METHODS: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed.
RESULTS: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free.
CONCLUSIONS: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.
方法:回顾了2010年至2023年在研究中心接受治疗的18三体综合征儿童的病历。
结果:69例患者中有6例发生肝母细胞瘤,其中三人接受了多学科治疗。此外,6名患者已被另一家医院转诊接受治疗,其中四人接受了多学科治疗。在接受多学科治疗的7名患者中,三,两个,和两个被归类为治疗前疾病程度(PRETEXT)分类组I,II,III,分别。3例进行新辅助化疗导致肿瘤缩小。在所有情况下,在病理安全的边缘实现了完全切除.围手术期并发症包括循环衰竭1例,胆漏2例。辅助化疗4例。术后观察期3个月至11年,所有的病人都没有复发.
结论:18三体综合征合并肝母细胞瘤的儿童,其心肺状况稳定,可能是化疗和手术的良好候选者。