Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Patients were imaged after every two cycles and at the conclusion of therapy. All computed tomography scans and pathology reports were centrally reviewed, and information was collected regarding lung nodule number, size, laterality, timing of resolution, and pulmonary surgery. Results Among the 29 evaluable patients, only 31% met Response Evaluation Criteria in Solid Tumors (RECIST) for measurable metastatic disease. The presence of measurable disease by RECIST, the sum of nodule diameters greater than or equal to the cumulative cohort median size, bilateral disease, and ≥ 10 nodules were each associated with an increased risk for an event-free survival event ( P = .48, P = .08, P = .065, P = .03, respectively), with nodule number meeting statistical significance. Ten patients underwent pulmonary resection/metastasectomy at various time points, the benefit of which could not be determined because of small patient numbers. Conclusion Children with metastatic HB have a poor prognosis. Overall tumor burden may be an important prognostic factor for these patients. Lesions that fail to meet RECIST size criteria (ie, those < 10 mm) at diagnosis may contain viable tumor, whereas residual lesions at the end of therapy may constitute eradicated tumor/scar tissue. Patients may benefit from risk stratification on the basis of the burden of lung metastatic disease at diagnosis.

中文翻译：

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儿童肿瘤学组方案 AHEP0731（肝母细胞瘤所有阶段儿童的治疗）治疗的肝母细胞瘤儿童肺转移的特征：来自儿童肿瘤学组的报告

目的 确定转移性肝母细胞瘤 (HB) 儿童肺结节的模式是否与结果相关。方法 将 32 名转移性 HB 患者纳入儿童肿瘤组协议 AHEP0731，并接受长春新碱和伊立替康（VI）治疗。VI 反应者接受两个额外周期的 VI 与六个周期的顺铂/氟尿嘧啶/长春新碱/多柔比星 (C5VD) 混合，无反应者单独接受六个周期的 C5VD。每两个周期后和治疗结束时对患者进行成像。集中审查所有计算机断层扫描和病理报告，并收集有关肺结节数量、大小、侧向性、消退时间和肺部手术的信息。结果 在 29 名可评估患者中，对于可测量的转移性疾病，只有 31% 符合实体瘤反应评估标准 (RECIST)。RECIST 可测量疾病的存在、结节直径总和大于或等于累积队列中位数大小、双侧疾病和≥10 个结节均与无事件生存事件的风险增加有关 (P = .48 , P = .08, P = .065, P = .03, 分别），结节数满足统计显着性。10 名患者在不同时间点接受了肺切除术/转移灶切除术，由于患者人数较少，无法确定其获益。结论转移性HB患儿预后较差。总体肿瘤负荷可能是这些患者的重要预后因素。不符合 RECIST 大小标准的病变（即那些 < 10 毫米）在诊断时可能包含存活的肿瘤，而治疗结束时的残留病灶可能构成根除的肿瘤/疤痕组织。根据诊断时肺转移性疾病的负担，患者可能会受益于风险分层。