衡道病理特邀撰稿作者翻译了WHO部分图⽚的图注，并用手帐的⽅式进行中英对照，且通过不同颜色的划线将晦涩难懂的英文单词与中⽂翻译同时标注，希望对专业英文的学习有所帮助。上一期分享了神经外胚层型肿瘤、单胚层囊性畸胎瘤及由畸胎瘤引起的躯体肿瘤，本期将带来性腺母细胞瘤。由于本篇目的以英文学习为主，篇幅有限，故未对各个疾病进行详细阐述。全部图片均来自WHO，若有不恰当之处，还请评论区指正。

WHO图注英文学习手帐

Gonadoblastoma

性腺母细胞瘤

Definition

Gonadoblastoma is a distinctive form of in situ germ cell neoplasia consisting of germ cells, at least some of which are similar to those of germ cell neoplasia in situ (GCNIS) of the testis, arranged in nests with incompletely differentiated sex cord cells.

定义

性腺母细胞瘤是一种由生殖细胞组成的独特的原位生殖细胞瘤，其中至少有一些与睾丸原位生殖细胞瘤(GCNIS)相似，排列呈巢状，内有不完全分化的性索细胞。

ICD-O coding

9073/1 Gonadoblastoma

ICD-O编码

9073/1性腺母细胞瘤

Histopathology

Multiple variably sized round nests are distributed in a fibrous to cellular gonadal stroma. The nests contain three components: germ cells, small sex cord cells, and globular basement membrane deposits. Some germ cells are identical to those of dysgerminoma/seminoma/GCNIS,but others may resemble spermatogonia.

组织病理学

多个大小不一的圆形巢分布在纤维型到细胞性的性腺基质中。巢由三部分组成:生殖细胞、小的性索细胞和球状基底膜样沉积物。一些生殖细胞与无性生殖细胞瘤/精原细胞瘤/GCNIS瘤细胞相同，但其他可能类似于精原细胞。

The sex cord cells may surround the germ cells or the basement membrane deposits or form a palisade at the periphery of nests. Leydig-like cells may be present in the surrounding stroma, and laminatedcalcifications are frequent on the basement membrane deposits, sometimes the only residual of a burnt-out gonadoblastoma. Occasional cases show large, anastomosing nests resembling germinoma. Testis or streak gonad may be found at the periphery.

性索细胞可包围生殖细胞或基底膜样物，或在巢周形成栅栏状结构。间质周围可见间质样细胞，基底膜沉积物常出现层状钙化，有时是退变的性腺母细胞瘤的唯一残余。偶有类似生殖细胞瘤的较大的吻合巢。周围可见睾丸或条纹性腺。

In two thirds of cases, the surrounding areas have a corded pattern of the same component cells. These have been termed both "dissecting gonadoblastoma" and "undifferentiated gonadal tissue". In the single largest study, about half of the cases showed invasive germinoma (dysgerminoma/seminoma), with other invasive germ cell tumours in 10%. These areas show the characteristic morphology of those tumours.

在三分之二的病例中，周围区域可见相同成分的细胞组成的条索状结构。

这些被称为“分割性性腺母细胞瘤”和“未分化性腺组织”。在一项最大的研究中，大约一半的病例表现为侵袭性生殖细胞瘤(无性细胞瘤/精原细胞瘤)，其他侵袭性生殖细胞瘤占10%。这些区域显示肿瘤的特征形态。

Immunohistochemical staining is positive in a subset of the germ cells for markers characteristic of germinoma/dysgerminoma/GCNIS (OCT3/4, podoplanin, PLAP). The sex cord cells are positive for inhibin, calretinin, SF1, FOXL2, and (weakly) SOX9.

免疫组化染色在生殖细胞亚型中，表达生殖细胞瘤/无性细胞瘤/GCNIS特征标记物(OCT3/4, podoplanin, PLAP)。性索细胞inhibin、calretinin、SF1、FOXL2和SOX9(弱阳)呈阳性。

Essential and desirable diagnostic criteria

Essential (in patients with disorders of sex development) arrangement in rounded nests; nests contain heterogeneous germ cells (some resembling germinoma, dysgerminoma or GCNIS), small sex cord cells, round deposits of basement membrane, and frequent calcifications; many germ cells express OCT3/4, PLAP, and podoplanin; sex cord cell express inhibin, calretinin, SF1, and FOXL2.

必要和理想的诊断标准:

必要的(对于性发育障碍患者)：排列在圆形巢中，巢中含有不同种类的生殖细胞(一些类似于生殖细胞瘤、 无性细胞瘤或GCNIS)、小的性索细胞、圆形基底膜和常见钙化;许多生殖细胞表达OCT3/4、PLAP和podoplanin; 性索细胞表达inhibin、calretinin、SF1和FOXL2。

Fig. Gonadoblastoma.

图：性腺母细胞瘤。

A Gonadoblastoma with germinoma. Gonadoblastoma shows a variegated, pink to tan to white granular cut surface (top) and is associated with a solid white area (bottom) corresponding to a germinoma.

伴生殖细胞瘤的性母细胞瘤, 性腺母细胞瘤表现为多种颜色，切面呈粉红色到棕褐色到白色(上)，并伴有与生殖细胞瘤相对应的实性白色区域(下)。

B Classic pattern of gonadoblastoma with discrete nests consisting of small, dark, sex cord cells; germ cells with clear cytoplasm; and round deposits of basement membrane material. One nest (bottom right) contains a calcification.

B经典的性腺母细胞瘤，由小的、深色的性索细胞的不连续的巢组成;有细胞质透明的生殖细胞和圆形的基底膜样物沉积。其中一个巢(右下)包含钙化。

C The sex cord cells form a partial palisade at the periphery of a nest and are also arranged around basement membrane deposits and germ cells.

性索细胞在巢的外围局灶呈栅栏状，也排列在基底膜样物和生殖细胞周围。

Fig. Gonadoblastoma.

A Burnt-out gonadoblastoma consisting of islands of irregular and mulberry-shaped calcifications devoid of tumour cells in a cellular stroma.

A 一种退变的性腺母细胞瘤，由不规则的细胞岛和桑葚样钙化组成，间质未见明显的肿瘤细胞。

B Large confluent nests of gonadoblastoma closely resemble germinoma at low power. Classic gonadoblastoma nests are seen at the top-right corner.

B性腺母细胞瘤的大融合巢在低倍镜下与生殖细胞瘤非常相似。右上方可见经典的性腺母细胞瘤巢。

C Immunostaining for SF1 highlights the consistent sex cord component in the tumour shown in Panel B.

C SF1标记图B性索细胞。

D Cords and small nests (called dissecting gonadoblastoma or, alternatively, undifferentiated gonadal tissue) resemble a common pattern of germinoma.

D条索和小巢(称为分割性性腺母细胞瘤或未分化性腺组织)类似于生殖细胞瘤的常见模式。

Fig. A Gonadoblastoma and germinoma. Gonadoblastoma with admixed germinoma.

A性腺母细胞瘤和生殖细胞瘤。性腺母细胞瘤混合生殖细胞瘤。

B Gonadoblastoma and germinoma. Inhibinstaining highlights the sex cord Component in the nests of gonadoblastoma and the lack thereof in the germinoma.

B性腺母细胞瘤和生殖细胞瘤。Inhibin染色在性腺母细胞瘤巢中的性索成分中阳性，而在生殖细胞瘤中缺乏。

C Gonadoblastoma. The germ cells of gonadoblastoma are variably positive for OCT3/4.

C性腺母细胞瘤的生殖细胞OCT3/4呈不同程度的阳性。

D Gonadoblastoma. An inhibin immunostaining highlights the sex cord cells within small nests and ill-defined cords of undifferentiated gonadal tissue or dissecting gonadoblastoma.

D 性腺母细胞瘤。inhibin免疫染色在未分化的性腺组织或分割性性腺母细胞瘤中的小巢和界限模糊的条索样结构内显示性索细胞表达。

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