Sex cord

  • 文章类型: Journal Article
    背景:卵巢纤维瘤是良性肿瘤,可能存在特殊的形态学特征,但未得到充分研究。
    方法:在这项回顾性研究中,连续75例卵巢纤维瘤与46例卵巢纤维瘤进行形态学比较,16个颗粒细胞瘤,和5硬化性间质瘤为以下因素:生长模式为弥漫性或结节性,透明斑块的存在,坏死,瘢痕疙瘩样硬化,钙化,囊性变性,纤维状或水肿间质,突出的血管,叶黄素细胞,cellularity,很少或丰富的细胞质,突出的细胞膜,核沟,非典型,和有丝分裂活性。
    结果:肿瘤在透明斑块方面存在显著差异,核沟,增长模式,基质类型,肿瘤细胞数量,细胞质,细胞膜的突出,非典型,有丝分裂活性,和突出的血管。
    结论:卵巢纤维瘤可以表现出一些意想不到的特征,如囊性变性,透明斑块,突出的血管,细胞增加,和一些有丝分裂活性,因此,他们的存在不应该总是提示另一种诊断。
    BACKGROUND: Ovarian fibromas are benign tumors that can present peculiar morphological features not studied sufficiently.
    METHODS: In this retrospective study, 75 consecutive cases of ovarian fibroma were morphologically compared with 46 thecomas, 16 granulosa cell tumors, and 5 sclerosing stroma tumors for the following factors: the growth pattern as diffuse or nodular, the presence of hyaline plaques, necrosis, keloid-like sclerosis, calcifications, cystic degeneration, fibrous or edematous stroma, prominent vascularity, lutein cells, cellularity, scant or abundant cytoplasm, prominent cell membranes, nuclear grooves, atypia, and mitotic activity.
    RESULTS: The tumors differed significantly in terms of hyaline plaques presence, nuclear grooves, growth pattern, stroma type, tumor cellularity, cytoplasm, prominence of cell membranes, atypia, mitotic activity, and prominent vascularity.
    CONCLUSIONS: Ovarian fibromas can present some maybe unexpected features rather frequently, such as cystic degeneration, hyaline plaques, prominent vascularity, increased cellularity, and some mitotic activity, thus their presence should not always prompt to an alternative diagnosis.
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  • 文章类型: Journal Article
    性索样子宫内膜样癌(SCLEC)是一种罕见的实体,可能构成诊断挑战。这项研究旨在进行临床病理,免疫组织化学,和卵巢SCLEC的分子重新评估。在13年期间,对来自单个机构的连续卵巢SCLEC病例进行了审查。测试了23个免疫组织化学标记物;通过下一代测序分析了10个基因。确定9例卵巢SCLEC。患者平均年龄为65.7岁;3例显示卵巢外延伸。建筑模式包括sertoliform(n=2),颗粒状(n=2),和混合颗粒样/血清型(n=5)。在四个肿瘤中观察到嗜酸性粒细胞变化并伴有核异型性增加。子宫内膜样特征(腺体,在6例中观察到鳞状/角膜分化)。大多数肿瘤细胞角蛋白-7阳性(8/9),EMA(9/9),雌激素和孕激素受体(9/9),CD10(7/9,包括令人联想到中肾肿瘤的管腔模式),核β-连环蛋白(8/9),和CDX2(8/9)。少数病例表现为块型p16模式(2/9),PAX8阳性(3/9),WT1(1/9)的非扩散阳性,抑制素(1/9),嗜铬粒蛋白(1/9),和突触素(2/9)。所有病例均为GATA3、TTF1、calretinin阴性,SF1。Ki67范围为15-90%。6例CTNNB13号外显子突变。8例“无特异性分子谱”(NSMP),1例p53异常。总之,SCLEC经常表现出混合的血清型/颗粒样结构,并表达上皮标志物。激素受体,核β-连环蛋白,和CDX2,具有管腔CD10阳性和CTNNB1突变。PAX8表达经常丢失,而其他中肾,性索,神经内分泌标志物呈阴性。
    Sex cord-like endometrioid carcinoma (SCLEC) is an uncommon entity which may constitute a diagnostic challenge. This study aimed to perform a clinicopathological, immunohistochemical, and molecular reappraisal of ovarian SCLEC. Consecutive ovarian SCLECs cases from a single institution were reviewed during a 13-year period. Twenty-three immunohistochemical markers were tested; 10 genes were analyzed by next-generation sequencing. Nine cases of ovarian SCLEC were identified. Mean patient age was 65.7 years; three cases showed extraovarian extension. Architectural pattern included sertoliform (n = 2), granulosa-like (n = 2), and mixed granulosa-like/sertoliform (n = 5). Eosinophilic changes accompanied by increased nuclear atypia were observed in four tumors. Endometrioid features (glands, squamous/morular differentiation) were observed in six cases. Most tumors were positive for cytokeratin-7 (8/9), EMA (9/9), estrogen and progesterone receptor (9/9), CD10 (7/9, including a luminal pattern reminiscent of mesonephric neoplasms), nuclear β-catenin (8/9), and CDX2 (8/9). A minority of cases showed block-type p16 pattern (2/9), PAX8-positivity (3/9), and non-diffuse positivity for WT1 (1/9), inhibin (1/9), chromogranin (1/9), and synaptophysin (2/9). All cases were negative for GATA3, TTF1, calretinin, and SF1. Ki67 range was 15-90%. Six cases showed CTNNB1 exon 3 mutation. Eight cases were of \"no specific molecular profile\" (NSMP) and one was p53-abnormal. In conclusion, SCLECs frequently exhibit a mixed sertoliform/granulosa-like architecture and express epithelial markers, hormone receptors, nuclear β-catenin, and CDX2, with luminal CD10 positivity and CTNNB1 mutations. PAX8 expression is often lost, while other mesonephric, sex cord, and neuroendocrine markers are negative.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    在这里,我们报告了一例具有血清型的输卵管卵巢高级别浆液性癌(HGSC)的临床病理和分子分析。一名45岁的妇女因双侧附件癌晚期并伴有腹膜和阑尾转移而接受了手术。组织学检查显示HGSC表现出明显的血清型成分。这种成分表现为弥漫性PAX8,p53(突变型),和p16(块型)表达式,与常规HGSC成分相比,波形蛋白增加和WT1表达减少,膜β-连环蛋白阳性,异质雌激素,和孕酮阳性,并保留了PTEN和错配修复的表达和对GATA3,TTF1,抑制素,calretinin,CD10,CDX2,嗜铬粒蛋白,和突触素。分子分析显示种系BRCA2突变;在POLE中未检测到突变,POLD1、MLH1、MSH2、MSH6、PMS2、APC、CTNNB1,MUTYH,和EPCAM。总之,血清型模式可以是BRCA相关HGSC的形态谱的一部分。
    Herein, we report a clinicopathological and molecular analysis of a case of tubo-ovarian high-grade serous carcinoma (HGSC) with a sertoliform pattern. A 45-year-old woman underwent surgery due to an advanced bilateral adnexal carcinoma with peritoneal and appendiceal metastases. Histological examination revealed an HGSC exhibiting a distinct sertoliform component. Such component showed diffuse PAX8, p53 (mutation-type), and p16 (block-type) expression, increased vimentin and decreased WT1 expression compared to the conventional HGSC component, membrane β-catenin positivity, heterogeneous estrogen, and progesterone positivity, and retained PTEN and mismatch repair expression and negativity for GATA3, TTF1, inhibin, calretinin, CD10, CDX2, chromogranin, and synaptophysin. Molecular analysis showed a germline BRCA2 mutation; no mutations were detected in POLE, POLD1, MLH1, MSH2, MSH6, PMS2, APC, CTNNB1, MUTYH, and EPCAM. In conclusion, a sertoliform pattern can be part of the morphological spectrum of BRCA-related HGSC.
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  • 文章类型: Journal Article
    儿童卵巢肿瘤并不常见。就像成年人中出现的那些,它们可以大致分为生殖细胞,性索,和表面上皮亚型;然而,生殖细胞肿瘤占儿童病变的大部分,而表面上皮起源的肿瘤在成人中占主导地位。诊断检查,包括成像的使用,需要一种通常不同于成年人所需的方法。本文为诊断和随访期间已知或怀疑患有原发性卵巢恶性肿瘤的儿科患者的影像学检查提供了共识建议。
    Ovarian tumors in children are uncommon. Like those arising in the adult population, they may be broadly divided into germ cell, sex cord, and surface epithelium subtypes; however, germ cell tumors comprise the majority of lesions in children, whereas tumors of surface epithelial origin predominate in adults. Diagnostic workup, including the use of imaging, requires an approach that often differs from that required in an adult. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary ovarian malignancy at diagnosis and during follow-up.
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  • 文章类型: Case Reports
    Endometrial stromal sarcoma with sex cord differentiation is a very unusual neoplasm affecting the uterine cavity with an incidence of only 0.25%. They can cause difficulties in diagnosis due to it is histologic appearance and rarity of occurrence. Histopathological diagnosis still remains the cornerstone of diagnosis. Immunohistochemistry and molecular studies are also very helpful. We report a case of a 47-year-old patient who was diagnosed clinically and radiologically as intramural leiomyoma, but on histopathology examination and immunohistochemically proved to be endometrial stromal sarcoma with sex cord differentiation.
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  • 文章类型: Journal Article
    BACKGROUND: Sex cord-like elements are rarely observed in uterine lesions, but these morphological patterns could appear in a variety of uterine tumors and non-tumorous lesions. In this review, we collected the literatures regarding the uterine tumorous and non-tumorous lesions containing sex cord-like elements and summarized these lesions in terms of clinicopathological, immunohistochemical, and molecular features in order to further understand these lesions and provide some new ideas for differential diagnosis.
    METHODS: This section provides a comprehensive overview of the clinicopathological, immunohistochemical, and molecular features of uterine lesions with sex cord-like architectures including uterine tumors resembling ovarian sex cord tumors, endometrial stromal tumors, adenomyosis, endometrial polyps, leiomyoma, epithelioid leiomyosarcoma, adenosarcoma, sertoliform endometrioid carcinoma, corded and hyalinized endometrioid carcinoma, mesonephric adenocarcinoma, and mesonephric-like adenocarcinoma. The differential diagnosis based on morphology, immunohistochemistry, and molecular alterations has also been discussed.
    CONCLUSIONS: The sex cord-like areas in these lesions show heterogeneous but similar morphological features. Additionally, immunohistochemical staining plays a limited role in differential diagnosis. Furthermore, it is of significance for pathologists to better understand these lesions in order to avoid confusion and mistakes during pathological diagnosis, especially in a biopsy/curettage specimen.
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  • 文章类型: Journal Article
    这项研究的目的是对类似于卵巢性索间质肿瘤(UTROSCT)的子宫肿瘤和其他具有性索样(SCL)分化的子宫病变进行免疫组织化学比较。检查了6种UTROSCT和10种具有局灶性SCL元素的潜在组织学模拟物,后者包括三个子宫内膜间质结节,三种低级别子宫内膜间质肉瘤,三个苗勒氏腺肉瘤,1例子宫腺肌病。所有病例均行免疫组织化学SF1、FOXL2、钙视网膜素和抑制素染色,对于特异性较低的平滑肌肌动蛋白标记,desmin,CD10,CD56,CD99,细胞角蛋白,雌激素受体和孕激素受体。三,四,六个和三个UTROSCT表达SF1,FOXL2,钙视网膜素和抑制素,分别。然而,在5例中,钙视网膜素染色是局灶性的(≤50%细胞阳性)。三种潜在的组织学模拟物证明了钙视网膜素,FOXL2和/或抑制素染色,但无SF1阳性。两组中的大多数病例都表达了较低的特异性免疫标记。UTROSCT中的SF1和FOXL2免疫反应性进一步支持了这些肿瘤表现出真正的性索-基质分化的概念。虽然钙调蛋白是最敏感的UTROSCT标记,染色通常是局灶性的,并且在10种潜在的组织学模拟物中的两种中也观察到表达。在该系列中,SF1染色对于UTROSCT是100%特异性的,但是这一发现应该在更大的研究中得到证实。
    The aim of this study was to perform an immunohistochemical comparison of uterine tumour resembling ovarian sex cord-stromal tumour (UTROSCT) and other uterine lesions with sex cord-like (SCL) differentiation. Six UTROSCTs and 10 potential histological mimics with focal SCL elements were examined, the latter comprising three endometrial stromal nodules, three low-grade endometrial stromal sarcomas, three Müllerian adenosarcomas, and one case of adenomyosis. All cases were stained immunohistochemically for SF1, FOXL2, calretinin and inhibin, and for the less specific markers smooth muscle actin, desmin, CD10, CD56, CD99, cytokeratin, oestrogen receptor and progesterone receptor. Three, four, six and three UTROSCT expressed SF1, FOXL2, calretinin and inhibin, respectively. However, calretinin staining was focal (≤50% cells positive) in five of the cases. Three potential histological mimics demonstrated calretinin, FOXL2 and/or inhibin staining but none was SF1 positive. Most cases in both groups expressed the less specific immunomarkers. SF1 and FOXL2 immunoreactivity in UTROSCT further supports the concept that these tumours demonstrate genuine sex cord-stromal differentiation. While calretinin was the most sensitive UTROSCT marker, staining was usually focal and expression was also seen in two of 10 potential histological mimics. SF1 staining was 100% specific for UTROSCT in this series but this finding should be confirmed in larger studies.
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  • 文章类型: Journal Article
    目的:卵巢性索基质肿瘤(SCSTs)包括几种组织学肿瘤亚型,这些亚型由特征性组织学特征定义。有些可以显示与SCST的其他亚型的形态重叠,以及非SCST。E-cadherin/catenin复合物构成粘附连接,在上皮组织中发育良好,但组成分子也在一些非上皮肿瘤中表达。这项研究的目的是确定卵巢SCST中E-钙黏着蛋白和连环蛋白的表达模式是否具有诊断价值。
    结果:我们研究了E-cadherin的表达,α-,免疫组织化学在55例肿瘤中的β-和γ-连环蛋白。我们发现所有肿瘤亚型均显示E-cadherin的核表达,虽然只有微囊性间质瘤(MCST)表现出明显的特征,在几乎所有情况下,所有三个连环蛋白的核定位。
    结论:我们得出结论,SCST中的E-cadherin表达谱可以帮助区分SCST和非SCST,其中E-cadherin没有核表达。连环蛋白的核定位可能在区分MCST与SCST的其他亚型中具有潜在的用途。
    OBJECTIVE: Sex cord stromal tumours (SCSTs) of the ovary encompass several histological tumour subtypes that are defined by characteristic histological features. Some can show morphological overlap with other subtypes of SCSTs, as well as with non-SCSTs. The E-cadherin/catenin complex constitutes the adherens junction, which is well developed in epithelial tissue, but the constituent molecules are also expressed in several non-epithelial tumours. The aim of this study was to determine whether the expression patterns of E-cadherin and catenins in ovarian SCSTs can be of diagnostic utility.
    RESULTS: We studied the expression of E-cadherin, α-, β- and γ-catenin in 55 tumours using immunohistochemistry. We found that all tumour subtypes showed nuclear expression of E-cadherin, while only microcystic stromal tumours (MCSTs) displayed a distinct profile, with nuclear localization of all three catenins in almost all cases.
    CONCLUSIONS: We conclude that the E-cadherin expression profile in SCSTs can assist in distinguishing between SCSTs and non-SCSTs in which there is no nuclear expression of E-cadherin. The nuclear localization of catenins may be of potential use in distinguishing MCST from other subtypes of SCST.
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  • 文章类型: Case Reports
    Steroid cell tumors (SCTs) of the ovary are a rare subgroup of sex cord tumors that account for less than 0.1% of all ovarian tumors. These tumors can produce steroids, especially testosterone, which produces symptoms such as hirsutism, amenorrhea/oligomenorrhea, and male patterned voice. For evaluation of the androgen excess, testosterone and dehydroepiandrosterone sulfate (DHEA-S) are the first laboratory tests to be measured. Abdominal ultrasound and magnetic resonance imaging (MRI) are useful radiologic imaging techniques. Although SCTs are generally benign, the risk of malignant transformation is always present. Surgical excision of tumor is the most important and hallmark treatment. The present case signifies the early preoperative diagnosis of a virilizing SCT, based on cytological features and its careful correlation with clinicopathological and radiological findings.
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