Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors.
Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors.
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DOI：
10.1002/pbc.21834
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发表时间：
2009-03
影响因子：
3.2
通讯作者：
Feusner JH
中科院分区：
文献类型：
--
作者：
Trobaugh-Lotrario AD;Tomlinson GE;Finegold MJ;Gore L;Feusner JH
I期肝母细胞瘤（HB）患者的小细胞未分化（SCU）组织学预测复发风险增加。我们试图确定SCU组织学在不可切除的HB患者中的意义。对入组HB儿科Intergroup（INT 0098）试验的患者和两位作者（MF、LG）个人咨询文件中的患者进行了SCU组织学病例审查。将这些患者与文献综述确定的SCU HB患者进行比较。研究了11名患者。所有报告AFP结果的患者血清AFP水平均正常或轻微升高。无患者存活：10例死于疾病进展，1例死于治疗并发症。免疫染色显示，6名患者中有6名患者的肿瘤呈INI 1阴性。1例患者（和文献综述中的2例患者）的细胞遗传学和分子异常与横纹肌样瘤中描述的相似。与文献综述中的患者进行比较，结果相似，但29例患者中有4例存活，无疾病证据。HB患者的SCU组织学与不良结局相关。这些肿瘤似乎与非SCU HB在生物学上不同。对于患有SCU、HB和/或AFP水平低的儿童，应通过大型合作组研究确定患者特征和结局。与此同时，我们建议不能切除的含SCU成分的HB患者进行仔细的细胞遗传学、分子和免疫组化评估，以确定横纹肌样特征，并接受与其他HB患者不同的治疗。
Small cell undifferentiated (SCU) histology in patients with stage I hepatoblastoma (HB) predicts an increased risk of relapse. We sought to determine the significance of SCU histology in patients with unresectable HB. Patients enrolled on the pediatric Intergroup (INT0098) trial for HB and patients from the personal consultation files of two of the authors (MF, LG) were reviewed for cases with SCU histology. These patients were compared with SCU HB patients identified by literature review. Eleven patients were studied. All patients with reported AFP results exhibited normal or minimally increased serum AFP levels. None of the patients survived: 10 died of disease progression, and 1 died from treatment complications. Immunostaining revealed that tumors from six of six patients tested were INI1 negative. Cytogenetic and molecular abnormalities in 1 patient (and 2 patients from the literature review) were similar to those described in rhabdoid tumors. Comparison with patients from the literature review revealed similar results except that four of 29 patients survived without evidence of disease. SCU histology in HB patients is associated with an adverse outcome. These tumors appear to be biologically different from non-SCU HB. Evaluation of patient characteristics and outcomes for children with SCU HB and/or those with low AFP levels should be determined from large cooperative group studies. In the meantime, we suggest patients with unresectable HB containing SCU elements have careful cytogenetic, molecular, and immunohistochemical evaluation to ascertain rhabdoid features and receive treatment that differs from that provided for other HB patients.
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影响因子：
8.4
作者：
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