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- B1590Interpretation: Ovarian stromal tumor with features of sclerosing stromal tumor and thecoma
| 姓 名： | ××× | 性别： | 年龄： | ||
| 标本名称： | |||||
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| 肉眼检查： | |||||
40-45 y/f with hx of cervical ca with radiation, chemotherapy, ovarian mass 11 cm. I did frozen. Gynecologist came to frozen room to sit with me to read frozen slide.
What can you tell your surgen about the case if your were the pathologist?
What is your differential dx?
I ordered IHC in frozen block and will know basic result tomorrow.
abin译：
20分钟前的卵巢肿瘤冰冻切片。我们能告诉妇科医生什么？
40-45岁女性，有宫颈癌病史和化疗、放疗史。卵巢肿块11cm。我做冰冻切片，妇科医生跑到冰冻室，和我坐在一起看冰冻切片。本例，如果你是病理医生，你能告诉手术医生什么？
你的鉴别诊断是什么？
我用冰冻切片做了免疫组化，明天会知道基本结果。
标签：卵巢 间质肿瘤
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本帖最后由 于 2009-07-24 08:08:00 编辑
相关帖子
×参考诊断
卵巢间质肿瘤，伴硬化性间质瘤和卵泡膜瘤的特征
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本帖最后由 于 2009-05-13 01:23:00 编辑
About two's analyses are very good. Seem that we have similar thought. One of our gynecologic fellows planed to write a case report for this case. She checked the history in details. this patient do have invasive cervical cancer (CIN3 with superficial stromal invasion) and received radiation treatment.
Wait to see if some  ones have other oppinion.
abin译：以上两段分析非常好。似乎我们有相似见解。我们的一名进修医生计划写一个病例报道。她核对了病史，患者确实有过浸润性宫颈癌（CIN3伴浅表间质浸润）并接收过放疗。
等待看是否有人提出不同观点。
看低倍，可见由富细胞区构成的假小叶和少细胞的硬化水肿区，如果是间质肿瘤，也只能归入硬化性间质瘤，但是从来没有见过硬化性间质瘤假小叶区的细胞有这么明确的条索状排列。近年，在硬化性间质瘤的基础上又分出来印戒细胞间质瘤（2003WHO分类）和微囊性间质瘤（Microcystic stromal tumor of the ovary: report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm.Irving JA, Young RH.Am J Surg pathol. 2009 Mar;33(3):367-75.）。难道这是另一种新类型的间质瘤？期待Dr. Zhao的分析讲解。
真是有趣的例子！
- If you have great talents, industry will improve them; if you have but moderate abilities, industry will supply their deficiency. 如果你很有天赋，勤勉会使其更加完美；如果你能力一般，勤勉会补足其缺陷。
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在Dr.Zhao的耐心提醒下，决定回到原点，重头再来！先忘掉可能干扰诊断思路的病史，忘掉以前的见解。
40多岁，单侧卵巢肿块直径11cm，有包膜，切面实性散在囊性变，大部分灰白灰红略带黄色，夹杂水肿区。估计灰白区富于纤维，质地较硬韧。
HE：
有2种结构：
1 大块的肿瘤细胞呈片状、巢状，分布于水肿间质中
2 小片肿瘤细胞或条索状、线状，分布于纤维间质中
细胞学：
胞浆透明，略呈嗜酸性或双嗜性。核卵圆形或短梭形，核膜光滑，染色质纤细，无核仁。细胞异型性不大，未见核分裂。
HE形态考虑性索-间质肿瘤，粒层？支持-间质？间质肿瘤？
IHC：
阳性标记物：VIM，calretinin，WT1
阴性标记物：Inhibin,CD99,SMA,caldesmon,Syn,Cga,ER,PR,S-100,HMB45
Inhibin和CD99同时阴性，可以排除性索成分。VIM、calretinin和WT1均阳性，可以是间质成分。
诊断：
间质肿瘤，良性。可能是硬化性间质瘤或卵泡膜细胞瘤。
华夏病理/粉蓝医疗
为基层医院病理科提供全面解决方案，
努力让人人享有便捷准确可靠的病理诊断服务。
About paper from Dr.Oliva and Young (MGH) is the best paper to describe the morphologic features of SCT. You should read the full paper several times if you want to know the morphology of SCT.
If some one is interested and cannot find the paper, tell me email and I can email you the full paper.
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Am J Surg Pathol. 2005 Feb;29(2):143-56.
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Sertoli cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 54 cases.
James Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA. eoliva@partners.org
Ovarian Sertoli cell tumors are rare, and their morphologic spectrum, behavior, and factors influencing the latter are not clearly established. They may be mimicked by many different tumors, some of them more frequent than Sertoli cell tumors; immunohistochemistry may aid in this differential, but its role has not been analyzed in a large series. We studied the clinicopathologic features of 54 Sertoli cell tumors, including the immunohistochemical profile of 23 of them. The patients, 6 of whom had Peutz-Jeghers syndrome, ranged from 2 to 76 years of age (mean, 30 years). Eleven patients had estrogenic and 4 had androgenic manifestations. The tumors ranged from 0.8 to 30 cm, with the majority being in the range of 4 to 12 cm. They were all unilateral, usually solid, and often yellow. The predominant microscopic pattern was tubular, seen, albeit often only focally, in all tumors; other patterns were cords or trabeculae (28), diffuse (21), pseudopapillary (4), retiform (3), islands or alveolar arrangements (3), and spindled (3). The tubules were solid or hollow with the former being somewhat more common. Delicate septa were occasionally seen and were conspicuous in areas of one tumor. The stroma was abundant in 15 tumors with marked sclerosis in 4. The cells usually had pale to occasionally densely eosinophilic cytoplasm, but 6 tumors were composed of cells with prominent foamy cytoplasm, falling in the category of "lipid-rich" Sertoli cell tumor, and one had cells with clear non-foamy cytoplasm. Forty-four tumors were stage I (42 of them were stage Ia and 2 were stage Ic), 1 was stage II, 3 were stage III, and 6 were not adequately staged. Follow-up was available for 27 patients with stage I tumors, and all were alive and well at last follow-up except for 2 patients with stage Ia and 1 with stage Ic disease. Those 3 patients had pelvic-abdominal recurrences 18, 36, and 9 months, respectively, after the initial diagnosis. Two of the three clinically malignant stage I tumors had moderate to severe cytologic atypia and brisk mitotic activity (>5 or more mitoses/10 high power fields [HPFs]), and one of these had tumor cell necrosis. Among the 10 clinically benign stage I tumors with more than 5 years of follow-up, only 3 had >5 mitoses/10 HPFs, but none had more than mild cytologic atypia and none had tumor cell necrosis. Two of the three patients with stage III disease had follow-up information and one was alive at 16 months and the second developed splenic metastases 2 years after the initial diagnosis. Two of the three stage III tumors had at least moderate cytologic atypia and brisk mitotic activity. Immunohistochemical stains showed positivity for AE1/3-Cam5.2 in 15 of 23 tumors; Epithelial membrane antigen (EMA) was negative in all the tumors. Inhibin was positive in 18 of 22 tumors, calretinin in 10 of 20, CD99 in 19 of 22, vimentin in 17 of 18, smooth muscle actin in 4 of 18, neuron specific enolase in 8 of 16, S-100 in 2 of 20, and chromogranin was negative in all 21 cases studied. Although Sertoli cell tumors usually have a distinctive tubular pattern that facilitates the diagnosis, other patterns may occasionally predominate, causing confusion with various other primary and metastatic ovarian tumors. EMA, inhibin, and chromogranin represent the most helpful triad of immunomarkers serving to exclude two common mimics of Sertoli cell tumors (endometrioid carcinoma [inhibin-; EMA+; chromogranin-] and carcinoid tumor [inhibin-; EMA+; chromogranin+]). Although CD99 and calretinin are often expressed in these tumors, they are much less specific and not as helpful in the differential diagnosis. Most Sertoli cell tumors are stage I, unilateral, cytologically bland, and clinically benign, but occasional examples are high stage, and about 11% of stage I tumors have worrisome histologic features that may portend an adverse outcome. The tumors typically occur in young females, sometimes children who typically present with sexual precocity, and occasional patients have Peutz-Jeghers syndrome.
Above table is my another previous study. Inhibin is positive in 39 of 40 pure sertoli cell tumors.
My study (floor 44) indicated WT1 are positive in sertoli cell tumor, but this case is not a sertoli cell tumor.
Why?
1. Mrphologic features do not support the diagnosis of Sertoli cell tumor. Tubules are most common structure in SCT. It can be found in all SCT if you take more sections. SCT can have many different growth patterns. my current case do not show any cytomorphologic feature of SCT.
2. IHC results do not support SCT, negative inhibin. Of cause negative inhibin is not the only reason to rule out SCT.
TABLE 2: Overall positivity for alternative and traditional immunohistochemical markers
|
Antigen |
Sertoli cell tumor |
Endometrioid borderline tumor |
Sertoliform endometrioid carcinoma |
Well-differentiated endometrioid carcinoma |
Carcinoid tumor |
Epithelial markers
|
CK7* |
13% |
100% |
85% |
100% |
24% |
|
ER* |
8% |
87% |
85% |
89% |
2% |
|
PR* |
13% |
84% |
77% |
93% |
2% |
|
Pan-CK† |
65% |
100% |
100% |
100% |
98% |
|
CK8/18† |
43% |
100% |
93% |
96% |
74% |
|
EMA† |
0% |
100% |
100% |
100% |
12% |
Sex cord markers
|
CD10* |
25% |
39% |
31% |
37% |
9% |
|
Inhibin† |
98% |
0 |
0 |
4% |
2% |
|
Calretinin† |
60% |
11% |
0 |
18% |
0% |
|
CD99† |
68% |
16% |
23% |
33% |
40% |
Neuroendocrine markers
|
CD56* |
48% |
16% |
16% |
30% |
57% |
|
Synaptophysin† |
35% |
8% |
8% |
22% |
98% |
|
Chromogranin† |
13% |
3% |
15% |
11% |
100% |
*, for purposes of this study, CK7, ER, PR, CD10, and CD56 were considered “alternative markers” for the differential diagnosis of ovarian Sertoli cell tumor vs. endometrioid tumors vs. carcinoid tumor
Zhao C, Bratthauer GL, Barner R, Vang R.
Comparative analysis of alternative and traditional immunohistochemical markers for the distinction of ovarian sertoli cell tumor from endometrioid tumors and carcinoid tumor: A study of 160 cases.
Am J Surg Pathol. 2007 Feb;31(2):255-66
So, it is a pure Sertoli cell tumor? without any Leydig cell component?
How about the differentiation? intermediated- poor?
Thank you.
- If you have great talents, industry will improve them; if you have but moderate abilities, industry will supply their deficiency. 如果你很有天赋，勤勉会使其更加完美；如果你能力一般，勤勉会补足其缺陷。
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本帖最后由 于 2009-05-10 13:35:00 编辑
This is a challenge case. When I first pasted the frozen photos here I did not know it would be so complicated case.（这是具有挑战性的病例。当我最初上传冰冻切片时，我不知道它会如此复杂）
It is more difficult for your guys to evaluate the case only based on a few photos.（你们仅根据几张图作出评价会更加困难）
All all agree that this tumor belongs to the category of ovarian sex cord-stromal tumor (SCST), the area I love. I did a lot of IHC studies on scst. I think my seral IHC studies are the most detailed and the most large studies in the world. （所有人都同意它属于卵巢性索-间质肿瘤<SCST>，这是我喜爱的领域。我对SCST做过很多研究。我认为我的几个研究是目前最详细最大宗病例的研究。）
SCSTs include tumors from sex cord and stromal components. There are no classic features of granulosa tumor for my case. Also think about. Did you see the CD99 and inhibin pure negative granulosa tumors in your practice or text books? Leydig cell tumors should be strongly and diffusely postive for calretinin and inhibin, and negativ for WT1. I once reviewed about 300 cases of ovarian pure sertoli cell tumors and sertroli leydig cell tumors for research. But no one like this case.
Now I hope you review all photos and IHC again to think it is a sex cord tumor or stromal tumor.
（SCST包括来自性索和间质成分。我这一例没有典型的粒层细胞瘤特征。也考虑一下，你在实践中和书本上见过CD99和inhibin完全阴性的粒层细胞瘤吗？Leydig细胞应该呈calretinin和inhibin弥漫强阳性，而WT-1阴性。为了研究，我曾经回顾过300例左右的Sertoli细胞瘤和Sertoli-Leydig细胞瘤。但没有一例像本例这样的。现在我希望你们再回顾一下所有图片和IHC，思考一下它是性索-间质肿瘤还是间质肿瘤__abin译）
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谢谢Dr. Zhao提供这么富有挑战性的例子，谢谢各位精彩的讨论。结合21楼和27楼图片，谈点浅显的看法：
在21楼的HE图片中，Dr. Zhao强调了该瘤的特点：条索状、列兵样、或脑回样结构。图1部分间质水肿，图2可见灶状小管结构。部分胞浆透亮，没有看到核沟，有些瘤细胞胞核“一头大一头小”，并且长轴与条索排列相垂直。
在27楼IHC结果示：
Epithelial markers are negative: AE1/AE3/Ck7/ck20/Cam5.2：排除上皮性肿瘤（包括转移和原发）
Other negative markers:
S100, HMB45：排除了恶黑，Mamaglobin：排除了转移性乳腺癌，Inhibin, CD99：阴性也不能排除性索间质肿瘤！SMA, Caldesmon：排除了肌源性肿瘤，Synaptophysin, chromogranin：排除神经内分泌肿瘤（包括原发性类癌），ER, PR：排除乳腺癌、宫内膜样癌等激素依赖性肿瘤，不能排除产生激素的肿瘤。
positive markers： vimentin， calretinin ，WT1
综上，考虑为性索-间质肿瘤，以中-低等分化的支持-间质细胞瘤可能性大。这里需要明确一个概念：支持-间质细胞瘤中，间质细胞不是指单纯的Leydig细胞，泛指卵巢各种特异的及非特异的间质成分，尤其是在中-低分化的支持-间质细胞瘤中，Leydig(莱迪)细胞常常不易见到。请问：该患者有无激素分泌异常的症状？
鉴别诊断包括：1.两性母细胞瘤，如果有明确的粒层细胞成分和支持细胞成分共存。2. 未分类的性索-间质肿瘤，如果形态提示为性索分化，但不能明确属于粒层细胞还是支持细胞。
期待Dr. Zhao给我们大家最后的分析讲解和诊断！
- If you have great talents, industry will improve them; if you have but moderate abilities, industry will supply their deficiency. 如果你很有天赋，勤勉会使其更加完美；如果你能力一般，勤勉会补足其缺陷。
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本帖最后由 于 2009-05-10 13:24:00 编辑
For this case, inhibin and CD99 are purely negative. Also if you read the photos carefully you will notice there are no typical features of granulosa cell tumors. Why did most people still think it is a granulosa tumor?
abin译：本例，Inhibin和CD99完全阴性。如果仔细看HE切片，你也会注意到，它没有粒层细胞瘤的典型特征。为什么大多数人仍然认为它是粒层细胞瘤？
从44楼表中看出：
1、WT-1、SF-1在成人型粒层细胞瘤、支持细胞瘤、支持-间质细胞瘤、纤维瘤/纤维肉瘤中的表达无明显差异。
2、MART-1在粒层细胞瘤阴性，在支持细胞、间质细胞多阳性表达。
3、Inhibin在成人型粒层细胞瘤、支持细胞瘤、支持-间质细胞瘤表达率高，与纤维瘤/纤维肉瘤有明显差异。
4、calretinin在粒层细胞瘤表达率高，在高、中分化的SC表达率低。纤维瘤/纤维肉瘤表达率也低。
5、CD99在粒层细胞瘤表达率高，在高、中分化的SC表达率低。
再看ＨＥ片，结合IHC结果，考虑为粒层细胞瘤。
不知分析的对否？自己水平有限
，表中也有点疑问，请赵老师指导！谢谢！
- 广州金域病理
