Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion.
Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion.
复制标题
恶性横纹肌样瘤：临床病理学回顾和概念讨论。
DOI：
--
复制
发表时间：
1995
影响因子：
2.3
通讯作者：
L. Dehner
中科院分区：
文献类型：
--
作者：
M. Wick;J. Ritter;L. Dehner
恶性横纹肌样瘤（MRT）自其开创性描述以来一直是一个有争议的病变。对于它是否代表一种独特的临床病理学实体，或者是几种不同肿瘤可能共有的表型模式，尚无共识。肾MRT是一种儿童肿瘤，与一致的攻击行为有关，但它显示了广泛的组织学，免疫表型和细胞遗传学的结果。恶性肾外横纹肌样瘤（MERT）已经在更广泛的患者年龄和解剖位置范围内观察到了纯粹的形式，但它们显示出与肾MRT的实质性形态和生物学同源性。最后，“复合”肾外横纹肌样瘤（CERT）-其中可识别的“母”肿瘤与MERT混合-也已在几个地形部位被确认。总之，这些观察结果表明，“横纹肌样瘤”是一组异质性病变与不同的分化谱系。特别是在CERT中，横纹肌样表型很可能代表了明显不同起源的肿瘤中克隆进化的共同终点。尽管有这些警告，作者还是支持保留“横纹肌样瘤”的诊断，因为尽管在亚细胞水平上存在潜在的差异，但附属的形态学模式一致地伴随着侵袭性生物学行为。
The malignant rhabdoid tumor (MRT) has been a controversial lesion since its seminal description. There is no consensus as to whether it represents a distinctive clinicopathological entity or, alternatively, a phenotypic pattern that is potentially common to several disparate neoplasms. MRT of the kidney is a childhood tumor that is associated with uniformly aggressive behavior, but it shows a wide spectrum of histologic, immunophenotypic, and cytogenetic findings. Malignant extrarenal rhabdoid tumors (MERTs) have been observed in pure form over a broader range of patient ages and anatomic locations, but they show substantial morphological and biological homology with renal MRT. Lastly, "composite" extrarenal rhabdoid tumors (CERTs)--in which recognizable "parent" neoplasms are admixed with MERTs--also have been recognized in several topographic sites. In aggregate, these observations suggest that "rhabdoid tumors" are a heterogeneous group of lesions with dissimilar lineages of differentiation. Particularly in CERTs, it is likely that the rhabdoid phenotype represents a common end point of clonal evolution in tumors of clearly different origins. Despite these caveats, the authors do support retention of the diagnosis of "rhabdoid tumor," because the affiliated morphological pattern is uniformly attended by aggressive biological behavior despite potential dissimilarities at a subcellular level.