Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.
J Pediatr Surg. 2012 Dec;47(12):2194-8. doi: 10.1016/j.jpedsurg.2012.09.006.
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.
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.
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.
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.
本研究旨在根据两家机构的经验，评估初始诊断为不可切除肝母细胞瘤的手术策略。
回顾性分析了 1998 年至 2011 年两家机构根据 JPLT-2（日本小儿肝肿瘤研究组-2）方案，对 29 例 M(-)肝母细胞瘤中 PRETEXT III 或 IV 期和 PRETEXT III 或 IV 期的 12 例患儿的 PRETEXT（肿瘤术前评估）和 POST-TEXT（术后疾病程度）分期、手术治疗和临床结果。
9 例 PRETEXT III 期中有 2 例降期为 POST-TEXT II 期。3 例 PRETEXT IV 期中有 1 例降期为 POST-TEXT III 期。PRETEXT 分期系统中 7 例 P2 或 V3（提示肝移植）中有 4 例在 CITA（JPLT-2 标准方案）2 个周期后 P2 或 V3，1 例在初始手术时 P2 或 V3，并进行了原发性肝移植。初始手术治疗为 1 例肝叶切除术、2 例节段切除术、6 例三叶切除术、2 例中肝切除术和 1 例原发性肝移植。行中肝切除术的 2 例患者均有胆漏，5 例三叶切除术中有 1 例右肝静脉急性梗阻。2 例患者行挽救性活体肝移植。这 2 例患者在 CITA 2 个周期后 POST-TEXT 分期均为 P2 或 V3 阳性。
对于初始诊断为不可切除的肝母细胞瘤，在 CITA 2 个周期后应评估 POST-TEXT 分期和 P、V 因子。如果此时 POST-TEXT IV、P2 或 V3 阳性，应将患者转至移植中心。鉴于手术并发症的发生率，建议行三叶切除术进行肝切除术。对于初始 CITA 2 个周期后出现 POST-TEXT IV、P2 或 V3 状态的病例，应考虑备用移植等精心治疗。
