Fonseca Adriana, Gupta Abha, Shaikh Furqan, Ramphal Raveena, Ng Vicky, McGilvray Ian, Gerstle J Ted
Division of Hematology Oncology, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Canada.
Pediatr Blood Cancer. 2018 Feb;65(2). doi: 10.1002/pbc.26820. Epub 2017 Sep 17.
Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible.
To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT.
Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015.
Among six children (median age 12 months (3-41 months)), PRETEXT classification was III (n = 2), III/IV (n = 1), and IV (n = 3). Patients received a median of six cycles (range 4-7) of platinum-based induction chemotherapy; five received doxorubicin. Experienced pediatric surgeons performed extended right and left hepatectomy in five and one patients, respectively, with assistance of an experienced liver transplant surgeon (n = 4). Microscopic margins were positive (n = 2) and negative but close (n = 4; 2-5 mm). Two patients required vascular reconstruction of the vena cava. At median follow-up of 3.3 years (1.7-4.6 years), there was no evidence of local recurrence. One patient had recurrence of pulmonary disease 3 months after surgery.
Patients with advanced HB treated with complex surgical resections with positive or close negative margins had good outcomes without OLT. We suggest that planned positive or close microscopic margins in highly selected HB patients may spare the morbidity of OLT and offer an alternative for those ineligible for OLT. Our experience illustrates the importance of a multidisciplinary team specialized in the management of liver tumors.
原位肝移植(OLT)被认为是无法进行完整手术切除的肝母细胞瘤(HB)患儿的标准治疗方法。然而,OLT并非总是可用或可行。
描述最初被认为无法切除且接受了切缘计划紧密的复杂肝切除术并最终避免了OLT的HB患儿的治疗结果。
收集了2010年1月至2015年12月期间接受HB治疗的患儿的人口统计学数据、手术和病理细节以及生存信息。
6名患儿(中位年龄12个月(3 - 41个月))中,PRETEXT分类为III期(n = 2)、III/IV期(n = 1)和IV期(n = 3)。患者接受了中位6个周期(范围4 - 7个周期)的铂类诱导化疗;5名接受了阿霉素治疗。经验丰富的小儿外科医生分别为5名和1名患者实施了扩大右肝切除术和扩大左肝切除术,有1名经验丰富的肝移植外科医生协助(n = 4)。显微镜下切缘阳性(n = 2),阴性但切缘紧密(n = 4;2 - 5毫米)。2名患者需要进行腔静脉血管重建。中位随访3.3年(1.7 - 4.6年)时,无局部复发迹象。1名患者术后3个月出现肺部疾病复发。
接受切缘阳性或切缘紧密阴性的复杂手术切除治疗的晚期HB患者,未进行OLT也取得了良好的治疗结果。我们建议,在经过严格筛选的HB患者中,计划切缘阳性或紧密阴性可能避免OLT的并发症,并为那些不符合OLT条件的患者提供一种替代方案。我们的经验说明了一个专门管理肝肿瘤的多学科团队的重要性。