Synchronous occurrence of malignant rhabdoid tumor two decades after Wilms' tumor irradiation.
Synchronous occurrence of malignant rhabdoid tumor two decades after Wilms' tumor irradiation.
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维尔姆斯肿瘤照射二十年后，恶性横纹肌样瘤同时发生。
DOI：
10.1097/00000478-199307000-00011
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发表时间：
1993
期刊：
影响因子：
--
通讯作者：
Barsky,SH
中科院分区：
文献类型：
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作者：
Litman,DA;Bhuta,S;Barsky,SH
我们描述了第一例同时发生在骨盆和肺部的恶性横纹肌样瘤，在这两个部位接受Wilms瘤放射治疗20年后。尽管恶性横纹肌样瘤的表型作为一种特殊的临床病理实体是有争议的，但该病例表现出恶性横纹肌样瘤的典型临床病理特征，包括小梁-肺泡生长模式的组织特征；特征性嗜酸性细胞质内含物的细胞特征，表现为中间细丝团、大核和突出的中央核仁，以及间质和上皮双重免疫细胞化学特征；以及诊断后2个月迅速恶化导致死亡的临床特征。Wilms瘤照射部位的同步恶性横纹肌样瘤的发生提出了一些有趣的问题，涉及到辐射诱发的恶性肿瘤与假定的肿瘤抑制基因缺陷的关系，同步二期与原发复发和转移的区别，以及恶性横纹肌样瘤与Wilms瘤的典型关系。
We describe the first case of synchronous malignant rhabdoid tumor arising in the pelvis and the lung two decades after both sites were irradiated for Wilms' tumor. Although the malignant rhabdoid tumor phenotype is controversial as a specific clinicopathological entity, this case exhibited classic clinicopathological features of malignant rhabdoid tumor, including tissue features of a trabecular to alveolar growth pattern; cellular features of characteristic eosinophilic cytoplasmic inclusions exhibiting intermediate filament clusters, large nuclei with prominent central nucleoli, and a dual mesenchymal and epithelial immunocytochemistry profile; and clinical features of a rapidly deteriorating course leading to death 2 months after diagnosis. The occurrence of synchronous malignant rhabdoid tumors in sites irradiated for Wilms' tumor raise interesting questions concerning the relationship of radiation-induced malignancies to putative tumor suppressor gene defects, the distinction of synchronous secondaries from primary recurrences and metastases, and finally the quintessential relationship of malignant rhabdoid tumor to Wilms' tumor.