孔祥田(Max)*Sacramento*CA] 03-23 12:43

56yo F fibroids treated with hormone, then hysterectomy performed. There is a 7 cm yellowish nodule in myometrium protruding to uterine cavity. Here are some pictures from this nodule.

孔祥田(Max)*Sacramento*CA] 03-23 12:43

孔祥田(Max)*Sacramento*CA] 03-23 12:43

孔祥田(Max)*Sacramento*CA] 03-23 12:43

孔祥田(Max)*Sacramento*CA] 03-23 12:44

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孔祥田(Max)*Sacramento*CA] 03-23 12:44

孔祥田(Max)*Sacramento*CA] 03-23 12:44

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孔祥田(Max)*Sacramento*CA] 03-23 12:44

孔祥田(Max)*Sacramento*CA] 03-23 12:44

孔祥田(Max)*Sacramento*CA] 03-23 12:44

孔祥田(Max)*Sacramento*CA] 03-23 12:45

孔祥田(Max)*Sacramento*CA] 03-23 13:00

杨鹏 淄博市妇幼保健院 03-23 15:55

HE 有些地方似卵巢性索肿瘤的子宫肿瘤。

孔祥田(Max)*Sacramento*CA] 03-23 20:56

@杨鹏 淄博市妇幼保健院 besides SF-1 negative, inhibin, calretinin, FOXL2 are all negative. Therefore, UTROSCT is out of the DDx.

孔祥田(Max)*Sacramento*CA] 03-23 21:13

Several questions we need to think about the case. 1) we all know that either PEComa or leiomyoma should have convinced desmin staining to support. However, this case has faint or almost negative desmin staining, can we rule out those two ddx based on desmin staining? 2) WT-1 and cd10 positive, can we allow those two stains positive for either PEComa or leiomyoma? Or those two positive stains support choice C? 3) HMB45 is focal, is the focal HMB45 staining supporting PEComa or not? Have you ever seen PEComa with such focal HMB45 staining? 4) tongue-like growth pattern of this tumor can be good for choice a) and c), and morphologically it’s hard to tell them apart. Do wt-1 and cd10 staining plus focal HMB45 support choice c? 5) do you want more work up to narrow down your ddx? What are you going to do?

姜彦多 沈阳202医院 03-24 10:13

@孔祥田(Max)*Sacramento*CA] 孔教授您好，我认为根据形态和现有的IHC结果，最支持诊断C，但不能完全除外A，理由如下：1.肌性标记物阴性不能除外PEComa，少数肿瘤是TFE3异位相关的，其特点就是以上皮样透明细胞为主，肌性标记物弱表达或阴性，但通常黑色素标记物强阳性，而本例两种黑色素标记物为弱阳性或阴性。2.本例鉴别诊断中，WT1和CD10阳性最支持ESS，文献也有报道ESS中的透明细胞可以表达黑色素标记物。我经历的病例和看到的文献中，似乎没有见到PEComa或肌瘤表达WT1。3.PEComa表达黑色素标记物，通常是HMB45或MelanA，但有时这两种标记物阴性，而表达其他黑色素标记物，如MiTF和Ca thepsin K等，所以应该使用一组抗体。4.建议做TFE3基因重排和低级别ESS相关的遗传学检测，可以明确诊断。

孔祥田(Max)*Sacramento*CA] 03-24 10:21

@姜彦多 沈阳202医院 分析合理

孔祥田(Max)*Sacramento*CA] 03-24 10:37

姜彦多 沈阳202医院 03-24 10:40

还是不能除外PEComa。TFE3蛋白强阳性有一定意义，否则没有什么意义，直接做基因重排检测才可靠。

上海闵行中心医院李国霞，群管 03-24 10:41

HMB45是有小片阳性是吧，子宫内膜间质肉瘤一般HMB45阴性吧。如果是PEComa，可以像这样从子宫内膜开始就有病变，一直延伸到子宫肌壁吗？

姜彦多 沈阳202医院 03-24 10:42

@上海闵行中心医院李国霞，群管 可以

上海闵行中心医院李国霞，群管 03-24 10:43

如果可以，我选PEComa。我看不出低级别间质肉瘤的形态来，没有螺旋小动脉嘛 。

上海闵行中心医院李国霞，群管 03-24 10:44

@姜彦多 沈阳202医院 ，谢谢姜老师！

上海闵行中心医院李国霞，群管 03-24 10:45

大概10%的PEComa会TFE3阳性

姜彦多 沈阳202医院 03-24 10:47

AJCP2014.141:850-855报道，ESS中出现透明细胞时可以表达黑色素标记物，本例是以透明细胞为主

上海闵行中心医院李国霞，群管 03-24 10:47

上海闵行中心医院李国霞，群管 03-24 10:48

这里面染色怎么是淡粉红色的?@孔祥田(Max)*Sacramento*CA]

姜彦多 沈阳202医院 03-24 10:49

期待TFE3基因重排和低级别ESS相关的遗传学检测结果

孔祥田(Max)*Sacramento*CA] 03-24 10:55

@上海闵行中心医院李国霞，群管 I used red chromogen for HMB45. Red is positive signal.

孔祥田(Max)*Sacramento*CA] 03-24 11:10

@姜彦多 沈阳202医院 @上海闵行中心医院李国霞，群管 I signed it out as spindled/epithelioid cell tumor with clear cell features, combined with the IHC findings favor PEComa. Then I wrote a long comment. This case is a tough one. I spent a week to figure it out with all those questions in my mind and step by step troubleshooting. PEComa can be negative for desmin, can be focally positive for HMB45. WT-1 can be positive in PEComa per report. Yes, LG ESS can have expression of melanocytic markers per report. MiTF is the one I ordered last in my work up. When I see such strong staining on MiTF, I believe it’s good for PEComa. I added TFE3 after I signed it out and will issue an addendum for that. Per Dr. Soslow’s paper in 2015, PEComa with TFE3 Translocation has worse prognosis. IHC positive for TFE3 doesn’t mean it will have the translocation. FISH maybe the final way if TFE3 IHC is positive. Let’s see. Thanks you all for the discussion. I don’t mention your names one by one here.

姜彦多 沈阳202医院 03-24 11:21

@孔祥田(Max)*Sacramento*CA] 谢谢孔教授分享

慧馨 03-24 14:19

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