studies obtained after treatment with neoadjuvant chemotherapy. Children with POST-TEXT III or IV tumors are referred to the pediatric liver transplant center and extreme liver resection at diagnosis if possible and no later than just after the second cycle of chemotherapy. This

Acknowledgments

The English used in this manuscript was reviewed by Brian Quinn (Editor-in-Chief, Japan Medical Communication).

References (10)

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    This requires careful review of imaging with experienced radiologists. Tijiri et al.37 retrospectively reviewed 12 patients with PRETEXT III and IV lesions without metastastic disease. Of four patients with P2V3 disease, three downstaged over the course of neoadjuvant treatment (one patient after two cycles and two after four cycles), and one was transplanted for persistent significant vascular involvement.

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    Patients with unifocal PRETEXT IV may become POSTEXT III and be resected with an extended hepatectomy or a mesohepatectomy. See Meyers et al.,36 Meyers et al.,37 Tahiri et al.38 In some multifocal tumors with PRETEXT II or III, a major resection for the main tumor with non-anatomic resection of satellite lesions has been reported, see Qureshi.39 What is still not recommended is to consider a multifocal PRETEXT IV for a localized extended resection if there is radiographic clearance of satellite lesions, and liver transplantation is the current recommendation for such cases.

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The authors declare that they have no conflicts of interest.

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