Uterine Mesenchymal Tumors: Update on Classification, Staging, and Molecular Features.
Uterine Mesenchymal Tumors: Update on Classification, Staging, and Molecular Features.
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DOI：
10.1016/j.path.2019.01.004
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发表时间：
2019-06-01
影响因子：
--
通讯作者：
Howitt, Brooke E
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文献类型：
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作者：
Parra-Herran, Carlos;Howitt, Brooke E
近年来，子宫间质瘤的范围有所扩大。首先，普遍的，复发性分子改变的识别导致了子宫内膜间质瘤的生物学和临床一致的分类。同样，在最近的病例系列（血管周围上皮样细胞瘤、炎性肌成纤维细胞瘤）中，一些罕见和杂项肿瘤的诊断标准也得到了改进。纯间充质肿瘤仍然是根据肿瘤细胞表型根据形态学进行分类的。以平滑肌肿瘤居多，其次为子宫内膜间质源性肿瘤；后者将在本文中深入讨论，重点是定义分子改变及其形态学和临床相关性。其余的实体包括一个杂项组，其中细胞来源在子宫中没有正常的对应物（例如，横纹肌肉瘤）或不明确（例如，未分化子宫肉瘤）。本文讨论了它们的临床相关性，最近对其分子生物学的见解，以及最重要的鉴别诊断。对于后者，免疫组织化学和（越来越多的）分子诊断在诊断工作中发挥作用。我们总结了一些关于术中咨询和宏观检查的考虑，以及根据最新的美国联合癌症委员会和国际妇产联合会分期系统对子宫肉瘤的病理分期和分级。
The spectrum of mesenchymal neoplasia in the uterus has expanded in recent years. First, the identification of prevalent, recurrent molecular alterations has led to a more biologically and clinically congruent classification of endometrial stromal tumors. Likewise, the diagnostic criteria of several rare and miscellaneous tumor types have been refined in recent case series (Perivascular Epithelioid Cell tumor, inflammatory myofibroblastic tumor). Pure mesenchymal tumors are still broadly classified based on morphology according to the tumor cell phenotype. Smooth muscle tumors predominate in frequency, followed by tumors of endometrial stromal derivation; the latter are covered in depth in this article with an emphasis on defining molecular alterations and their morphologic and clinical correlates. The remaining entities comprise a miscellaneous group in which cell derivation does not have a normal counterpart in the uterus (eg, rhabdomyosarcoma) or is obscure (eg, undifferentiated uterine sarcoma). This article discusses their clinical relevance, recent insights into their molecular biology, and the most important differential diagnoses. Regarding the latter, immunohistochemistry and (increasingly) molecular diagnostics play a role in the diagnostic workup. We conclude with a few considerations on intraoperative consultation and macroscopic examination, as well as pathologic staging and grading of uterine sarcomas as per the most recent American Joint Cancer Commission and the Federation Internationale de Gynecologie et d'Obstetrique staging systems.