腹膜假黏液瘤临床病理分析

目的探讨腹膜假黏液瘤(pseudomyxoma peritonei, PMP)的临床病理特征、诊断及治疗.方法对1992至2002年经手术和病理诊断的腹膜假黏液瘤10例患者的临床资料进行分析总结并进行随访.结果全组均行手术治疗,其中2例行姑息性局部切除,2例行局部切除,3例行局部切除加阑尾切除术,3例行局部切除加阑尾及卵巢切除术.1例术后10个月死亡,1例18个月死亡,2例失访,余6例3～72个月健在.结论腹膜假黏液瘤是一个既包括良性又包括交界性和恶性的一类黏液性病变,应综合分析做出诊断.阑尾与腹膜假黏液瘤的发生有直接的关系.其预后与浸润状况及分化程度有关.     

卵巢交界性浆液、粘液性肿瘤病理诊断新概念

本文总结近年有关卵巢交界性浆液、小数点 液性肿瘤文献论述病理诊断的新概念。分别为：（1）卵巢交界性肿瘤基本诊断标准;（2）卵巢交界性浆液性肿瘤概括Piura(1992),Russel(1994)、Silva(1996),Eichhorn(1999),Scully(1999)病理诊断标准;（3）卵巢交界性粘液肿瘤概括Piura(1992),Kuman(1996),Riopel(1999),Scully(1999)病理诊断标准。（4）腹膜假粘液瘤;（5）上皮内癌;（6）微浸润;（7）种植;（8）淋巴结转移;（9）多中心性;（10）命名;（11）预后。     

腹膜假黏液瘤的基础研究进展

摘 要：腹膜假黏液瘤是黏液癌的一种罕见亚型,大部分临床病例由阑尾黏液肿瘤在腹腔种植引发。腹膜假黏液瘤的发病机制仍不清楚,当前的临床治疗手段无法有效避免其复发。全文论述近年来腹膜假黏液瘤的基础研究进展,主要包括腹膜假黏液瘤动物模型研究、腹膜假黏液瘤中黏液分泌相关信号通路、降解黏液的物理化学方法研究,以及腹膜假黏液瘤中的基因突变研究等,以期为腹膜假黏液瘤的临床治疗提供参考。     

卵巢交界性上皮性肿瘤90例临床病理及预后分析

[目的]针对卵巢交界性上皮性肿瘤的临床特点及治疗方法以探讨其复发的相关因素.[方法]回顾性分析卵巢交界性上皮性肿瘤90例,所有患者均经手术治疗及术后病理证实,随访36～154个月.[结果]90例患者平均发病年龄39.2岁;临床表现以无症状的盆腔肿块为主,共39例(43.3％);浆液性交界瘤中CA125升高28例(56.0％),黏液性交界瘤中CA19-9升高18例(56.3％);所有患者均进行手术治疗,41例行保留生育功能手术;I期72例(80.0％),Ⅱ期6例(6.7％),Ⅲ期12例(13.3％),无Ⅳ期病例;浆液性50例(55.6％),黏液性32例(35.6％),混合性4例(4.4％),其他类型4例(4.4％);20例术后辅以化疗;术后随访1例为非肿瘤复发导致死亡,余均存活,10例(11.1％)复发;保守性手术患者的复发与肿瘤分期、单双侧、包膜破裂、手术类型、腹膜种植、微浸润、微乳头等因素有显著关系(P＜0.05).[结论]交界性肿瘤患者发病年龄较年轻;血CA125、CA19-9检测对诊断有帮助;肿瘤FIGO分期、腹膜种植、微浸润、微乳头结构、手术类型及包膜破裂与预后有关.     

肾脏黏液样小管状和梭形细胞癌一例并文献复习

目的探讨肾脏黏液样小管状和梭形细胞癌（MTSCC）的临床病理学特点、诊断、治疗方法及预后。方法报告1例左肾MTSCC合并肾盂输尿管畸形患者的临床资料、病理特点及免疫组织化学、特殊染色情况并复习相关文献。结果本例临床症状明显，肿瘤边界清楚，合并。肾盂输尿管重复畸形。镜下表现肿瘤由紧密排列的小管构成，细胞异型性小，小管间为淡染黏液样间质并见梭形细胞区域。黏液样间质Alcianblue染色阳性。上皮样细胞区CK（＋），EMA（＋），AE1／AE3（＋），Vimentin（＋）。行肾癌根治术后恢复良好，未行辅助治疗，随访18个月未见肿瘤复发和转移。结论肾脏MTSCC是一类低度恶性的肾上皮性肿瘤，组织形态温和，合并发育畸形者罕见。肿瘤边界清楚，可有假包膜，极少囊性变和坏死。预后较好，可长期生存。少数病例发生局部复发或淋巴结转移。正确诊断非常重要，早期手术是其首选的治疗方法。     

心脏黏液瘤的外科治疗

目的 探讨心脏黏液瘤的诊断和治疗经验。方法 回顾性分析自1995年10月～2001年10月收治的83例心脏黏液瘤病例(其中左心房黏液瘤57例,右心房黏液瘤19例,左心室黏液瘤2例,右心室黏液瘤5例)。术前经彩色超声心动图确诊,均在体外循环下行黏液瘤摘除术,同期行二尖瓣成形术8例,二尖瓣机械瓣置换术3例,三尖瓣成形术62例,三尖瓣机械瓣置换术1例,房间隔缺损修补术6例,隔膜型主动脉瓣下狭窄解除术1例。结果 无围术期及手术死亡,随访72例,时间1个月到6年,心功能(NYHA分级):Ⅰ级51例,Ⅱ级11例,Ⅲ级4例,1例发生脑梗死死亡,1例死于车祸,1例死因不明,3例复发再次手术,复发率4.2％。结论 心脏黏液瘤一经确诊应尽快手术,手术效果满意,复发率低,彩色超声心动图对诊断及术后随访有重要作用,应注意术后随访。     

3459例卵巢肿瘤病理统计分析

全文统计分析近5年经病理检查证实的3 459例卵巢肿瘤,其中瘤样病变1 960例（56.66%）,良性肿瘤1 292例（37.35%）,交界性肿瘤64例（1.85%）,恶性肿瘤143例（4.13%）,瘤样病变、良性、恶性、交界性之比为30.63：20.19：2.33：1.发生部位以右侧卵巢较多,良性肿瘤发生在双侧卵巢的情况较恶性肿瘤多见.总平均发病年龄为36.25岁,良性肿瘤的发病年龄小于总平均年龄,交界性及恶性肿瘤的发病年龄大于总平均年龄.在良性肿瘤中以生殖细胞肿瘤最多见,占72.60%,其次为表面上皮-间质肿瘤（23.22%）、性索-间质肿瘤（3.95%）等;交界性肿瘤中,以黏液性肿瘤为多,占70.31%;恶性肿瘤中以表面上皮-间质肿瘤最多见,占69.23%,其次为性索-间质肿瘤（13.99%）、生殖细胞肿瘤（10.49%）等;瘤样病变中,以子宫内膜异位症最多,占33.78%.     

卵巢成熟性畸胎瘤恶变临床病理分析

目的:探讨卵巢成熟性囊性畸胎瘸恶变的临床病理特点.方法:收集8例卵巢成熟性囊性畸胎瘤恶变肿瘤,分析病变的临床表现、病理特征分期及随访结果.结果:卵巢成熟性囊性畸胎瘤恶变多发生于绝经后女性.8例中2例为类癌,2例为恶性甲状腺,1例黏液腺癌,1例上皮内癌,1例甲状腺肿类癌,1例鳞状细胞癌.8例均为临床Ⅰ期,除1例死亡外,其余7例均无瘤生存.结论:卵巢成熟性囊性畸胎瘤恶变率低,病理检查需细致全面,结合临床综合治疗1期患者预后良好.     

卵巢癌组织中前列腺素类物质水平及其生物学意义的研究

目的 :研究前列腺素类物质 (prostaglandins ,PGs)与卵巢上皮性浆液性肿瘤发生发展的关系。方法 :采用放射免疫法对 5 4例卵巢上皮性浆液性肿瘤组织 (浆液性卵巢癌 33例 ;交界性浆液性瘤 10例 ;良性浆液性瘤 11例 )和 10例正常卵巢组织进行前列腺素E2 (PGE2 )、6 酮 前列腺素F1α(6 keto PGF1α)及血栓素B2 (TXB2 )水平检测。结果 :PGE2 、6 keto PGF1α及TXB2 在浆液性癌组织中的水平明显高于交界性浆液性瘤、良性浆液性瘤和正常卵巢组织中的水平 ,均P <0 0 5 ,而交界性、良性瘤和正常卵巢之间比较差异均无显著意义 ,均P >0 0 5。在卵巢上皮性浆液性癌临床Ⅰ +Ⅱ与Ⅲ +Ⅳ期间 ,组织学分级各级别间 ,有无腹水及有无淋巴结转移间PGs水平比较差异均无显著意义 ,均P >0 0 5。结论 :1)PGs可能与卵巢上皮性浆液性肿瘤发生发展有关 ;2 )PGE2 、6 keto PGF1α及TXB2 可作为卵巢上皮性浆液性癌的诊断和鉴别诊断指标。     

卵巢来源腹膜假黏液瘤临床病理分析

  目的  探讨卵巢来源腹膜假黏液瘤（pseudomyxoma peritonei,PMP）的临床病理学特征及免疫组织化学表型。  方法  回顾性分析2010年1月至2019年1月272例于首都医科大学附属北京世纪坛医院确诊为PMP患者的临床资料,研究PMP肿瘤来源,复阅病理切片并行免疫组织化学标记,标记抗体包括CK7、CK20、CEA、Villin、CDX2、SATB2、CA125、ER、PR、PAX8、MUC1、MUC2等。  结果  272例PMP中阑尾来源245例（90.1%）、非阑尾来源27例（9.9%）。卵巢来源PMP仅5例（1.8%）,其中4例为黏液性囊腺瘤、1例为交界性黏液性囊腺瘤,均发生在单侧,5例中2例合并成熟性囊性畸胎瘤;腹膜播散肿瘤中2例为无细胞性黏液、2例为低级别腹膜黏液癌、1例为高级别腹膜黏液癌。免疫组织化学法检测显示,5例PMP患者组织中CK20、CEA、Villin、CDX2均阳性,2例黏液性囊腺瘤合并畸胎瘤患者的SATB2部分阳性、2例SATB2阴性、1例SATB2灶状阳性。  结论  卵巢来源PMP罕见,需对阑尾全部取材或对可疑组织块行连续切片,以排除阑尾黏液性肿瘤,并结合临床症状体征、影像学表现、手术所见、组织学特征及免疫组织化学法检测进行综合分析。      

OTUB1在90例卵巢黏液性肿瘤中的表达分析

背景与目的：卵巢黏液性肿瘤是卵巢上皮性肿瘤的主要亚型之一,其恶变机制目前尚未明确。泛素化是人体主要的翻译后修饰机制,并与肿瘤密切相关。去泛素化酶可通过逆转泛素化过程从而影响肿瘤的发生、发展。该研究旨在分析编码人体最主要的去泛素化酶的癌基因泛素醛结合物1(OTU deubiquitinase,ubiquitin aldehyde binding 1,OTUB1)在卵巢黏液性肿瘤中的蛋白表达及临床应用。方法：收集2010-2015年复旦大学附属妇产科医院病理科诊断的卵巢良性黏液性囊腺瘤、卵巢交界性黏液性肿瘤及卵巢原发性黏液性癌病例共90例,收集临床病理资料,完成OTUB1的免疫组织化学法表达实验并作统计分析。结果：90例卵巢黏液性肿瘤中,卵巢良性黏液性囊腺瘤有14例,卵巢交界性黏液性肿瘤有17例,卵巢原发性黏液性癌有59例。OTUB1在卵巢原发性黏液性癌的蛋白表达率及阳性程度显著高于卵巢良性黏液性囊腺瘤(P<0.01);OTUB1在卵巢交界性黏液性肿瘤中上皮内癌的阳性率显著高于肠型交界性黏液性肿瘤(P<0.01);随着FIGO分期增高,OTUB1的阳性率及阳性程度增加(P<0.05);OTUB1在输卵管有累及的患者中表达率及阳性程度均高于未累及者(P<0.05);OTUB1在子宫和大网膜累及的患者中阳性率及阳性程度均高于未累及者(P<0.05);OTUB1在有淋巴结转移的患者中表达率及阳性程度均高于无转移者(P<0.05)。结论：OTUB1在卵巢原发性黏液性癌中的蛋白表达水平明显高于卵巢良性黏液性囊腺瘤,并且和FIGO分期及肿瘤侵袭转移性高度相关。在临床病理中可将OTUB1作为卵巢黏液性肿瘤恶变与否的评价指标及肿瘤进展的辅助判别指标。     

Borderline ovarian tumour: pathological diagnostic dilemma and risk factors for invasive or lethal recurrence

By comparison with ovarian carcinomas, borderline ovarian tumours are characterised clinically by superior overall survival, even in women with peritoneal spread. In this Review, we aimed to clarify the histological and clinical factors potentially defining a high-risk group in whom disease is likely to evolve to invasive disease. Invasive peritoneal implants (in serous borderline ovarian tumours) and residual disease after surgery were the two factors clearly identified. Other factors are controversial owing to increased risk of invasive recurrence: micropapillary patterns in serous borderline ovarian tumour, intraepithelial carcinoma in mucinous lesions, stromal microinvasion in serous lesions, and use of cystectomy in mucinous borderline ovarian tumours. The pathologist has a pivotal role in assessment of the borderline nature of ovarian tumours and in identification of high-risk criteria, most of which are histological. But, reproducibility of the histological interpretation of some of these potential criteria--eg, classification of peritoneal implants (particularly in desmoplastic subtype), stromal microinvasion, micropapillary patterns, and intraepithelial carcinoma in mucinous borderline ovarian tumours--remains unclear, and should be investigated.     

Noninvasive, microinvasive, and invasive mucinous carcinomas of the ovary: a clinicopathologic analysis of 40 cases

BACKGROUND: Whether ovarian mucinous tumors with epithelial stratification of more than three cell layers in the absence of stromal invasion (i.e., carcinomas diagnosed according to the Hart and Norris criteria) should be placed in the same category as mucinous tumors with stromal invasion (i.e., unquestionable carcinomas) remains controversial. Because individual mucinous tumors frequently contain benign, borderline, and malignant components, the adequacy of sampling has been emphasized. METHODS: We examined 21 mucinous carcinomas with no destructive stromal invasion (MCNI), 4 mucinous carcinomas with microinvasion (MCMI) of < 2 mm, and 15 mucinous carcinomas with invasion (MCI) of > or = 2 mm. Tumors were diagnosed as MCNI according to Hart and Norris criteria (12 tumors) or when severe nuclear atypia was present (9 tumors). Cases of MCNI were selected for review if a section had been taken for each 2 cm or less of the tumor's greatest diameter. The International Federation of Gynecology and Obstetrics stage and follow-up data of each case were examined, and differences among MCNI, MCMI, and MCI were analyzed. RESULTS: All 21 patients with MCNI and all 4 patients with MCMI had Stage I disease; there was no recurrence or death in these cases. In contrast, 7 of 15 patients with MCI had Stage II or III disease, and 8 patients died. CONCLUSION: MCNI clearly should be distinguished from MCI and be classified as noninvasive carcinomas after the absence of destructive stromal invasion has been confirmed by examining a sufficient number of sections.     

Cytoplasmic and stromal expression of laminin γ 2 chain correlates with infiltrative invasion in ovarian mucinous neoplasms of gastro-intestinal type

Ovarian mucinous neoplasms of gastro-intestinal type (GI-type) are known to be a heterogeneous tumor composed of benign, borderline and non-invasive and invasive malignant lesions. The presence of infiltrative invasion is also known to be an important prognostic factor of this neoplasm. Laminin γ 2 chain, known to stimulate tumor cell invasion and migration, has not been sufficiently investigated in ovarian mucinous neoplasms. The purpose of this study was thus to clarify the role of laminin γ 2 in ovarian mucinous neoplasms of GI-type. We selected each morphological phase of tumor development from 61 cases of mucinous neoplasms of the GI-type: 55 adenoma lesions, 60 borderline lesions, 20 microinvasive lesions, 17 intraepithelial carcinoma lesions, 38 expansile invasive carcinoma lesions, 19 infiltrative invasive carcinoma lesions and 5 mural nodules lesions; and evaluated the localization of laminin γ 2 in the lesions using immunohistochemical method. The staining pattern was classified into i) basement membranous (BM), ii) cytoplasmic (CYT) and iii) stromal (S) pattern. The BM pattern was characteristic in adenoma, borderline, and interaepithelial and expansile invasive carcinoma lesions. The CYT and S patterns were characteristic in infiltrative invasive lesions. The staining pattern of mural nodules was similar to that of infiltrative invasion. The infiltrative invasion of GI-type ovarian mucinous neoplasms may be promoted by cytoplasmic and/or stromal expression of laminin γ 2 chain.     

Advanced stage mucinous adenocarcinoma of the ovary is both rare and highly lethal

BACKGROUND:

Primary mucinous adenocarcinomas of the ovary are uncommon, and their biological behavior is uncertain. Retrospective studies have suggested that many mucinous carcinomas initially diagnosed as primary to the ovary have in fact metastasized from another site. A prospective randomized trial provided an opportunity to estimate the frequency of mucinous tumors, diagnostic reproducibility, and clinical outcomes.

METHODS:

A phase 3 trial enrolled 4000 women with stage III or IV ovarian carcinoma, treated by surgical staging and debulking, with randomization to one of five chemotherapeutic arms. Slides and pathology reports classified as primary mucinous carcinoma were reviewed independently by three pathologists. Cases were reclassified as primary or metastatic to the ovary according to two methods. Overall survival (OS) of reclassified groups was compared within the groups and with that of patients with serous carcinomas.

RESULTS:

Forty‐four cases were classified as mucinous adenocarcinoma upon review. Using either method, only about one third were interpreted by the three reviewers as primary mucinous carcinomas. Reproducibility of interpretations among the reviewers was high, with unanimity of opinion in 30 (68%) cases. The median survival (MS) did not differ significantly between the groups interpreted as primary or metastatic, but the OS was significantly less than that for women with serous carcinoma (14 vs 42 months, P < 0.001).

CONCLUSION:

Advanced stage mucinous carcinoma of the ovary is very rare and is associated with poor OS. Many mucinous adenocarcinomas that are diagnosed as primary ovarian neoplasms appear to be metastatic to the ovary. Cancer 2011. © 2010 American Cancer Society.     

Whole-genome allelotyping identified distinct loss-of-heterozygosity patterns in mucinous ovarian and appendiceal carcinomas.

PURPOSE: Mucinous adenocarcinoma of the ovary is one of the common histologic types of ovarian cancer. Its pathogenesis is largely unknown. In addition, the differential diagnosis of metastatic mucinous carcinomas to the ovaries, particularly those originating from the appendix, remains challenging. The purpose of this study is to identify molecular biomarkers for mucinous ovarian adenocarcinoma and compare them with those of appendiceal origin. EXPERIMENTAL DESIGN: Genome-wide loss-of-heterozygosity (LOH) analysis was done on DNA isolated from 28 microdissected primary mucinous ovarian carcinomas and five appendiceal adenocarcinomas. Markers from high-loss regions were selected for further analysis on a total of 32 ovarian and 14 appendiceal cancers. RESULTS: High levels of LOH rates (>40%) were detected on chromosome arms 9p, 17p, and 21q in mucinous ovarian carcinoma cases. The frequency of allelic loss was similar between high-grade and low-grade mucinous ovarian carcinoma cases but was significantly higher in ovarian versus appendiceal cases. In addition, LOH rates on five chromosomal loci were statistically different between ovarian and appendiceal carcinomas. CONCLUSION: A high frequency of LOH can be found in mucinous ovarian adenocarcinomas independent of grade. Despite histologic similarities between mucinous ovarian carcinomas and metastatic appendiceal carcinomas, they have distinct LOH profiles, which may be used for distinguishing the two diseases.     

Mucinous tumors of the ovary. A clinicopathologic study of 70 cases

Seventy cases of mucinous ovarian tumor were reviewed. All patients were followed for a minimum of 5 years. Clinicopathologically, three groups were defined: (1) mucinous cystadenoma, which demonstrated no nuclear stratification and no stromal invasion (15 cases); (2) mucinous tumor of uncertain malignant potential, which was characterized by nuclear stratification of two to three layers and no stromal invasion (21 cases); and (3) mucinous carcinoma, which showed stromal invasion and/or nuclear stratification in excess of three layers (34 cases; 15 with invasion, 19 without). All patients with mucinous cystadenomas remained tumor-free after initial surgery. Two patients with mucinous tumors of uncertain malignant potential died of tumor at 55 and 72 months, respectively, whereas 18 with mucinous carcinomas died after intervals ranging from 2 to 71 months. All mucinous tumors of uncertain malignant potential were Stage I at presentation. Twenty-one mucinous carcinomas were Stage I (six tumor deaths), one was Stage II (tumor death), ten were Stage III (nine tumor deaths), one was Stage IV (tumor death), and one was of uncertain stage (tumor death). Patients with mucinous carcinomas having stromal invasion demonstrated poorer survival (10 of 15 dead) than those with mucinous carcinomas lacking this finding (8 of 19 dead); however, stromal invasion was related to higher stage (5 with invasion Stage I; 16 without invasion Stage I).     

肾脏黏液性小管状和梭形细胞癌研究进展

肾脏黏液性小管状和梭形细胞癌(mucinous tubular and spindlecell carcinoma,MTSCCa)是新确定一种罕见的低度恶性肾上皮性肿瘤。多见于青年女性,临床上无明显症状。以往常被诊断为梭形细胞(肉瘤样)肾细胞癌或不能分类的肾细胞癌。大体肿物界限清楚,切面实性、灰白色。组织学特点是肿瘤细胞排列成管状和实性梁索状漂浮于黏液性基质中,Alcianblue染色阳性。免疫组化显示复合性免疫表型。临床预后好,可复发并具有潜在远处转移的可能,应重视与其他肾脏良恶性肿瘤(后肾腺瘤、肉瘤样癌和集合管癌等)相鉴别。     

COTA在卵巢粘液性肿瘤中的分布及其在诊断上的应用

The localization and diagnostic value of colon-ovarian tumor antigen (COTA) were studied by immunohistochemical S-P method. COTA was found in all 18 cases of mucinous ovarian cystadenocarcinoma (100%) and 3 border line cystadenomas (100%). Yet in 18 cases of ovarian benign mucinous cystadenoma, only 3 were positive (16.6%). These results indicate that COTA is a highly sensitive and specific antigen for mucinous ovarian cystadenocarcinoma and has potential for the early detection of malignant changes in mucinous ovarian cystadenoma.