Pancreatic carcinosarcoma with the same KRAS gene mutation in both carcinomatous and sarcomatous components: molecular evidence for monoclonal origin of the tumour

Pancreatic carcinosarcoma with the same KRAS gene mutation in both carcinomatous and sarcomatous components: molecular evidence for monoclonal origin of the tumour
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癌性成分和肉瘤性成分均具有相同 KRAS 基因突变的胰腺癌肉瘤:肿瘤单克隆起源的分子证据

DOI:
10.1111/his.12975
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发表时间:
2016-09-01
期刊:
影响因子:
6.4
通讯作者:
Sheng, Weiqi
Sheng, Weiqi
中科院分区:
医学2区
文献类型:
--
作者:
Bai, Qianming;Zhang, Xin;Sheng, Weiqi

文献摘要

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AimsTo更好地了解胰腺癌肉瘤,一种罕见类型的neoplasia.Methods和resultsWe研究了8个额外的情况下,胰腺癌肉瘤在一个单一的机构,包括临床病理,免疫组化和KRAS突变的特点,组织发生,预后和可行的治疗。我们也回顾了目前关于此罕见类型肿瘤的文献,并总结其临床病理特征及可行的治疗方法。结果显示,在8例癌性和肉瘤性病变中,有5例显示了一致的强核免疫反应性P53蛋白和相同类型的KRAS基因突变,c.35G>A(p.G12D)或c.35G>T(p.G12V)。此外,我们发现手术加术后化疗的患者比仅手术治疗的患者生存期更长(总生存期和无病生存期分别为P = 0.034和P = 0.131),尽管考克斯回归多变量分析表明这不是一个独立的预测因素。此外,我们发现KRAS突变等位基因特异性的不平衡,在我们的5例胰腺癌肉瘤,这是与先进的疾病和预后较差。ConclusionsThis是迄今为止研究的胰腺癌肉瘤的情况下,包括临床病理,免疫组化和分子细胞遗传学特征的最大面板。我们的研究结果表明,肿瘤可能是单克隆起源的,肉瘤成分可能是由癌成分的化生转化引起的。我们的研究结果还表明,手术加POC包括吉西他滨可能是一个很好的选择胰腺癌肉瘤患者。
AimsTo better understand the histogenesis, prognosis and feasible treatment of pancreatic carcinosarcoma, a rare type of neoplasia.Methods and resultsWe investigated eight additional cases of pancreatic carcinosarcoma at a single institution, including the clinicopathological, immunohistochemical, and KRAS mutation characteristics. We have also reviewed the current literature on this rare type of neoplasia, and summarized the clinicopathological features and feasible treatments. As a result, concordant strong nuclear immunoreactivity for P53 protein and the same type of KRAS gene mutation, c.35G>A (p.G12D) or c.35G>T (p.G12V), were showed in both carcinomatous and sarcomatous components in five of eight cases. Furthermore, we found that the patients treated with surgery plus postoperative chemotherapy had longer survival than those treated with surgery only (P = 0.034 and P = 0.131 for overall survival and disease-free survival, respectively), although Cox regression multivariate analysis indicated that it was not an independent predictor. In addition, we found KRAS mutant allele-specific imbalance in four of our five cases of pancreatic carcinosarcoma, which was associated with advanced disease and a worse prognosis.ConclusionsThis is the largest panel of cases of pancreatic carcinosarcoma studied so far, including clinicopathological, immunohistochemical and molecular cytogenetic features. Our findings indicate that the tumour could have been of monoclonal origin, and that the sarcomatous components might have arisen from metaplastic transformation of the carcinomatous components. Our results also suggest that surgery plus POC including gemcitabine may be a good choice for patients with pancreatic carcinosarcoma.