卵巢幼年型颗粒细胞瘤一例并文献复习

<正>卵巢颗粒细胞瘤(ovarian granulosa cell tumor,OGCT)是一种具有颗粒细胞形态特征和内分泌功能的卵巢性索间质肿瘤^[1]。按临床和病理特征,可分为成人型颗粒细胞瘤(adult granulosa cell tumor,AGCT)和幼年型颗粒细胞瘤(juvenile granulosa cell tumor,JGCT),其中JGCT仅占OGCT的5%,是一种罕见且恶性程度极高的肿瘤,常见于年轻女性、临床症状不典型,患者常因体检时发现腹部包块或因包块压迫引起     

幼年型卵巢颗粒细胞瘤伴破裂出血1例及文献回顾

卵巢颗粒细胞瘤(ovarian granulosa cell tumor,OGCT)是具有粒层细胞形态特征的性索间质肿瘤,仅占卵巢恶性肿瘤的1％ ～2％.分为成人型颗粒细胞瘤(adult granulosa cell tumor,AGCT)和幼年型颗粒细胞瘤(juvenile granulosa cell tumor,...     

Ovarian juvenile granulosa cell tumor associated with Maffucci’s syndrome: case report

Granulosa cell tumors (GCTs) are the most common ovarian sex-cord stromal tumors. Two histopathologically well defined patterns of GCTs are known : adult granulos acell tumor (AGCT) and juvenile granulosa cell tumor(JGCT). JGCTs are rare and those associated with enchondromatosis are much rarer. A review of the literature revealed four previous cases of JGCT were associated with Maffucci‘s syndrome (MS), and nine with Ollier‘s disease (OD). This report describes ovarian JGCT with MS in a 21-year-old woman, and reviews the clinicopathological causes of both disorders.     

以腹水急剧增加为唯一特征的幼年型卵巢颗粒细胞瘤1例并文献复习

目的 初步探讨幼年型卵巢颗粒细胞瘤(juvenile granulosa cell tumor, JGCT)临床诊断及治疗方法。方法 回顾性分析皖南医学院附属弋矶山医院妇科1例JGCT伴雌激素正常、大量腹水迅速形成的罕见病例,并总结JGCT的常见临床表现及治疗方法。结果 该病例手术顺利,术后病理证实为幼年型颗粒细胞瘤,现已完成术后辅助化疗,目前密切随访未见明显复发。结论 对于有不明原因的大量腹水迅速形成为首发症状的年轻女性,尽管雌激素正常,仍要警惕存在JGCT的可能,结合影像学资料等明确诊断,并选择合适的手术范围,最后根据手术病理结果进一步指导后续治疗。     

8例儿童卵巢颗粒细胞瘤临床病理特征及预后分析

目的·探讨儿童卵巢颗粒细胞瘤（granulosa cell tumor,GCT）的临床病理特征和预后。方法·收集上海市儿童医院2008年6月至2018年6月共8例卵巢GCT患儿的临床病理资料和随访资料,总结其临床病理学特征并分析预后情况。结果·患儿发病年龄为2～12岁（中位年龄6.5岁）。2例为成人型颗粒细胞瘤（adult granulosa cell tumor,AGCT）,6例为幼年型颗粒细胞瘤（juvenile granulosa cell tumor,JGCT）。首发症状均表现为腹痛或腹部包块。6例患儿并发性早熟症状;其中5例为真性性早熟,1例为假性性早熟。外周血性激素水平均有不同程度的改变。肿瘤最大径4～22 cm （平均12.8 cm）,大体形态呈囊实性。AGCT在光学显微镜下可见明显核沟,并有微滤泡结构,内含嗜酸性物质,即特征性的Call-Exner小体;JGCT具有大小不等的滤泡结构,内含嗜碱性分泌物,滤泡壁内层由粒层细胞构成,周围可被卵泡膜细胞环绕,未见Call-Exner小体,核沟少见。免疫组织化学检测结果显示：所有患儿α抑制素（α-inhibin）和CD99均为阳...     

无低血钾肾球旁细胞瘤（1例报告及文献复习）

肾球旁细胞瘤(juxtaglomerular cell tumor of kidney,JGCT)是肾脏罕见的良性肿瘤,以严重的高血压和低血钾为特征[1].无低血钾肾球旁细胞瘤极为少见.     

Juxtaglomerular cell tumor of the kidney: a clinicopathological analysis of five cases

Juxtaglomerular cell tumor (JGCT) is a kind of rare renal neoplasm that was first described by Robertson in 1967.1 This tumor typically is found in young adults, and patients usually have hypertension, hyperaldosteronism and hypokalemia secondary to tumor renin secretion .1-8 To our knowledge, only about 70 cases have been reported including no more than 8 cases in China. Because of its rarity, the clinicopathological features of the tumor have not been clearly established. In this article, 5 cases of JGCT were reported, in order to give a better understanding of its morphological and immunohistochemical profiles. In addition, the immunophenotypical findings of JGCT were compared with 5 cutaneous glomus tumors and 5 hemangiopericytomas, which shared morphological features with JGCT.     

卵巢巨大幼年型颗粒细胞瘤并肝脏局灶性结节增生一例

<正>卵巢幼年型颗粒细胞瘤(juvenile granulosa cell tumor,JGCT)是一种少见的卵巢肿瘤,常合并生殖激素异常。杭州市萧山区第一人民医院收治卵巢巨大幼年型颗粒细胞瘤合并肝脏局灶性结节增生1例,现报道如下。1病例介绍患者,女,13岁,因发现盆腔包块2天入院。患者2天前无意中发现右下腹包块,伴腹部膨隆,遂至杭州市萧山区第一人民医院外科就诊,查体包块以站立时明显,平躺消失,考虑为腹股沟疝及腹腔包块,门诊进一步B超检查:站立时右侧腹股沟内见约     

肾球旁细胞瘤1例报告并文献复习

肾球旁细胞瘤(juytaglomerular cell tumor of the kidney,JGCT)是罕见的肾实质良性肿瘤,主要见于年轻人,其临床表现为严重的高血压、高血浆肾素、继发性醛固酮增多和低血钾.Robertson[1]1967年首次报告,我院2011年8月收治1例,现报告如下. 1病例报告 患者,男,35岁,以体检时发现右肾肿物20d为主诉入院.     

SCANNING ELECTRON MICROSCOPY OF MALIGNANT BRENNER TUMORS OF THE OVARY

Six cases of malignant Brenner tumors of the ovary were observed by lightmicroscopy (LM) and scanning electon microscopy (SEM). The histologic features of the tu-mors revealed nonkeratinized squamous cell carcinoma. The SEM showed the tumor nests werecomposed of squamous cells, accompanied with a few ciliated cells. These findings evide-nced that malignant Brenner tumor like the other epithelial tumors of ovary originated fromthe same Mullerian epithelium, but recapitulated the vagino cervical epithelium. The histoge-nesis of different types of ovarian epithelial tumors corresponded to the development oforgans of female genital tract differentiating from Mallerian epithelium in embryo. It dosenot conform to fact to think that there were present transitional cell carcinomas in ovarianepithelial tumors, or the malignant Brenner tumors were composed of transitional epithelium.     

Application of ovarian transposition during hysterectomy

Objective To study the optimal position and method for ovarian transposition and its benefits and indications.Methods We performed ovarian transposition in 34 patients from August 1989 to December 2000.Twelve patients were diagnosed with stage Ⅰ b to Ⅱ a cervical cancer, 4 had stage Ⅰ a endometrial carcinoma, 12 had stage Ⅲ to Ⅳ endometriosis, 4 had myoma of uterus, 1 had dysfunctional uterine bleeding, and 1 had an ovarian granulosa cell tumor. Surgery went as follows: the ovary was dissociated by clamp, the skin was incised and a tunnel was made, then the ovary was translocated to the subcutaneous site. In the cases of benign lesions, the ovarian vessel pedicel went in through the abdominal cavity, but in malignant tumors, it went out through the peritoneum.Results In both cases (benign lesions or malignant tumors), the short-term and long-term endocrine function of the translocated ovary remained normal. Furthermore, patients could supervise their translocated ovary themselves.Conclusions Subcutaneous ovary transposition might prevent not only implantation of gastrointestinal cancer but also the extension of pelvic carcinoma to the ovary. Because of the shallow transposition and the incision scar, it is easy for patients to supervise themselves. Moreover, the site of the ovary is easy to locate for ultrasound examinations. Thus, it can obtain the goal of early prevention for cancer.Subcutaneous ovarian transposition with skin incision is the optimal selection and suitable for all patients with various gynecologic diseases in which ovary removal is not necessary.     

PTEN和Ki-67在成年型卵巢颗粒细胞瘤中的表达及意义

目的探讨PTEN和Ki-67在成年型卵巢颗粒细胞瘤（adult-type granulosa cell tumor of the ovary,AGCTO）中的表达及临床意义。方法采用免疫组织化学法分别检测了47例成年型卵巢颗粒细胞瘤和20例正常卵巢组织中FrEN和Ki-67的表达,并对PTEN与Ki-67的关系进行分析。结果PTEN在AGCTO中的表达（63．8％）低于正常卵巢组织（90．0％,P〈0．05）,在临床分期Ⅲ-Ⅳ组明显低于临床Ⅰ、Ⅱ期（P〈0．05）,组织分化差的组明显低于分化较好的组（P〈0．05）。Ki-67在AGCTOⅢ-Ⅳ期的表达明显高于Ⅰ、Ⅱ期（P〈0．05）,低分化组明显高于高、中分化组（P〈0．05）。PTEN与Ki-67的表达呈负相关关系。PTEN、Ki-67的阳性表达率与AGCTO的临床分期和组织分化程度有关。结论PTEN表达下降可能在成年型颗粒细胞瘤的发生发展中起一定的作用,联合检测PTEN和Ki-67的表达可为诊断及判定预后提供理论依据。     

Expression of Leptin Long-form Receptor mRNA in Luteinized Granulosa Cells of Obese Women with Polycystic Ovary Syndrome

To investigate the expression of mRNA of leptin long-form receptor(OB-Rb) in lu-teinized granulosa cells of obese women with polycystic ovary syndrome(PCOS),and to determine the role of leptin in the physiopathology of PCOS,luteinized granulosa cells were collected from the follicle fluid of 10 obese women who met the diagnostic criteria for PCOS and their BMI was equal to or greater than 25 kg/m2,and at the same time,granulosa cells were collected from 10 normal women undergoing IVF-ET who served as the control group.Some luteinized granulosa cells were taken from normal women for in-vitro culture,into which human leptin of different concentrations was added(0,10,100 and 1000 ng/mL).After stimulation with leptin for 48 h,RT-PCR was em-ployed for the detection of the expression of OB-RLmRNA in the luteinized granulosa cells.Our re-sults showed that the level of OB-RLmRNA in luteinized granulosa cells of obese PCOS women was higher than those in the control(P<0.05).In luteinized granulosa cells cultured in vitro and stimulated by human leptin for 48 h,the level of OB-RLmRNA was higher than those without leptin stimulation(P<0.01),and when leptin concentration was at 100 ng/mL,and the level of OB-RLmRNA reached a peak.It is concluded that in obese PCOS women,the level of serum leptin is increased,which pro-motes the expression of OB-RL in luteinized granulosa cells and increases the sensitivity of the granulosa cells to leptin.Leptin may contribute to anovulation in obese women with PCOS     

Ovarian juvenile granulosa cell tumor in a 20-month-old infant

To editor： Ovarian juvenile granulosa cell tumors （JGCTs） occur in infants. Here we reported a JGCT in a 20-month- old female infant who presented with isosexual precocious pseudopuberty.     

Expression of the novel gene NM23-H1B in ovarian cancer

Objective：To study the expression of the human novel gene NM23-H1B in ovarian cancer.Methods: Totally 24 samples from patients with epithelial ovarian tumor at different clinical stages and 4 from normal ovaries were examined for NM23-H1B mRNA expression by RT-PCR and Northern blot. Results: All samples expressed NM23-H1B mRNA through RT-PCR, while the level of expression in ovarian tumor was higher than that of normal ovary. The results of Northern blot showed that NM23-H1B was overexpressed in ovarian cancer while lowexpressed in normal ovary or low malignant potential (LMP).The level of expression at early stage cancer (stage Ⅰ and Ⅱ ) was higher than those in advanced cancer(stage Ⅲ and Ⅳ ). In early stage carcinoma, the expression level was involved in the differentiation of tumor cell, and well-differentiated cancer expressed NM23-H1B mRNA in comparatively higher level. Conclusion: The novel gene NM23 N1B is closely correlated with the ovarian cancer.     

The Influence of LHRH, EB and PGF2α on the Development of Ovarian Follicles in Guinea Pigs

Effects of luwinizing hormone releasing hormone ( LHRH), estradiot benzoate (EB), prostaglandin (PGF2α) and the age of animals on the development of ovarian follicles in immature and mature guinea pigs were studied. Administration of 5ng LHRH / hr, 10 times a day, starting from 20-30 days Of age induced the flrst vaginal opening within 4 days, and 5 of the 10 animals had first ovulation. Tire results also show that appropriate doses of EB facilitated the development of ovary. The granulosa cells of guinea pigs at different ages were cocuttured with the pituitart, glands of mature ones in vitro. Results indicate that the responsiveness of granulosa cells of 20-day-guinea prgs to gonadotropins was similar to that of the matured ones. Effects of gonadotrophin on ovulation in hysterectomized and PGF2α or saline-treated animals were tested. Results demonstrate that when PGF2α was given simultaneously with hCG or FSH, 80% of the guinea pigs ovulawd and had fresh, healthy looking corpus tuteum. It is concluded that increased LHRH release from hypothatamus may be the key factor for tire onset of puberty in guinea prgs. EB and PGF2α can influence the development of the ovary.     

Effect of Matrine on Human Ether à go-go Related Gene (HERG) Channels Expressed in Chinese Hamster Ovary Cells

Objective: To observe the effect of matrine on human ether à go-go related gene (HERG) potassium channels expressed in Chinese hamster ovary (CHO) cells and investigate whether HERG channel is a new target of the pharmacological effect of matrine on arrhythmia and tumor. Methods: HERG channel potassium current in CHO cell was recorded using whole-cell patch-clamp technique, and the influence of matrine on the current was explored. Results: Matrine inhibited HERG potassium current in a dose-dependent manner, and the 50% inhibitory concentration (IC50) was 411±23 μmol/L. Matrine had no significant effect on the activation kinetics, and mainly blocked HERG channels in their closed state. Conclusions: The blocking effect of matrine on HERG channels might be one of the mechanisms against arrythmias and tumors. Unlike most other blockers exerting blocking effect at the intracellular sites by entering the cell with the opening of HERG channel, matrine blocked HERG channels at the extracellular sites.     

Ultrastructure of malignant Brenner tumor of the ovary.

Six malignant Brenner tumors of the ovary were studied electronmicroscopically. The histologic features of the tumors resemble to those of nonkeratinized squamous cell carcinoma of the uterine cervix. The ultrastructural features of malignant Brenner tumor included intracytoplasmic glands and cysts, presence of cilia, abundant glycogen, numerous desmosome-tonofilament complex, of which the cilium is most characteristic in the ovarian surface epithelium or Mullerian epithelium, but not in uroepithelium. Desmosome-tonofilament complex, the ultrastructure specific for the squamous epithelium, was not seen in the uroepithelium. We conclude that the appearance of cilium in malignant Brenner tumor indicates that the tumor is derived from the Mullerian epithelium, and that the presence of desmosome-tonofilament complex evidences that the tumor cells have differentiated into squamous epithelium. Our findings have proved that the malignant Brenner tumor arises from the Mullerian epithelium and recapitulates the vaginocervical epithelium.

     

Treatment of ovarian malignant germ cell tumors with preservation of fertility.

Conservative operation and postoperative chemotherapy were given to 15 patients with malignant germ cell tumors of the ovary with the preservation of fertility and ovarian functions. Four patients, one with endodermal sinus tumor and three immature teratoma, had full term deliveries after the operation. The possibility was discussed to preserve young women's fertility and ovarian function in treating their malignant germ cell tumors.