报道五例女性生殖道中的类间肾样腺癌（MLAs）的新观察结果。其中两例为子宫内膜类间肾样腺癌，伴有内膜样癌和非典型增生，另外三例为含为肉瘤组织（介于间肾样癌和肉瘤之间的肉瘤瘤）的病例（其中一例为子宫内膜类间肾样癌，另两例是卵巢的病例）。所有病例中都检测出了MLA特征性的KRAS突变，有趣的是，在其中的混合癌症中，KRAS突变仅限于内膜样组织中。在一例病例中的与MLA、内膜样癌和非典型增生同时存在的组织中，检测到了相同的EGFR、PTEN和CCNE1突变，这表明非典型增生可能会导致含内膜样和类间肾样组分的Müllerian癌症。所有的肉瘤瘤均含有MLA组分和软骨样成分的肉瘤组织。在卵巢肉瘤瘤中，上皮组分和肉瘤组分均含有一些共同的突变，包括KRAS和CREBBP，这表明它们可能是同源的。此外，在一例肉瘤瘤病例中，检测到的MLA和肉瘤组分中的CREBBP和KRAS突变也在相关联的未分化癌组分中检测到，这表明它与MLA和肉瘤组分可能具有同一克隆性。我们的研究提供了更多证据，证明MLAs具有Müllerian来源，并且描述了包含软骨样成分在内的类间肾样癌瘤，这是其特征。在报道这些发现时，我们提供了区分类间肾样肉瘤和带有纺锤细胞组分的MLA的建议。本文受版权保护，版权所有。

To report novel observations in five mesonephric-like adenocarcinomas (MLAs) of the female genital tract.We report two endometrial MLAs in association with endometrioid carcinoma and atypical hyperplasia and three (one endometrial, two ovarian) cases with a sarcomatoid component (mesonephric-like carcinosarcoma). Pathogenic KRAS mutations, which are characteristic of MLA, were identified in all cases, although interestingly in one of the mixed carcinomas, this was confined to the endometrioid component. The concurrent MLA, endometrioid carcinoma and atypical hyperplasia components in one case harboured identical EGFR, PTEN, and CCNE1 mutations, suggesting that the atypical hyperplasia gave rise to a Müllerian carcinoma with both endometrioid and mesonephric-like components. The carcinosarcomas all contained a component of MLA and a sarcomatous component with chondroid elements. In the ovarian carcinosarcomas, the coexisting epithelial and sarcomatous components shared some mutations including KRAS and CREBBP, suggesting that they are clonally related. Furthermore, in one case CREBBP and KRAS mutations detected in the MLA and sarcomatous components were also detected in an associated undifferentiated carcinoma component, suggesting that it was clonally related to the MLA and sarcomatous components.Our observations provide additional evidence that MLAs have a Müllerian origin and characterize mesonephric-like carcinosarcomas in which chondroid elements appear to be characteristic. In reporting these findings, we provide recommendations for distinction between a mesonephric-like carcinosarcoma and a MLA with a spindle cell component.This article is protected by copyright. All rights reserved.