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Tajiri T.,Kyoto Prefectural University of Medicine | Tajiri T.,Japanese Study Group for Pediatric Liver Tumor JPLT | Kimura O.,Kyoto Prefectural University of Medicine | Fumino S.,Kyoto Prefectural University of Medicine | And 13 more authors.
Journal of Pediatric Surgery | Year: 2012

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. © 2012 Elsevier Inc.

Arai Y.,National Cancer Center Research Institute | Honda S.,Research Institute for Clinical Oncology | Haruta M.,Research Institute for Clinical Oncology | Kasai F.,Research Institute for Clinical Oncology | And 9 more authors.
Genes Chromosomes and Cancer | Year: 2010

In a single-nucleotide polymorphism array-based analysis of 56 hepatoblastoma (HB) tumors, allelic imbalances were detected in 37 tumors (66%). Chromosome gains were found in 1q (28 tumors), 2q (24), 6p (8), 8q (8), 17q (6), and 20pq (10), and losses in 1p (6), 4q (9), and 16q (4). Fine mapping delineated the shortest overlapping region (SOR) of gains at 1q32.1 (1.3 Mb) and 2q24.2-q24.3 (4.8 Mb), and losses at 4q34.3-q35.2 (8.7 Mb) and 4q32.3 (1.6 Mb). Uniparental disomy of 11pter-11p15.4 (IGF2) and loss of 11pter-p14.1 were found in 11 and 2 tumors, respectively. Expression of HTATIP2 (11p15.1) was absent in 9 of 20 tumors. Amplification was identified in four tumors at 1q32.1, where the candidate oncogene MDM4 is located. In the 4q32.3-SRO, ANXA10S, a variant of the candidate tumor suppressor ANXA10, showed no expression in 19 of 24 tumors. Sequence analysis of ANXA10S identified a missense mutation (E36K, c.106G>A) in a HB cell line. Multivariate analysis revealed that both 4q deletion and RASSF1A methylation (relative risks: 4.21 and 7.55, respectively) are independent prognostic factors. Our results indicate that allelic imbalances and gene expression patterns provide possible diagnostic and prognostic markers, as well as therapeutic targets in a subset of HB. © 2010 Wiley-Liss, Inc.

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