Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney A Rare and Lethal Entity
Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney A Rare and Lethal Entity
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DOI：
10.5858/arpa.2014-0367-rs
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发表时间：
2016-03-01
影响因子：
4.6
通讯作者：
Cai, Guoping
中科院分区：
文献类型：
--
作者：
Celli, Romulo;Cai, Guoping
尤文氏肉瘤/原始神经外胚层肿瘤代表了一系列具有相似生物学特性的未分化肿瘤，它们共同代表了儿科-青年年龄范围内第二常见的肉瘤。这种肿瘤很少表现为原发性肾脏肿瘤。该肿瘤的临床表现不明确，其他肾脏肿瘤可能表现出类似的组织学表现。建立正确的诊断是至关重要的，因为肾尤因肉瘤/原始神经外胚层肿瘤的预后非常糟糕，因此需要特定的治疗策略。建议使用辅助分子检测的低阈值，特别是在诊断有问题的病例中。关于形态学、免疫组织化学和分子改变的重要考虑将在这里进行回顾，并在做出这种罕见且致命的诊断之前应予以考虑。
Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.