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卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)

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楼主 发表于 2010-12-01 18:13|举报|关注(4)
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姓    名: ××× 性别:  女 年龄:  25岁
标本名称:  右卵巢肿块
简要病史:  足月顺产2个月后,自觉右下腹包块,无腹痛,无不规则阴道流血及异常阴道排液。妇科检查:右附件区扪及约10×7cm大小的肿块,质硬,活动差,无压痛。盆腔CT示右侧附件占位,伴有盆腔积液
肉眼检查:  右侧卵巢肿瘤大小约9.5×8.5×6cm,有包膜,表面光滑,略呈分叶状。切面灰黄色,部分呈桔黄色,局灶伴出血和微囊变,质地偏硬。
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图1
    图1
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图2
    图2
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图3
    图3
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图4
    图4
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图5
    图5
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图6
    图6
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图7
    图7
  • 卵巢肝样肿瘤-罕见病例(2010上海-大阪-墨尔本病理读片会病例1,同济大学东方医院提供)图8
    图8
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王军臣
×参考诊断
肝样SLCT。

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61 楼    发表于2010-12-13 12:30:00举报|引用
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以下是引用shandongzhang在2010-12-13 9:31:00的发言:

 肝样分化的肿瘤    是最后的诊断?


不是最后诊断。

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62 楼    发表于2010-12-13 12:42:00举报|引用
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最后诊断:具有肝样分化的Sertoli-Leydig细胞瘤(SLCT,低分化。
也就是金主任在引用文献中称为分泌AFP的
SLCT。
本例也称为:肝样SLCT

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63 楼    发表于2010-12-13 16:54:00举报|引用
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本帖最后由 于 2010-12-14 06:02:00 编辑

D'Antonio A, De Dominicis G, Addesso M, Caleo A, Boscaino A.

Hepatoid carcinoma of the ovary with sex cord stromal tumor: a previously unrecognized association.unrecognized association. Arch Gynecol Obstet. 2010:765-8.

BACKGROUND: Hepatoid carcinoma (HC) of ovary is a rare type of epithelial tumor composed mainly of epithelioid cells with abundant acidophilic cytoplasm,histologically indistinguishable from hepatocellular carcinoma. We report a previously unrecognized case of HC of ovary concurrent with a Sertoli cell tumor. CASE REPORT: A 42-year-old woman patient with a long-term history of hepatitis C  presented with a mass of left ovary without evidence of hepatic tumor. After initial diagnosis of primary ovarian carcinoma (FIGO Stage I), she had experienced a first recurrence in upper abdomen. Histologically, the primary tumor was composed of epithelioid cells with "hepatoid features" in association with a sex cord stromal tumor of Sertoli-type. Immunohistochemistry hepatoid cells stained positively for hepatocyte paraffin-1, alpha-fetoprotein and alpha-1 antitrypsin; moreover, Sertoli-type cells were positive for alpha-inhibin, calretinin and CD99. A final diagnosis of HC concurrent with Sertoli-type tumor was made. CONCLUSION: The occurrence of this unreported association of HC with Sertoli-like tumor, the problems of differential diagnosis and therapeutic management of these tumors are the subject of this presentation. A diagnosis of ovarian metastasis from hepatocellular carcinoma is easy in patients with known primary tumor of liver and should be always excluded in these cases as an hepatoid variant of yolk sac tumor. Immunohistochemistry is not useful in these cases. However, a combination of clinical and pathological features is necessary for a correct diagnosis.

 

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64 楼    发表于2010-12-13 17:13:00举报|引用
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本帖最后由 于 2010-12-14 06:12:00 编辑

 

极为罕见的病例,非常感谢王主任的分享!

1)文献已报道卵巢可分泌AFP的肿瘤包括:卵黄囊瘤、伴有卵黄囊成分的生殖细胞肿瘤、卵巢表面上皮癌(浆液、粘液、内膜样)伴有肝样分化(肝样腺癌)、性索间质肿瘤伴有肝样分化、苗勒氏管混合性肿瘤。

2)如何鉴别性索间质肿瘤(支持-间质细胞瘤)伴肝样分化和肝样癌伴性索间质肿瘤成分?二者是一回事还是二回事?

3) 此病例如诊断为“伴肝样分化的支持-间质肿瘤”,可能为世界英文文献报道第26例;诊断为“肝样腺癌伴性索间质肿瘤”, 为文献报道的第2例。

4)如何鉴别诊断及其临床意义(肿瘤的恶性程度和治疗建议)?

5)理论上如何解释“性索间质细胞向肝细胞分化”?

 

谢谢!

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65 楼    发表于2010-12-14 08:28:00举报|引用
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 学习
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66 楼    发表于2010-12-14 14:34:00举报|引用
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67 楼    发表于2010-12-14 20:06:00举报|引用
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 好病例,学习了!学习明月老师和金老师!
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68 楼    发表于2010-12-06 19:02:00举报|引用
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 卵巢的肝样腺癌
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69 楼    发表于2010-12-06 19:04:00举报|引用
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 Alpha-fetoprotein producing tumors other than hepatoma and germ cell tumors have been widely reported, especially in carcinoma with hepatoid differentiation (hepatoid carcinoma). Hepatoid carcinoma has mostly been found in the stomach, but also occurs in many other organs. A rare case of hepatoid carcinoma of the ovary is presented. A 57-year-old Taiwanese woman was admitted because of lower abdominal pain. Magnetic resonance imaging showed a 10 cm right adnexal mass. She underwent a total hysterectomy and bilateral salpingo-oophorectomy with omentectomy. A right ovarian mass measuring 13 x 9 x 8 cm was found. Microscopic examination showed characteristic features for hepatoid carcinoma. Immunohistochemical staining was performed on the tumor using a panel of eight markers (AFP, p-CEA, CD10, Hep Par 1, thyroid transcription factor-1, CK7, CK19 and CK20). This study contradicts the theory that hepatoid carcinoma derives from the surface epithelium of the ovary. Hepatoid carcinoma of the ovary commonly contains a population of clear cells, which may lead to the misdiagnosis of yolk sac tumor or clear cell adenocarcinoma that may arise in many anatomic sites. Histologically, it is also difficult to distinguish hepatoid carcinoma from hepatoid yolk sac tumor. In such cases, demonstration of CD 10, Hep Par 1, membraneous patterns of p-CEA and CK7 would be invaluable for characterizing the tumor as hepatoid carcinoma. More studies are needed to confirm this observation.
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70 楼    发表于2010-12-06 20:00:00举报|引用
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 肝样卵黄囊瘤
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71 楼    发表于2010-12-06 22:25:00举报|引用
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 卵巢肝样癌
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72 楼    发表于2010-12-06 23:09:00举报|引用
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 卵巢肝样癌和肝样腺癌哪些标志物阳性?

卵巢卵黄囊瘤哪些标志物阳性?

卵巢krukenberg tumor哪些标志物阳性?AFP阳性?

与妊娠有关的支持-间质细胞肿瘤是什么概念?哪些标志物阳性?

卵巢印戒细胞间质肿瘤哪些标志物阳性?

卵巢原发的到底有哪些肿瘤会是高表达AFP?

会有哪些AFP表达的肿瘤将可能转移到卵巢?

这些都是我们要思考的问题。“众人拾柴火焰高”。请大家一起来讨论这些问题。知多少说多少。谢谢!

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73 楼    发表于2010-12-01 18:19:00举报|引用
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 图1 ×25
 图2 ×200
 图3 ×200
 图4 ×50
 图5 ×200
 图6 ×50
 图7 ×200
 图8 ×200

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74 楼    发表于2010-12-01 19:41:00举报|引用
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 该患者在产后两个月发现右下腹包块去医院检查,实验室检测显示:血清AFP水平显著升高,达到60500.00 ng/ml。而CA-125HCG等血中含量未发现异常。
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75 楼    发表于2010-12-11 18:36:00举报|引用
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IHC结果:
图1 AFP
图2 Hep Par1
图3 a1-AT
图4 CKpan
图5 CAM5.2
图6 CK7
图7 EMA
图8 WT1
图9 inhibin
图10 CD99
图11 Calretinin
图12 CEA
  • 图1
  • 图2
  • 图3
  • 图4
  • 图5
  • 图6
  • 图7
  • 图8
  • 图9
  • 图10
  • 图11
  • 图12
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76 楼    发表于2010-12-11 18:47:00举报|引用
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IHC全部结果如下:
阳性标记:AFP、HepPar1、a-1-AT、WT1、CD99、CKpan、CAM5.2
小灶或点状阳性:inhibin、Calretinin、PR
阴性标记:CK7、EMA、CEA、CA125、CA19.9、CD10、CD15、CD30、actin、HCG、  CgA、D2-40、Vimentin、AR、ER、HMB45
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77 楼    发表于2010-12-12 18:41:00举报|引用
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本帖最后由 于 2010-12-31 23:23:00 编辑 组织形态具有梁状、窦状血管等特点,细胞浆呈嗜酸性着色,呈肝样形态特征,尽管有的区域的细胞呈现胞浆空泡化(如同脂肪变)。免疫标记见HepPar1、a-1-AT、AFP的表达,支持肝样表型,不管是明显肝样区域还是空泡化的细胞区域。形态结合IHC,支持肝样分化的肿瘤
肝样癌或肝样腺癌?虽然CKpan和CAM5.2阳性,但CK7和EMA阴性。CA125和CA19.9也是阴性。因此,不支持肝样癌或肝样腺癌
肝样卵黄囊瘤(YST)?YST的诊断需结合临床和形态学特点。不管是何种组织来源的肿瘤,即便是差分化,只有取材合理,总能寻找到肿瘤起源的蛛丝马迹。本例虽然见到细胞空泡化的区域,但细胞界限清楚,见不到空泡化后形成的大片网状区域。YST过去称为内胚窦瘤,但本例见不到有YST特征性的Shiller-Duvei内胚窦小体和均质红染小球。虽然有作者说IHC不总是有鉴别诊断的帮助,但实质上还是有帮助的,YST不表达WT1,而本例却表达。即便是表达AFP,但在YST表达AFP多是呈灶性阳性,不是像本例那样弥漫强阳性。所以,也不是肝样YST
肝样krukenberg tumor或肝样转移性肿瘤?也不是。首先,转移癌要发现其他部位有原发癌,本例的临床、影像学和血清学检查,没有特别的发现。要是癌,那会表达EMA的,可是本例不表达。因此,综合起来也不支持肝样转移性肿瘤。 
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78 楼    发表于2010-12-13 09:31:00举报|引用
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 肝样分化的肿瘤    是最后的诊断?

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79 楼    发表于2010-12-13 10:22:00举报|引用
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本帖最后由 于 2010-12-14 06:06:00 编辑
以下是引用XLJin8在2010-12-13 10:22:00的发言:

 

1. Indian J Cancer. 2009 Jan-Mar;46(1):64-6. Recurrent alpha-fetoprotein secreting Sertoli-Leydig cell tumor of ovary with an unusual presentation.

Poli UR, Swarnalata G, Maturi R, Rao ST.

Department of Surgical Oncology, MNJ Institute of Oncology and Regional Cancer Centre, Hyderabad, India. ushapoli@yahoo.co.in

Alpha-fetoprotein secreting (AFP) Sertoli-Leydig cell tumors of ovary (SLCT) are now identified as a distinct entity among the uncommon group of sex cord tumors of ovary. We report an unusual case of recurrent AFP secreting ovarian tumors and as ileocecal mesenteric cyst in a 25-year-old patient resulting in difficulty in initial diagnosis of AFP producing SLCT. Although six recurrent cases were described out of the 25 reported cases of AFP secreting SLCTs, this patient with an unusual presentation of recurrence is the second case in the literature to the best of our knowledge.

 

2. Gan No Rinsho. 1989 Jan;35(1):107-13.

[An alpha-fetoprotein producing Sertoli-Leydig cell tumor--a case report] [Article in Japanese]

Taniyama K, Suzuki H, Hara T, Yokoyama S, Tahara E.

Dept. of Pathology, Shizuoka General Hospital.

A case of an ovarian, poorly differentiated, Sertoli-Leydig cell tumor in a 55-year-old woman is reported. The patient showed no virilization, but did show elevated serum alpha-fetoprotein (AFP) levels. The tumor, measuring 70x45x50 mm, consisted mainly of a diffuse proliferation of spindle cells.Among this proliferation, some tubules of Sertoli cells and groups of leydig cells were found. Immunoperoxidase studies, using anti-AFP, localized the AFP in the leydig cells.

 

3. Arch Gynecol. 1985;236(3):187-96.

AFP-producing Sertoli-Leydig cell tumor of the ovary.

Sekiya S, Inaba N, Iwasawa H, Kobayashi O, Takamizawa H, Matsuzaki O, Nagao K.

A tumor of the right ovary in a 21-year-old single woman is reported. Secondary amenorrhea, hirsutism, acne and deepening of the voice were associated with the tumor. Light and electron microscopic examinations showed that the tumor was composed of cells resembling Sertoli and Leydig cells of the testis in their cytology features and growth patterns. High levels of circulating dehydroepiandrosterone, androstenedione, testosterone and alpha-fetoprotein (AFP) were found preoperatively. Preoperative estrogen and progesterone levels were all slightly above the upper limits of normal for females. These hormone and AFP levels fell to within the normal range after removal of the tumor. Direct hormone and AFP production of this tumor was confirmed by immunohistochemical techniques  and long-term cell cultures in vitro. This is possibly the first report on a Sertoli-Leydig cell tumor in which AFP has been identified in the patient's plasma, in part of the tumor cells and the culture fluid.

 

4. Int J Gynecol Pathol. 1984;3(2):213-9.

Sertoli-Leydig cell tumor of the ovary producing alpha -fetoprotein.

Chumas JC, Rosenwaks Z, Mann WJ, Finkel G, Pastore J.

A 16-year-old girl with virilization and elevated serum alpha-fetoprotein (AFP) levels was found to have a Sertoli-Leydig cell tumor of the ovary. Immunoperoxidase studies using anti-AFP localized AFP in cells with the histologic appearance of Leydig cells. Serum AFP fell to undetectable levels after excision of the tumor. The patient is alive and well 1 year postexcision.

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80 楼    发表于2010-12-13 10:41:00举报|引用
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 难,期待最终结果!
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