MSCTA辅助儿童卵巢肿瘤蒂扭转定位诊断及精准手术

目的探讨多层螺旋CT血管成像(MSCTA)在辅助儿童卵巢肿瘤蒂扭转中的定位诊断价值及精准手术意义。方法回顾性分析2008年1月至2015年9月重庆医科大学附属儿童医院15例进行了MSCT双期增强的经手术证实为儿童卵巢肿瘤蒂扭转的CT影像数据,采用多平面重建(MPR)、最大密度投影(MIP)、容积再现(VR)等多种血管成像后处理方法显示卵巢血管变化,定位肿瘤起源及扭转侧别,并与手术结果对照。结果 15例2个月至11岁的卵巢肿瘤蒂扭转患儿中良性肿瘤11例(畸胎瘤9例,浆液性囊腺瘤2例),恶性肿瘤4例(卵黄囊瘤3例,混合性生殖细胞肿瘤1例)。MSCTA显示的卵巢血管变化主要表现为患侧卵巢供血动脉远端反常增粗、扭曲以及肿瘤血管网形成,引流静脉表现为卵巢周围静脉丛增粗、迂曲、密度增高,个别可见"漩涡征"。术前MSCTA显示卵巢肿瘤供血动脉为下行的卵巢动脉供血来源5例,为上行的子宫动脉卵巢支供血来源10例,定位肿瘤起源于左侧卵巢并扭转4例,起源于右侧卵巢并扭转11例,均与术中发现一致。术中依据MSCTA提供信息准确选择了手术切口位置、精准离断了肿瘤血管并切除了肿瘤及病变侧卵巢,节约了手术时间,减少了出血量。结论 MSCTA能在术前准确显示卵巢肿瘤血管、定位肿瘤起源及扭转侧别,对儿童卵巢肿瘤蒂扭转精准手术具有重要辅助指导意义。     

一种以复现性NCOA2/3基因融合为特征的类型肿瘤:类似卵巢性索肿瘤的子宫肿瘤

类似卵巢性索肿瘤的子宫肿瘤是一种罕见且独特的肿瘤,其组织学起源不明,具有不确定的恶性潜能.这些肿瘤在形态学上类似于卵巢的性索间质肿瘤,并拥有多种免疫表型.目前,类似卵巢性索肿瘤的子宫肿瘤分子发病机制仍尚未阐明.值得注意的是,该肿瘤缺乏其他具有性索样分化子宫肿瘤(如子宫内膜间质肉瘤)发生的改变.     

卵巢黏液性囊腺瘤合并性索-间质肿瘤的临床病理观察

目的 探讨卵巢黏液性囊腺瘤合并性索-间质肿瘤(MCSC)的临床病理特点、组织起源及其诊断和鉴别诊断.方法 对1例MCSC进行组织形态学、免疫组化观察,并复习相关文献.结果 患者发现盆腔肿物1年,无明显不适及内分泌症状.大体上肿瘤为多房囊性,囊内壁可见散在的实性结节,镜检肿瘤由黏液性囊腺瘤和性索间质肿瘤两种成分组成,前者囊壁衬覆良性肠型黏液柱状上皮,性索间质成分瘤细胞呈小管状、条索状、梁状排列,局部有黄素化,两种成分混合存在,未见移行.免疫组化:性索成分瘤细胞呈Melan A弥漫强阳性;αInhibin和CD56局灶阳性;并见CKpan、vimentin、ER及PR的阳性表达;而EMA、CEA、CD99、S-100、CgA、Syn、nestin及SMA均阴性;Ki-67指数小于5%.结论 MCSC是罕见的卵巢肿瘤,性索间质成分可能是黏液性囊腺瘤囊壁内间质的一种反应性增生.诊断时需与类癌、伴异源成分的支持-间质细胞瘤、畸胎瘤等鉴别.     

卵巢肿瘤蒂扭转11例MSCT表现

目的：探讨卵巢肿瘤蒂扭转的MSCT表现。方法回顾性分析11例经手术病理证实的卵巢肿瘤蒂扭转的CT征象。结果11例中,CT表现为肿瘤蒂部实性团块11例、瘤壁偏心性增厚且与蒂部相连10例、卵巢血管的充盈或不连续8例、瘤壁轻度强化或不强化7例,子宫偏向扭转侧5例,肿瘤内或肿瘤蒂部出血3例。结论卵巢肿瘤蒂扭转的CT表现具有一定的特征性,MSCT可清晰显示这些异常征象并为早期诊断做出有益提示。     

胰腺黏液性囊性肿瘤临床病理分析

目的；探讨胰腺黏液性囊性肿瘤（MCN）的临床病理学特点。方法：6例MCN均行B超或CT检查。除1例外，均作了手术切除治疗。对6例（MCN）作常规HE及免疫组织化学染色观察。结果：6例MCN中，女性4例，男性2例，平均年龄47岁，均位于胰腺体尾部。黏液性囊腺瘤（MCA）3例，囊壁内衬高柱状黏液上皮，上皮周围可见卵巢样间质组织；黏液性囊腺癌（MCC）3例，黏液上皮 有不典型增生，均有囊壁或胰腺组织的浸润，1例上皮周围可见卵巢样间质。CEA和CK 7在上皮中均阳性，c-erbB－2均阴性，间质SMA均阳性。结论：胰腺MCN是好发于中年女性的少见肿瘤，绝大多数发生于胰腺体尾部。MCA内衬高柱状上皮，上皮外来卵巢样间质包绕；MCC上皮细胞有不典型增生， 浸润性生长。MCN手术切除率高。     

孤立性纤维性肿瘤 MSCT 表现

目的：探讨孤立性纤维性肿瘤（ SFT）的多层螺旋CT（ MSCT）表现。方法：收集我院经手术病理证实的9例孤立性纤维性肿瘤患者的临床资料,对其MSCT表现进行回顾性分析。结果：9例孤立性纤维性肿瘤中,位于胸部6例,腹腔2例,盆腔1例。 MSCT检查见：较小病灶边缘规则,较大病灶呈分叶样;6例边界清晰,3例边界不清;7例密度较均匀,2例见不规则坏死区,1例见点状钙化;增强扫描动脉期呈轻到中度强化,静脉期持续性强化。结论：SFT的MSCT表现具有一定特征性,正确认识其MSCT的影像学特点,可有助于提高该病术前诊断的准确率。     

胰腺导管内乳头状黏液性肿瘤的诊断和鉴别诊断

目的探讨胰腺导管内乳头状黏液性肿瘤的临床病理学特征及其与黏液囊性肿瘤的鉴别诊断要点。方法复习17例导管内乳头状黏液性肿瘤的临床病理学特征,与13例黏液囊性肿瘤对照;行HE染色及免疫组织化学EnVision法染色,检测肿瘤内黏液素MUC（1、2、5AC）的表达。结果17例导管内乳头状黏液性肿瘤中10例发生在男性;13例位于胰头。大体切面可观察到15例肿瘤与胰腺主导管相通。镜下可见到胰腺导管增生呈乳头状,并有上皮轻至重度不典型增生的改变。无卵巢样间质,肿瘤内交错出现萎缩或正常的胰腺腺泡和胰岛。9例主要表达MUC2,4例主要表达MUC5AC,4例伴有浸润癌者主要表达MUC1。13例黏液囊性肿瘤中11例发生于中老年女性;胰尾部10例,胰头1例,全胰腺2例;肿瘤与主导管不相通。组织学特征是含有卵巢样间质。肿瘤细胞主要表达MUC5AC,不表达MUC2,伴有浸润癌的2例,癌组织也表达MUC1。结论导管内乳头状黏液性肿瘤预后较好,患者性别、年龄、肿瘤部位、卵巢样间质、与主胰管是否相通及表达MUC2和（或）MUC1检测均可帮助诊断,并与黏液囊性肿瘤鉴别。后者主要表达MUC5AC。MUC1阳性提示侵袭性生物学行为。     

卵巢黏液性囊性肿瘤伴实性附壁结节

目的：探讨卵巢黏液性囊性肿瘤伴实性附壁结节的临床病理学特点。方法：对2例卵巢黏液性囊性肿瘤进行光镜观察和免疫组化染色并复习文献。结果：1例卵巢黏液性交界性乳头状囊腺瘤伴实性附壁恶性纤维组织细胞瘤结节，结节内瘤细胞呈多形性。1例卵巢黏液性囊腺瘤伴实性附壁间变性癌结节；间变性癌结节内瘤细胞体积较大，胞质丰富，嗜酸，少数胞质透明，排列呈巢或索状；免疫表型：EMA和cytokeratin阳性。恶纤组结节中肿瘤细胞AACT和vimentin阳性。结论：免疫组化有助于卵巢黏液性囊性肿瘤伴实性附壁结节的诊断及鉴别诊断。     

左肺门支气管和血管在横断面上的配布特点

目的：探讨左肺门支气管和血管在横断面上的配布特点。方法：在44例成人胸部连续横断层标本和10例多层螺旋CT图像上，追踪观察了左侧第1、2、3肺门处支气管、肺动脉和肺静脉的分支（或属支）及其毗邻关系。结果：在气管权层面上，尖后段静脉居中，其前方，前段动脉居前段支气管的内前方；其后方，尖后段动脉上行于相应支气管内侧。在肺动脉权层面上，左肺动脉发出尖后段动脉和前段动脉，其外侧可见尖后段支气管和前段支气管。在27例（61、4％）断层标本上，左肺动脉与前段动脉一起呈“鱼钩”状勾绕左上肺静脉。在左肺上叶支气管层面上，左肺上叶支气管居中，其前方，前段静脉汇入左上肺静脉；其后方，上段动脉发自左肺下叶动脉。在左肺下叶支气管层面上，上段支气管发出。于上叶内，下舌段支气管居中，其前方，舌静脉干汇入左上肺静脉；其后方，舌动脉干发自左肺下叶动脉。在底段上、下静脉层面上，内前、外侧和后底段支气管呈顺时针排列，各底段动脉相对同名支气管呈周围性分布，底段上、下静脉呈向心性走行。结论：通过寻找标志性结构，在横断面上可准确识别左肺肺段和亚肺段的支气管、肺动脉和肺静脉。     

64排CT重建对先天性肺隔离症的诊断价值

目的 探讨64排CT重建技术对先天性肺隔离症的诊断价值.方法 对经外科手术证实的4例先天性肺隔离症患儿的64排CT增强扫描及后重建技术的影像资料进行分析.结果 4例经外科手术证实均为肺内型先天性肺隔离症,其中3例位于左下叶后基底段、1例位于右下叶后基底段,均位于下叶内后方脊柱旁沟内,呈边界清晰多囊腔样占位表现,MPR、MIP、VRT等重建技术能明确显示先天性肺隔离症的异常体动脉供血、引流静脉及隔离肺内的丰富血管网,并可通过调整窗宽、窗位显示隔离肺全貌及其与周围组织的关系.结论 64排CT重建技术对先天性肺隔离症的诊断具有明显的优势,尤其VRT重建图像能够立体、逼真、直观显示先天性肺隔离症的异常体动脉供血、引流静脉及隔离肺内的丰富血管网.     

具有内分泌功能的卵巢肿瘤

目的探讨具有内分泌功能的卵巢肿瘤的临床病理特征.方法对本院临床表现有内分泌功能并经手术治疗的24例卵巢肿瘤进行临床内分泌表现、组织学类型分析,并行免疫组织化学EnVision二步法染色,抗体为AE1/AE3、上皮膜抗原(EMA)、α-抑制素、Calretini、平滑肌肌动蛋白(SMA).结果 (1)临床内分泌的表现主要为性激素异常, 在幼女或绝经后妇女均有较明显的症状和体征,而在生育年龄妇女则表现的较为隐匿和复杂.(2)肿瘤的组织学类型主要为卵巢性索-间质肿瘤卵巢型13例(颗粒细胞瘤8例,泡膜纤维瘤2例,硬化性间质瘤3例),睾丸型7例(支持细胞瘤1例,支持- Leydig细胞瘤5例,Leydig细胞瘤1例),非特异性类固醇细胞瘤2例;这类肿瘤11例直径<5 cm,4例较大或巨大,最大直径达18 cm;切面多为灰粉黄色,实性或囊实性.另外2例为原发上皮性肿瘤,直径分别为12 cm和14 cm.(3)免疫组织化学染色显示卵巢性索-间质肿瘤α-抑制素全部(22/22)和Calretini绝大多数(18/22)呈阳性表达,组织形态分化好的区域表达强于分化差的区域;2例上皮性肿瘤的间质黄素化细胞也呈阳性表达.SMA在5例泡膜纤维瘤和硬化性间质瘤均呈强阳性表达,部分(3/8)颗粒细胞瘤呈弱阳性表达.部分(6/22)性索-间质肿瘤AE1/AE3阳性表达,但EMA均为阴性.结论具有内分泌功能的卵巢肿瘤多数临床表现为性激素的异常,临床表现与肿瘤的组织学类型不完全一致.其组织学类型主要为性索-间质肿瘤.非性索-间质性卵巢肿瘤也可表现为性激素异常.免疫组织化学染色可协助诊断,并用于与上皮性肿瘤鉴别.     

Immunoexpression of inhibin alpha subunit, inhibin/activin betaA subunit and CD99 in ovarian tumors

OBJECTIVE: Anti-inhibin alpha and inhibin/activin betaA subunit and anti-CD99 monoclonal antibodies (mAbs) have recently been demonstrated to be able to label ovarian granulosa cells; thus, they may be of value in the diagnosis of granulosa cell tumors. The present study aimed to determine what combination of these mAbs may be useful for the differential diagnosis of sex cord-stromal tumors of ovary. DESIGN: Immunohistochemical analyses with anti-inhibin alpha and inhibin/activin betaA subunit antibody and anti-CD99 mAb were performed on 42 ovarian tumors, including sex cord-stromal tumors (29), ovarian epithelial cancers (10), and Krukenberg tumors (3). RESULTS: All sex cord-stromal tumors were positive for inhibin alpha subunit, and 17 cases (58.6%) of sex cord-stromal tumors were immunoreactive for inhibin/activin betaA subunit. Epithelial tumors and Krukenberg tumors were all negative for inhibin/activin betaA subunit except mucinous carcinoma, which showed strong cytoplasmic immunoreactivity. All sex cord-stromal tumors except one granulosa cell tumor showed membranous staining for CD99. A case of serous carcinoma and a case of mucinous carcinoma were positive for CD99, and the remaining epithelial tumors and Krukenberg tumor were all negative for CD99. CONCLUSIONS: The results of immunohistochemical analysis, together with literature review, suggest that inhibin alpha subunit may be a useful diagnostic marker for sex cord-stromal tumor of the ovary. In addition, anti-CD99 antibody may be useful for the differential diagnosis between ovarian tumors. Inhibin/activin betaA subunit has a limited usefulness in the differential diagnosis of ovarian tumor because of its wider immunoreactivity for both sex cord-stromal tumors and mucinous carcinomas. The differential diagnosis of sex cord-stromal tumors of the ovary would be better made with a combined use of both anti-inhibin alpha subunit and anti-CD99 mAbs.     

Immunhistochemische Keimstrangmarker

Sex cord markers comprise proteins and hormones that are produced in sex cord-derivatives in normal ovaries and testes as well as in gonadal sex cord-stromal tumors. Sex cord markers (e.g. inhibin-alpha) are used clinically as serum tumor markers. Immunohistochemical staining of sex cord markers may be helpful in the differential diagnosis of ovarian sex cord-stromal tumors versus surface epithelial-stromal tumors, germ cell tumors, other ovarian tumors, and ovarian metastases. Inhibin-alpha has been shown to be the most specific marker of sex cord differentiation. In comparison, calretinin is a somewhat more sensitive albeit less specific marker. Currently, an immunohistochemical panel including inhibin-alpha and calretinin is considered most helpful in the differential diagnosis of ovarian sex cord-stromal tumors. CD99, Müllerian inhibiting substance (MIS), melan A and CD10, being sex cord markers of limited sensitivity and specificity, should only be used as part of an antibody panel in specific diagnostic settings. EMA, CK7 and chromogranin are considered additional markers that may be useful in the differential diagnosis of ovarian sex cord-stromal tumors.     

An ovarian steroid cell tumor causing virilization and massive ascites

Steroid cell tumors, not otherwise specified (NOS), are rare ovarian sex cord-stromal tumors with malignant potential. The majority of these tumors produce several steroids, particularly testosterone. Various virilizing symptoms such as hirsutism, temporal balding, and amenorrhea are common in these patients; however massive ascites is an infrequent symptom. A 52-year-old woman with the sudden onset of virilization and massive ascites presented for treatment at Severance Hospital. After clinical evaluation, the patient underwent an exploratory laparotomy and a complete surgical staging procedure. She recovered from the surgery uneventfully and was discharged from the hospital five days after surgery. We present here an unusual case of an ovarian steroid cell tumor, NOS, and a brief review of the literature regarding these types of tumors.     

Gonadotropic hormone binding to human ovarian tumors

A variety of normal human ovarian tissues as well as benign and malignant human ovarian neoplasms have been examined in vitro for the presence of gonadotropin binding sites and gonadotropin-stimulable cAMP production, in order to determine whether gonadotropic hormones have an effect on ovarian tumors. Binding of either FSH or hCG, or both, was demonstrated in several tumors from each of the histologic subgroups of primary ovarian tumors, including epithelial, sex cord-stromal, and germ cell types. Cyclic AMP stimulation was found in one tumor of sex cord-stromal origin, in two of epithelial origin, in two of germ cell origin, and in one tumor-like condition, pregnancy luteoma. Additional biochemical studies are needed to characterize receptor sites and to relate them to histologic features, growth behavior in vivo, and serum gonadotropin levels.     

Metastasis of breast lobular carcinoma in a cervical leiomyoma

We report here a case of 40-year-old female admitted in view of bilateral ovarian tumors. There was past history of modified radical mastectomy. CT scan revealed a uterine cervicalfibroid in addition to bilateral adnexal masses. Lobular carcinoma metastasis were seen in the leiomyoma as well as in the bilateral ovaries. Metastasis of ductal carcinoma to uterine leiomyoma have been reported in literature. The present case is unique in two ways--(1) metastasis of lobular carcinoma (2) occurring in a cervical leiomyoma.     

Immunoreactive beta-endorphin in ovarian sex cord-stromal tumors

The possible production of the opioid polypeptide beta-endorphin (beta-EP) was investigated in paraffin-embedded tissue from 17 ovarian tumors with the use of a specific anti-beta-EP antibody and the avidin-biotin-peroxidase staining technique. Only sex cord-stromal tumors (ten cases) showed positive staining. Strong beta-EP immunoreactivity was present in Leydig's cells of Sertoli-Leydig cell tumors; weaker sporadic staining was present in cells of granulosa cell tumors, and faint staining was present in occasional, luteinized theca cells of fibrothecomata. These findings suggest that cells with the sex cord-stromal phenotype that are capable of steroid production can also produce beta-EP. The latter may be a component of the "functional" status associated with some ovarian sex cord-stromal tumors and may serve as a helpful marker in distinguishing this type of tumors from germ cell or epithelial neoplasms.     

Uterine tumors resembling ovarian sex cord tumors are polyphenotypic neoplasms with true sex cord differentiation.

In this study, we present the clinicopathologic features and immunophenotypic characteristics of five cases of uterine tumors resembling ovarian sex cord tumors and three cases of endometrial stromal tumors with sex cord-like elements, with emphasis on immunohistochemical markers of sex cord differentiation. The mean patient age was 42 years (range 19-69 years), and vaginal bleeding was the most common clinical presentation. The tumors were usually polypoid masses arising in the uterine fundus, with a mean tumor size of 6.7 cm. Sex cord patterns in uterine tumors resembling ovarian sex cord tumors, including anastomosing cords, trabeculae, small nests, tubules, and in one case, a striking retiform architecture with Leydig-like cells, comprised from 70 to 100% of the tumor volume. All uterine tumors resembling ovarian sex cord tumors were positive for two or more markers of sex cord differentiation; all five cases showed strong immunoreactivity for calretinin, with coexpression of CD99 (four cases), Melan-A (two cases), and inhibin (two cases). Endometrial stromal tumors with sex cord-like elements were less frequently positive for markers of sex cord differentiation, with each case positive for one marker (calretinin, two cases; CD99, one case). In addition, all eight cases were frequently positive for cytokeratin, CD10, vimentin, estrogen receptor, and progesterone receptor; desmin immunoreactivity, when present, was limited to minor foci of smooth muscle. Overall, the morphologic and immunohistochemical findings in uterine tumors resembling ovarian sex cord tumors strongly support that these unusual uterine tumors are polyphenotypic neoplasms with true sex cord differentiation.     

Uterine tumors resembling ovarian sex cord-stromal tumors: synchronous uterine tumors resembling ovarian sex cord-stromal tumors and ovarian sex cord tumor

Uterine tumors resembling ovarian sex cord-stromal tumors (UTROSCTs) are very rare. In this article, we present 3 cases that manifest classical histomorphological features alongside diverse immunohistochemical findings. As a distinctive finding, one of the patients had UTROSCT in the uterus and an ovarian sex cord tumor, called granulosa cell tumor, in the left ovary, simultaneously. Problems in diagnosing such pathologic condition generally arise because of the variable histologic picture of UTROSCT and may cause problems for general and other nongynecologic surgical pathologists. Immunohistochemically, these tumors express different markers that indicate their polyphenotypic origins.