A Retrospective Study of Ovarian Sex Cord Stromal Tumors at the Egyptian National Cancer Institute

Background: Ovarian sex cord-stromal tumors are an uncommon heterogeneous group of tumors with different biological behaviors and clinico-patho- logic aspects. Aim of the Work: This study will review the clinico-pathologic aspects of sex-cord stromal ovarian tumors at the National Cancer Institute (NCI), Cairo University, Egypt, as well as their management and follow-up regarding disease free survival and overall survival. Patients and Methods: This retrospective study was conducted at the National Cancer Institute Cairo University, Egypt on female patients with ovarian sex cord stromal tumors in the period from January 2008 to December 2012 with a follow-up period of 24 to 84 months. The age of the patients, different clinical presentations, radiological findings, associated uterine bleeding (need for endometrial biopsy), pre-operative CA125 levels, surgical management done, different histopathological types, different biological behaviors, presence of ascites (and its correlation with the histopathology), Adjuvant chemotherapy (according to biological behavior and pathological type), and follow-up of non-benign cases for up to 84 months will all be documented and studied. Results: The mean age at presentation was 47.34;abdominal pain and mass were the commonest presentations 54.5% and 53.2% respectively;the main radiologic findings were a pelvic mass +/- ascites which had no correlation to the pathological type (p = 0.075). Endometrial hyperplasia and endometrial carcinoma were associated with 22% and 2.5% of cases respectively. Stages I and II represented 95% of patients with non-benign tumors (48 patients). Panhysterectomy +/- infracolic omentectomy or fertility sparing surgery were done in 70.1% and 29.9% of patients respectively. AGCTs were the commonest pathological type (49.4%). Adjuvant chemotherapy was given to 14 patients (46.7%) with non-benign tumors. 6 recurrences (20%) in 30 patients with non-benign tumors on regular follow-up were documented. The median of disease free survival (DFS) was 50.5 months. The median overall survival was 49.5 months. Conclusion: Ovarian SCSTs are uncommon neoplasms with different biological behaviors where AGCTs are the commonest among Egyptian females. Hormonal manifestations are uncommon where abnormal vaginal bleeding is the commonest one. The presence of ascitic fluid has no correlation with the pathological type of the tumor. Early stages (I and II) represented about 95% of non-benign tumors. Surgical management without lymphadenectomy +/- adjuvant chemotherapy is the main line of treatment at our institute. The OS was shorter than that documented in the literature. A small number of patients, reluctance of follow-up and unavailability of some patients’ data were the main drawbacks in this study.

Ovarian Sex Cord-Stromal Tumors in Postmenopausal Women and Total Laparoscopical Management

BACKGROUND: Ovarian sex-cord stromal tumors (SCST) take up 5% of the ovarian neoplasm and may develop in?to?an ovarian mass or a haemoperitoneum. The surgical management of SCST in early-stage adult patients is not well?defined. CASE REPORT: A 69 year-old postmenopausal woman was admitted for metrorrhagia, a right ovary mass and?increasing pelvic pain. Preoperative clinical and instrumental examination suspected an ovarian tumor, and the?laparoscopic right ophorectomy and the frozen section suggested an ovarian SCST. To fast restore and preserve woman?integrity, total laparoscopic hysterectomy (TLH) plus left salpingo-ophorectomy (SO) were performed, without complications?in the short and long term follow-up. CONCLUSION: In the authors’ opinion, the minimally invasive management?of SCST by TLH plus bilateral SO followed by a prolonged surveillance and without intensive surgical staging,?could be an appropriate clinical and surgical choice in elder patient at early stage, since these tumors are slow at?growth, recurring locally and only a long time after initial treatment. We suggest, after a minimally invasive treatment,?a possible “wait and see” option, as in our case report.

Serous cystadenoma with co-existing stromal tumor with sex-cord stromal elements: An extremely rare tumor

Surface epithelial-stromal tumors are the most common neoplasm of ovary in the reproductive age and beyond. Less than 1% of epithelial ovarian tumors occur before the age of 21 years while 75% of sexcord stromal tumors (Sertoli and Leydig Cell) occur in the third and fourth decades of life. Serous tumors rarely occur in combination with germ cell tumors, sex-cord stromal tumors or granulosa cell tumors. Thus it is extremely rare for a combination of the above two tumors to occur before 21 years although a case report for a tumor with epithelial, stromal and sex-cord stromal elements for a 58-year-old patient has been documented. We believe ours is the first case in literature of a massive Serous Cystadenoma with co-existing stromal tumor with minor sex-cord stromal (Sertoli-Leydig Cell) elements.

Sclerosing stromal tumor of the ovary with masculinization,Meig’s syndrome and CA125 elevation in an adolescent girl:A case report

BACKGROUND Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of theovary. It was first reported and named in 1973. These tumors typically presentwith pelvic/abdominal pain and tenderness, a mass, and/or abnormal menses,but rarely present with masculinity in children and adolescents. Only 2 cases ofthese tumors have been reported in premenarchal girls, who demonstratedhormonal activity, with a history of the development of a virilizing female due tohyperandrogenism. Here, we report a case of a giant SST with obviousmasculinity combined with Meig’s syndrome and CA125 elevation.CASE SUMMARY A 17-year-old female presented with a 7-year history of the development ofmasculinity and a 2-year history of amenorrhea. She had hirsutism, acne, obviouslaryngeal prominence, and voice deepening. Physical examination showed a malesuprapubic hair pattern and a 4.0 cm × 1.5 cm enlarged clitoris. Laboratory testsshowed that the testosterone level was > 15.00 ng/mL (normal range: 0.14-0.76ng/mL), and androstenedione level was > 10.00 ng/mL (normal range: 0.3-3.3ng/mL). A computed tomography scan of the abdomen and pelvis was carriedout and showed a large, solid and cystic, partly calcified pelvic mass in the rightovary measuring 27.1 cm × 20.0 cm × 11.0 cm, 15 cm above the umbilicus (to thelevel of the upper part of L1). Intraoperative findings at laparotomy revealed alarge tumor arising from the right ovary. Approximately, 500 mL of pale-yellow clear liquid was found in the pelvic cavity. A right salpingo-oophorectomy wasperformed. Microscopic examination and immunohistochemical staining of thesurgical specimen showed an SST of the ovary.CONCLUSION This report is remarkable as our patient was not only diagnosed with an SST ofthe ovary, which is extremely rare in this age group, but was the largest and mostobvious reported patient with this tumor who presented with virilization.Therefore, gynecologists should be aware of this potential complication inadolescent girls with a mass in the ovary.

Uterine tumor resembling an ovarian sex cord tumor:A case report and review of literature

BACKGROUND Endometrial stromal tumors originate from the endometrial stroma and account for<2%of all uterine tumors.Uterine tumor resembling an ovarian sex cord tumor(UTROSCT)is a rare histological class of endometrial stromal and related tumors according to the latest World Health Organization classification of female genital tumors.Here,we report a case of UTROSCT in a 51-year-old woman.CASE SUMMARY A 51-year-old woman had irregular menses for 6 mo.The patient visited a local hospital for vaginal bleeding.Pelvic computed tomography(CT)showed a mass in the pelvic cavity.Five days later,she came to our hospital for further diagnosis.The results of contrast-enhanced CT and pelvic ultrasound at our hospital suggested a malignant pelvic tumor.She then underwent total removal of the uterus with bilateral salpingectomy.Postoperative histological examination showed that the tumor cells had abundant cytoplasm,ovoid and spindle-shaped nuclei,fine chromatin,a high nucleoplasm ratio,and a lamellar distribution.The findings were consistent with UTROSCT,and the results of immunohistochemical analysis supported that diagnosis.The tumor was International Federation of Gynecology and Obstetrics stage IB.No adjuvant therapy was administered after radical surgery.The patient was followed up for 58 mo,and no recurrence was found.CONCLUSION We report a case of UTROSCT with abnormal menstruation as a symptom,which is one of the most common symptoms.In patients with vaginal bleeding,ultrasonography can be used as a screening test because of its convenience,speed,and lack of radiation exposure.For patients with long-term tamoxifen use,routine monitoring of the endometrium is recommended.As UTROSCT may have low malignant potential,surgery remains the primary management strategy.Additionally,fertility preservation in patients of childbearing age is a vital consideration.

MRI鉴别诊断卵巢颗粒细胞瘤与卵泡膜纤维瘤

目的观察MRI鉴别诊断卵巢颗粒细胞瘤(OGCT)与卵泡膜纤维瘤(OTF)的价值。方法回顾性分析37例OGCT(OGCT组)与74例OTF(OTF组)女性,比较其MRI参数;行多因素logistic回归分析,观察各参数单独及联合鉴别诊断OGCT与OTF的效能。结果组间病灶囊实性分型,囊变程度、囊区最大径,实性部分T2WI信号、强化程度、表观弥散系数(ADC),以及是否合并蜂窝征/奶酪征、有无肿瘤内血管及出血差异均有统计学意义(P均<0.05)。其中,囊变程度、ADC及是否合并蜂窝征/奶酪征为MRI鉴别OGCT与OTF的影响因素,其鉴别OGCT与OTF的曲线下面积(AUC)分别为0.834、0.868及0.744,而三者联合的AUC为0.934,大于其单一AUC(P均<0.05)。结论病灶囊变程度、实性部分ADC及是否合并蜂窝征/奶酪征等MRI特征有助于鉴别OGCT与OTF。

Adult Granulosa Cell Tumor in a Young Woman: A Case Report and Literature Review

Granulosa cell tumors belong to the group of stromal and sex cord tumors of the ovary. The adult type is the most common type and usually occurs during the perimenopausal period while the juvenile type mostly affects young women. This is a report of a 26 years old woman who presented an adult type of granulosa cells tumor, with review of the literature. She complained abdominal pain and distension associated with abundant ascites and underwent a right adnexectomy. The pathology examination diagnosed an adult granulosa cell tumor. The two entities of granulosa cell tumors (juvenile and adult types) are distinguished by their characteristic morphological aspects on histological examination allowing diagnostic orientation.

Retiform Sertoli-Leydig Cell Tumor of the Ovary

Sertoli-Leydig cell tumor of the ovary is a kind of sex cord-stromal tumor, which occurs between teens and twenties with symptoms including abdominal pain and swelling. The incidence rate is infinitely rare comprising less than 0.5% of all ovarian tumor. The average age of “retiform Sertoli-Leydig cell tumor” is 17 years as compared to 25 years for Sertoli-Leydig cell tumors as a group. We have experienced this rare case of retiform Sertoli-Leydig cell tumor in a 25-year-old foreign patient with the complaint of palpable mass on the right lower quadrant and an irregular menstrual period. The patient underwent right salpingo-oophorectomy and tumor stage was FIGO stage 1A. We report with a brief review of literature.

青春期前女童双侧卵巢硬化性间质瘤一例

卵巢硬化性间质瘤(ovarian sclerosing stromal tumor,OSST)是一种罕见的良性卵巢性索间质肿瘤,目前文献报道的病例总数约为100例。OSST主要发病群体为20~30岁育龄期女性,临床表现为腹痛、附件包块,肿瘤可具有内分泌功能,易导致月经紊乱、不孕,少数患者出现多毛痤疮、声音低沉等男性化表现。该病在青春期前女童中极为罕见,半数OSST患儿为体检时发现盆腔肿物,也有因性激素升高引起临床表现而就诊。其通常为单侧卵巢发病,目前仅有3例报道发生于双侧卵巢。对于双侧OSST的年轻女性患者,可行双侧卵巢肿物切除术或至少保留一侧正常卵巢组织手术,最大程度保留患者生育力。该病预后良好且术后随访均未见复发及转移。本文报道1例罕见的青春期前女童双侧OSST病例,并通过相关文献复习对该疾病的临床特征、鉴别诊断、治疗及预后进行讨论,以提高临床医生对该疾病的认识。

卵巢性索间质肿瘤的相关临床表现与超声特征分析

目的探讨卵巢性索间质肿瘤相关临床表现与超声特征。方法选取50例卵巢性索间质肿瘤患者,均予以超声检查,分析临床表现、检验异常及不同类型卵巢性索间质肿瘤的超声特征。结果50例患者均绝经,腹痛14例,腹部包块22例,泌尿症状17例,腹水4例,阴道出血13例;检验值异常包括糖类抗原125(CA125)异常10例,糖类抗原199(CA199)异常5例,人附睾蛋白4(HE4)异常3例。卵泡膜纤维瘤28例,形态规则、边界清晰、内部实性回声,后方回声多衰减,多无血流信号;纤维瘤10例,形态规则,边界多不清晰,实性内部回声,后方回声衰减,检出血流信号占比50%;卵泡膜细胞瘤4例,形态规则,边界清晰,实性内部回声,后方回声无变化或衰减,检出血流信号占比50%;纤维肉瘤4例,形态规则或不规则、边界清晰或不清晰,不规则囊实性内部回声,后方回声无变化或增强,有血流信号;颗粒细胞瘤4例形态规则,边界清晰,不规则囊实性回声,后方回声无变化,检出血流信号占比50%。结论卵巢性索间质肿瘤好发于绝经女性,部分肿瘤标志物水平异常,部分有较典型超声表现。

类似卵巢性索肿瘤的子宫肿瘤8例临床病理特征分析

目的:探讨类似卵巢性索肿瘤的子宫肿瘤(UTROSCT)的临床病理特征。方法:收集8例UTROSCT患者的临床病理资料及随访资料,进行光镜观察及免疫组化染色。结果:8例均为绝经前患者,年龄27~52岁。肿瘤均为单发,直径2.1~14.0 cm。5例表现为子宫出血,2例表现为腹痛,1例在体检时发现。5例位于子宫肌壁间,3例位于黏膜下。镜下肿瘤均由上皮样或性索样细胞构成,呈小梁状、巢片状或性索样排列。细胞无明显异型性,无明确坏死及核分裂。肿瘤细胞同时表达性索标记(α-inhibin,WT-1,CD56,CR)、肌源性标记(SMA,desmin)及上皮性标记(CK、CAM5.2、EMA),ER与PR均为阳性表达,但H-caldesmon常为阴性表达。4例不同程度表达神经内分泌标记物(Sy N、NSE、Cg A)。1例子宫全切术后30个月复发,1例子宫及双附件切除术后26个月后死于肺癌,其余6例均无病生存。结论:UTROSCT确诊主要依靠特征性的形态及免疫表型,多呈良性临床经过,但可复发或转移,患者需长期随访。

α-抑制素CD99联合应用在卵巢性索间质肿瘤表达意义的研究

目的 :探讨α -抑制素、CD99、EMA在卵巢性索间质肿瘤中的表达情况及在该类肿瘤的诊断、鉴别诊断及预后等方面的意义 ,为临床病理诊断和鉴别诊断提供科学依据。方法 :收集我院病理确诊的卵巢性索间质肿瘤 4 1例 ,其中颗粒细胞瘤 2 1例 ,卵泡膜纤维瘤 11例 ,环状小管性索瘤 4例 ,支持间质细胞瘤 3例 ,卵巢支持细胞瘤 2例。同时选择 10例卵巢上皮性肿瘤作为对照进行对比观察(见附表 )。所有标本均经中性福尔马林固定 ,4um切片 ,用SP法免疫组化标记 ,α -抑制素、CD99、EMA均为鼠抗单克隆抗体。DAB显色后 ,苏木素复染、中性树胶封固。以正常一抗动物血清代替一抗做阴性对照 ,抗体由北京中山生物技术公司提供。结果判定 :α -抑制素阳性在核或浆 ,CD99阳性表达在细胞膜或浆。结果 :颗粒细胞瘤 2 1例中 ,α -抑制素 19例阳性 ,阳性率为 90 .5 % ,CD99有 17例阳性 ,阳性率 80 .9% ;卵泡膜纤维瘤 11例中 ,α -抑制素 9例阳性 ,CD99均为阳性 ;环状小管性索瘤4例 ,α -抑制素均为阳性 ,CD993例阳性 ;支持间质细胞瘤 3例、支持细胞腺瘤 2例 ,α -抑制素、CD99均为阳性 ,幼年性颗粒细胞瘤复发者 1例两次均为阳性。结论 :α -抑制素、CD99联合应用标记性索间质肿瘤有很高的敏感性和特异性 ,对区分性索间质肿瘤类型、

卵巢环状小管性索瘤6例临床病理分析

目的：探讨卵巢环状小管性索瘤良恶性区别的标准。方法：对６例卵巢环状小管性索瘤，良性４例，恶性２例，进行临床病理观察，ＡｇＮＯＲ计数及术后随访。结果：恶性者瘤细胞有轻至中度异型性，且见到分裂象，单纯性和复合性小管减少，伴有梁状或弥漫性颗粒细胞瘤样成分。临床随访转移在术后５年以上。结论：此瘤是一种真性肿瘤，可分为单纯型、颗粒细胞为主型和支持细胞为主型。

卵巢硬化性间质瘤的超声影像及临床病理特征

目的探讨卵巢硬化性间质瘤(ovarian sclerosing stromal tumor,OSST)的超声图像及临床病理特征。方法回顾性分析1996年3月至2013年9月北京协和医院经手术病理证实的12例OSST患者的临床、病理及影像学表现,着重对其超声图像特点进行分析。结果 12例OSST患者中,8例主诉为月经不规律,6例患者性激素水平异常,4例CA125升高。OSST均呈单侧发病,病灶声像图特点:12例(100%)病灶边界清晰;10例(83.3%)呈典型分叶状;7例(58.3%)为囊实性以实性为主结构,4例(33.3%)为实性结构;8例(66.7%)实性部分表现为低回声;12例(100%)病灶实性部分回声不均;10例(83.3%)内部散在细条状高回声;11例(91.7%)病灶后方回声衰减;10例(83.3%)病灶呈丰富规则的血流信号,多为周边及内部规则血流,内部血流呈轮辐状向心分布是其特征性表现。结论 OSST具有特征性的临床表现,有着与其病理特点相对应的典型超声图像特征,超声检查对于术前明确诊断可起到重要作用。

卵巢非妊娠性绒癌临床病理观察

目的探讨卵巢非妊娠性绒癌的临床病理学特征。方法观察2例卵巢非妊娠性绒癌的临床表现、组织学形态及免疫组化特征并复习文献。结果 2例患者均为13岁女童,肿瘤均位于左侧卵巢,瘤组织中可见各型滋养细胞成分,呈巢状、筛状、乳头状排列,伴明显出血坏死;其中1例伴右侧卵巢环状小管性索瘤,免疫组化显示2例绒癌均hCG和CK(+),伴发的环状小管性索瘤CD56和CD99(+)。结论卵巢非妊娠性绒癌为罕见的高度恶性的生殖细胞肿瘤,伴发环状小管性索瘤更为罕见。非妊娠性绒癌易早期转移,预后极差。

卵巢支持莱迪细胞瘤15例临床病理分析

目的:探讨卵巢支持莱迪细胞瘤的临床特点、病理特征、治疗及预后。方法:回顾性分析我院1962年至2009年诊治的15例卵巢支持莱迪细胞肿瘤患者的临床和病理资料。结果:15例术前均未考虑本病,其中13例有男性化、女性化及去女性化表现。6例女性化表现患者同时合并雌激素相关疾病(如子宫肌瘤、子宫内膜癌、子宫内膜增生)。14例术中见大体肿瘤,1例因子宫内膜癌手术,术后病理检查发现本病。肿瘤高分化6例,中分化5例(含有网状及异型成分1例),低分化4例。2例低分化肿瘤术中冰冻病理检查分别误诊为卵巢癌及卵巢颗粒细胞瘤。5例患者保留生育功能,其中3例实现生育。8例患者接受分期手术,其中1例术中发现肿瘤大网膜转移。15例中,9例中、低分化者术后接受辅助化疗。随诊2～34年,14例患者无瘤存活,1例低分化肿瘤治疗后4年复发。结论:支持莱迪细胞瘤难以及时诊断,患者的内分泌表现是提示本病的重要线索。预后良好,对于本病患者可行保留生育功能手术,但需注意低分化肿瘤的复发问题。

睾丸支持细胞瘤1例报告并文献复习

目的探讨睾丸支持细胞瘤患者的临床诊治特点及预后。方法回顾性分析1例睾丸支持细胞瘤患者临床资料,并结合文献进行讨论。结果该患者术后病理诊断普通型睾丸支持细胞瘤,未行放、化疗治疗。随访6月无肿瘤复发转移。结论睾丸支持细胞瘤是一种少见的性索间质肿瘤,恶性程度低,预后较好,术后需定期随访。

伴有性索-间质肿瘤样结构的卵巢子宫内膜样癌临床病理观察

目的探讨伴有性索-间质肿瘤样结构卵巢子宫内膜样癌(ECSCS)的临床病理学特征、免疫表型、诊断和鉴别诊断。方法对1例ECSCS行HE和免疫组化染色,并同时进行文献复习。结果组织学上ECSCS由卵巢性索-间质细胞肿瘤样结构和经典型子宫内膜样癌组成。肿瘤细胞排列成实性片状或条索状,其间可见中空的小管状或长的实性管状,类似于Call-Exner小体样结构,腔内含有嗜酸性分泌物。肿瘤细胞核圆形或卵圆形,核透亮。免疫组化染色显示肿瘤细胞表达CKpan、EMA、CA125、ER和PR(+),而CD99、CEA和inhibinα(-)。结论ECSCS是卵巢子宫内膜样癌一种少见的形态学亚型,类似于卵巢的Sertoli-Ledig细胞肿瘤或成人型粒层细胞瘤,免疫组化对二者的鉴别诊断有帮助。

卵巢性索-间质肿瘤端粒酶活性的免疫组化检测及其临床病理学意义

背景与目的:端粒酶在女性生殖道发生的上皮性肿瘤中的检测已有相关报道,但对于卵巢性索-间质瘤中端粒酶活性的研究尚未见报道,本研究旨在探讨端粒酶活性在卵巢性索-间质肿瘤中的表达状况及其临床病理学意义。方法:收集25例卵巢性索-间质肿瘤,包括粒层细胞瘤、卵泡膜纤维细胞瘤、硬化性间质瘤及支持-间质细胞瘤。采用免疫组化SLAB法,以多克隆抗体H231检测人端粒酶反转录酶(humantelomerasereversetranscriptase,hTRT)在石蜡包埋肿瘤组织中的表达。结果:hTRT蛋白主要定位于瘤细胞(特别是黄素化或/和间质细胞)胞浆,少部分胞核同时表达。hTRT总阳性率为60.0%,不同类型肿瘤阳性表达分别为:硬化性间质瘤4/4、支持-间质细胞瘤5/5、粒层细胞瘤6/9,卵泡膜纤维细胞瘤0/7。出现内分泌功能紊乱症状者16例,12例表达hTRT,占75.0%。无内分泌症状者则呈阴性或弱阳性。hTRT阳性率与肿瘤类型和内分泌功能紊乱有关(P值分别为0.01和0.041),但与肿瘤分化程度及患者预后无关(P值均>0.05)。结论:在卵巢性索-间质肿瘤中,向黄素化细胞和间质细胞分化的瘤细胞端粒酶活性较高,内分泌功能活跃的细胞其端粒酶活性也可能增强,从而导致患者出现内分泌功能紊乱的症状。

卵巢环状小管性索瘤2例报道并文献复习

报道2例罕见的卵巢环状小管性索瘤（SCTAT），讨论该瘤的临床病理特征。通过临床和随访资料复习、光镜组织学检查和免疫组化染色，对2例SCTAT的临床和病理学特征进行研究并复习文献。临床表现均为月经紊乱和腹部包块。病理检查2例均为单侧卵巢肿块，最大径分别为29cm和9cm，镜下肿瘤均由简单和复杂的环状小管组成，瘤细胞α-抑制素（＋），其中1例有盆腔和腹股沟淋巴结转移且于2年内复发并死亡。回顾分析显示，SCTAT在1994年1月1日～2004年12月31日我国文献（清华同方：中国医院数字图书馆、CHKD期-t1全文数据库。并列图书馆查阅及后参考文献核实病例无重复）中共有79例报道，包括我们的2例共约81例，但有Peutz—Jegher，s综合征（PJS）肿瘤仅1例，远低于国外报道。无PJS的肿瘤中约22．5％为恶性，较国外报道相近。初步研究结果显示，与PJS无关的SCTAT中约1／5有恶性生物学行为。α-抑制素是SCTAT首选的免疫标记物。