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.

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.

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.

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.

α-抑制素联合网状纤维双重染色在卵巢性索-间质肿瘤诊断中的应用

目的卵巢性索-间质肿瘤(sexcord-stromal tumor,SCST)是一类罕见的原发性卵巢肿瘤,种类多样且形态各异。免疫组织化学染色和网状纤维特殊染色在辅助诊断中具有重要作用。然而,单独使用免疫组织化学染色或特殊染色在识别肿瘤细胞时往往既费时又费力,导致诊断困难。本研究旨在通过联合染色技术提高诊断的效率和准确性。方法本研究采用免疫组织化学染色与网状纤维染色的联合应用,以帮助诊断SCST。为了获得更佳的染色效果,我们使用免疫组织化学红染法替代传统的显色方法。结果在采用双重染色法的标本中,免疫组织化学阳性的肿瘤细胞显示为红色,而网状纤维则呈现黑色,两者色彩鲜明且对比明显。结论在卵巢性索-间质肿瘤的诊断中,使用免疫组织化学红染法结合网状纤维双重染色可以清晰地揭示网状纤维与肿瘤组织之间的关系,直观展示肿瘤细胞的类型及其分布。这种方法有助于提高诊断的准确性,值得在临床上推广应用。

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。

巢蛋白和α-抑制素蛋白在不同卵巢性索-间质肿瘤中的表达

目的:探讨巢蛋白(Nestin)和α-抑制素(α-inhibin)蛋白在不同卵巢性索-间质肿瘤(SCST)中的表达情况。方法:选取卵泡膜细胞瘤、卵巢纤维瘤、成年型粒层细胞瘤、卵巢微囊性间质瘤和卵巢低分化支持-莱迪(Sertoli-Leydig)细胞瘤组织标本,制备石蜡切片,行苏木精-伊红染色和α-inhibin、Nestin免疫组织化学染色。采用Spearman法分析Nestin和α-inhibin表达评分的相关性。结果:Nestin在SCST中的卵泡膜细胞瘤、卵巢纤维瘤、成年型粒层细胞瘤中呈阴性表达,在卵巢微囊性间质瘤和卵巢低分化Sertoli-Leydig细胞瘤组织中Leydig细胞呈阳性表达。α-inhibin在卵泡膜细胞瘤、卵巢纤维瘤和成年型粒层细胞瘤呈阳性表达,在卵巢微囊性间质瘤和卵巢低分化Sertoli-Leydig细胞瘤组织中呈阴性表达。Nestin与α-inhibin表达评分无相关性(均P<0.05)。结论:卵泡膜细胞瘤、卵巢纤维瘤、成人型颗粒细胞瘤组织中Nestin蛋白阴性表达,α-inhibin蛋白阳性表达。卵巢微囊性间质瘤、卵巢低分化Sertoli-Leydig细胞瘤组织中Nestin蛋白阳性表达,α-inhibin蛋白阴性表达,且Nestin与α-inhibin蛋白表达无明显关系。

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。

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。

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。

卵巢性索间质肿瘤的超声表现

目的探讨卵巢性索间质肿瘤(OSCSTs)的超声表现。方法回顾性分析28例OSCSTs的临床表现、超声资料、手术及病理结果,探讨其声像图特征。结果 28例OSCSTs患者共30个肿瘤,根据超声表现分为3型:①实性型19例,分别为纤维卵泡膜瘤13例、纤维瘤4例、卵泡膜瘤2例,表现为低回声肿瘤内见条状阴影,后方回声衰减,瘤体周边可见稀疏血流信号。②规则囊实性型5例,均为纤维卵泡膜瘤囊性变,实性部分回声均匀,囊性部分囊壁光滑。③不规则囊实性型4例,为颗粒细胞瘤及纤维肉瘤各2例,呈单房或多房囊实性肿瘤,囊腔大小不等,囊壁及分隔厚薄不均,可见结节状突起,可探及丰富血流信号。结论部分OSCSTs具有较典型超声表现,结合临床及实验室检查,有助于初步诊断OSCSTs。

卵巢性索间质肿瘤的CT诊断

目的探讨卵巢性索间质肿瘤的CT特点,以提高此类肿瘤的诊断水平。方法回顾性分析经手术病理证实的17例卵巢性索间质肿瘤的CT资料,分析其特点。结果 17例卵巢性素间质肿瘤中卵泡膜细胞瘤7例,纤维瘤5例,颗粒细胞瘤4例,纤维-卵泡膜细胞瘤1例。肿瘤形态呈类圆形或卵圆形,边缘清楚,其中实性为主肿瘤14例,囊实性3例;肿瘤大小在2.1～15.0cm之间,平均为8.7cm。CT平扫17例均呈软组织密度肿块,内可有散在小囊状低密度区,增强扫描肿瘤实质无强化或仅有轻微强化;有2例增强后肿瘤内见纤细血管影,其他合并腹腔积液7例,子宫内膜增生4例,子宫内膜癌2例,子宫肌瘤7例。结论卵巢性索间质肿瘤表现为边界清晰的实性肿块,CT表现具有一定的特征,结合患者的临床表现有助于提高这类肿瘤诊断准确率,但确诊仍需要依靠病理。

卵巢颗粒细胞瘤的临床特点和CT诊断分析

目的分析卵巢颗粒细胞瘤的临床资料及CT资料,提高对该疾病的诊断能力。方法回顾性分析13例经手术病理证实的卵巢颗粒细胞瘤的临床及CT资料。结果13例患者中11例初发,且均为单个病灶,左侧卵巢5例,右侧卵巢6例,呈圆形或椭圆形。2例患者为复发,1例单发病灶,呈不规则形,1例3个病灶,呈圆形或椭圆形。CT表现初发病例与复发病例相似,多数肿块边缘光滑,与周围组织分界清楚,11个病灶表现为囊实性肿块,2个病灶以实性为主肿块,2个病灶以囊性为主肿块。增强扫描实性部分、囊壁及分隔不同程度强化,3例呈轻度强化,8例呈中度强化,2例呈明显强化。8例患者伴有不同程度子宫体积增大、子宫内膜增厚,3例患者伴有子宫肌瘤,6例患者伴有腹水。结论卵巢颗粒细胞瘤的临床特点及CT表现具有一定的特征性,但缺乏特异性,综合分析有助于提高该疾病的正确诊断率。