【衡道丨干货】WHO图注英文学习手帐（卵巢网囊腺瘤、腺瘤和腺癌及卵巢中肾管肿瘤）

Rete cystadenoma, adenoma, and adenocarcinoma

卵巢网囊腺瘤、腺瘤和腺癌

Definition

Rete cystadenoma, adenoma, and adenocarcinoma are benign and malignant tumours derived from the rete ovarii that mirror their testicular counterparts.

定义

卵巢网囊腺瘤、腺瘤和腺癌是源自卵巢网的良性和恶性肿瘤，与睾丸网相似。

ICD-O coding

9110/0 Adenoma of rete ovarii

9110/3 Adenocarcinoma of rete ovarii

ICD-O编码

9110/0卵巢网腺瘤

9110/3卵巢网腺癌

Histopathology

组织病理学

Rete cysts are characterized by irregularly spaced crevices along their inner lining, which displays flat to cuboidal cells with scant eosinophilic to clear (uncommon) cytoplasm and minimal nuclear stratification.Cytological atypia and mitoses are rare or absent.

卵巢网囊肿的特征是其内层有不规则间隔分隔，细胞呈扁平或立方状，少量胞质呈嗜酸性或透明(不常见)，轻度核复层。细胞学异型性和核分裂象罕见或缺乏。

Hilus cells may form a bandperipheral to the cyst wall. Rete ovarii can also be seen in the vicinity .Rete adenomas are centred in the ovarian hilus;they are well circumscribed and composed of closely packed small elongated to round and dilate tubules, some with focal, variably complex intraluminal papillas. The lining cells are cuboidal to slightly columnar, with scant eosinophilic to pale cytoplasm, typically lacking cytological atypia and mitotic activity.  

卵巢门细胞可在囊肿周围形成一条带，附近也可见卵巢网。卵巢网腺瘤以卵巢门为中心;边界清楚，由紧密排列的细长至圆形膨大的小管组成，部分腺瘤局灶有复杂的管腔内乳头，内衬细胞呈立方状至略呈柱状，少量胞质呈嗜酸性至淡染，缺乏细胞异型性和核分裂。

They may contain Leydig/luteinized cells within the scant intervening stroma .The reported rete carcinoma was characterized by branching tubules, cysts, papillae, and solid growth with cytological atypia and mitotic activity.

在稀少的间质中可能含有间质/黄素化细胞。报道的卵巢网癌的特征是分支小管、囊肿、乳头和实性生长，具有细胞异型性和核分裂活性。

Essential and desirable diagnostic criteria

Essential：rete cystadenoma： hilar location, crevices in inner lining and no cilia, with or without Leydig cells within wall;

rete adenoma：hilar location,circumscription, small compact tubules,and no cytological atypia;

rete carcinoma：hilar location, cytological atypia, and mitoses- this is a diagnosis of exclusion.

必要和理想的诊断标准：

必要的：卵巢网囊腺瘤：位于卵巢门部，内层有裂隙，无纤毛，伴或不伴有管壁内Leydig细胞;

卵巢网腺瘤：位于卵巢门部，边界清楚，小管致密，无细胞异型性;

卵巢网癌：位于卵巢门部，有细胞学异型性和核分裂-这是一种排除性诊断。

Rete cyst.

卵巢网囊肿

The inner wall shows spaced crevices lined by innocuous cuboidal cells lacking cilia.  Note the presence of a band of Leydig cells within the cyst wall.

卵巢网内壁可见间隔分隔，排列着没有纤毛的温和立方细胞。囊内壁可见一条Leydig细胞带。

Wolffian tumour of the ovary

卵巢Wolffian肿瘤

Definition

Wolffian tumour is a rare but distinctive epithelial tumour of Wolffian (mesonephric) origin.

定义

Wolffian肿瘤是一种起源于中肾管的罕见但独特的上皮肿瘤。

ICD-O coding

9110/1 Wolffian tumour

ICD-O编码

9110/1 Wolffian肿瘤

Histopathology

组织病理学

Microscopic examination typically reveals a well-circumscribed lesion composed of varying proportions of four distinct patterns：

显微镜检查通常显示一个界限清楚的病变，由不同比例的四种不同的模式组成：

(1)a diffuse or solid pattern characterized by a spindle cell population;

(1)以梭形细胞为特征的弥漫性或实性模式;

(2) a tubular pattern showing tubular structures of various sizes and shapes, some of which are lined by columnar cells with basally located nuclei, and compressed tubules with a slit like lumen lined by small cuboidal cells;

(2)表现为大小和形状各异的管状结构，其中一些衬附着细胞核位于基底部的柱状细胞，以及由小立方体细胞衬附的具有狭缝状管腔的压缩小管;

(3) a retiform (sieve-like) pattern characterized by a network of elongated and branching tubules occasionally in a sieve-like arrangement;

(3)网状(筛状)模式，其特征是由细长和分支的小管形成网状结构，偶尔呈筛状排列;

and (4) a multicystic pattern with variable cystic spaces lined by a single flattened layer of cuboidal cells.

(4)多囊模式，囊腔由单层扁平的立方体细胞排列。

The nuclei are bland, without nucleoli, and the mitotic count is low (<1 mitosis/mm², equating to < 3 mitoses/10 HPF of 0.55 mm in diameter and 0.24 mm² in area).

细胞温和，无核仁，核分裂计数低(<1个核分裂/mm²，相当于< 3个核分裂/10 HPF，直径0.55 mm，面积0.24 mm²)。

Occasionally, as many as 3-4 mitoses/mm², equating to 7-9 mitoses/10 HPF of 0.55 mm in diameter and 0.24 mm² in area, can be found.

有时，可发现多达3-4个有丝分裂/mm²，相当于7-9个核分裂/10 HPF，直径0.55 mm，面积0.24 mm²。

A colloid-like, PAS-positive secretion may be seen within the lumina of tubules and cystic spaces. The stroma varies from a delicate network of reticulin fibres, separating the solid tubules and unmasking a tubular pattern in what appears to be a solid proliferation, to large areas of hyalinized collagen, sometimes with calcifications.

小管腔内和囊腔内可见胶质样PAS阳性分泌物。间质由纤细的网状纤维组成的纤维网，分隔增生的小管或间质增生的管状模式，到大面积透明胶原化，有时伴钙化。

Pankeratin (AE1/AE3) and vimentin are diffusely positive,whereas CK7 usually shows focal staining. EMA, ER, and PR are negative or only focally positive, whereas AR is more diffusely expressed.

pankeratin (AE1/AE3)和vimentin弥漫阳性，而CK7通常表现为局灶性染色。EMA、ER和PR呈阴性或仅局部阳性，而AR呈弥漫性阳性表达。

Calretinin, a-inhibin, FOXL2, and WT1 are usually positive, albeit focally. CD10 shows a luminal pattern of staining . GATA3 has been shown to be expressed in mesonephric remnants of the cervix and the fallopian tube and in cervical mesonephric carcinomas, whereas in adnexal Wolffian tumours it is usually negative or shows weak multifocal staining (in 17% of cases) . PAX8 and SF1 are usually not expressed.

Calretinin, a-inhibin, FOXL2和WT1通常呈阳性，尽管是局部阳性。CD10呈腔缘阳性。GATA3已被证明在子宫颈和输卵管的中肾管残余以及子宫颈中肾癌中表达，而在附件Wolffian肿瘤中，它通常为阴性或显示多灶弱阳(17%的病例)。PAX8和SF1通常不表达。

Adnexal Wolffian tumour should be distinguished from ovarian mesonephric-like adenocarcinoma. Features that may be helpful in the diagnosis of mesonephric-like carcinoma include atypia, a high mitotic count, GATA3 and TTF1 positivity, and molecular studies showing KRAS/NRAS mutation.

附件Wolffian肿瘤应与卵巢中肾管样癌鉴别。可能有助于诊断中肾管样癌的特征包括异型性、高核分裂计数、GATA3和TTF1阳性以及分子研究显示KRAS/NRAS突变。

Endometrioid carcinoma should also be distinguished from Wolffian tumour.EMA, ER, PR, and PAX8 positivity, as well as the presence of squamous, mucinous, or ciliated metaplasia, points towards as diagnosis of Müllerian-derived carcinoma.Sex cord tumours such as Sertoli-Leydig cell tumour and Sertoli cell tumour must be distinguished from Wolffian tumour because they express α-inhibin, calretinin, FOXL2, and WT1.

However, the absence of SF1 positivity and the absence of a Leydig cell component, as well as the lack of somatic DICER1 mutation, are characteristio of Wolffian tumours.

子宫内膜样癌也应与Wolffian肿瘤鉴别。EMA, ER, PR和PAX8阳性以及鳞化、黏液性或纤毛化生的存在，都支持Müllerian衍生的癌。性索肿瘤如Sertoli- leydig细胞瘤和Sertoli细胞瘤必须与中肾管肿瘤区分开来，因为它们表达α-inhibin、calretinin、FOXL2和WT1。

然而，缺乏SF1阳性和缺乏Leydig细胞成分，以及缺乏体细胞DICER1突变是中肾管肿瘤的特征。

Essential and desirable diagnostic criteria

必要和理想的诊断标准：

Essential：a well-circumscribed lesion with varying proportions of four distinct patterns (spindle cells in diffuse/solid, tubular retiform, multicystic); bland cell nuclei without nucleoli and usually a low mitotic count; colloid-like, PAS-positive secretions within lumina of tubules and cystic spaces; stroma varying from a delicate network of reticulin fibres to large areas of hyalinized collagen.

必要的：病灶边界清楚，有四种不同比例的不同模式(弥漫性/散在梭形细胞、管状网状细胞、多囊细胞);无核仁的温和细胞核，通常核分裂计数低;小管腔内和囊腔内有胶质样pas阳性分泌物;间质数量不等的网状纤维到透明胶原成分。

Desirable： immune marker expression as described above.

理想的：如上所述的免疫标记表达。

Fig. Wolffian tumour of the ovary.

卵巢Wolffian肿瘤

A Note the mixture of solid, retiform, and sieve-like patterns.

A 注意实性、网状和筛状结构成分的混合。

B Note the retiform pattern showing elongated and branching tubules with morphological similarity to the rete ovarii.

B注意网状结构，显示细长分支的小管，与卵巢网形态相似。

C Note the well-differentiated tubules lined by columnar cells without nuclear atypia at higher magnification.

C高倍镜下，可见分化良好的小管衬附柱状细胞，细胞核无异型性。

E Note the well-circumscribed nodule within theovarian hilum.

E注意卵巢门内界限清楚的结节。

F Note the solid pattern composed of spindle cells without nuclear atypia.

F 可见梭形细胞组成的实性结构，无核异型性。