β-catenin在软组织肿瘤和组织起源未定的实质脏器肿瘤中的研究进展

摘 要：β-catenin（CTNNB1）是Wnt信号通路的关键调节分子,对细胞增殖、分化和功能的调节至关重要。目前在实质器官肿瘤中的研究相对较多,而在一些软组织肿瘤和组织起源未定的实质脏器肿瘤中研究相对较少。通过阅读文献复习发现,β-catenin在许多间叶性肿瘤的发生中起着十分重要的作用,包括韧带样型纤维瘤病、栅栏状肌纤维母细胞瘤、鼻腔鼻窦型血管外皮细胞瘤、青少年鼻咽血管纤维瘤及卵巢微囊性间质肿瘤。此外,β-catenin与一些组织起源未定的实质脏器肿瘤密切相关,包括肺硬化性肺泡细胞瘤（过去称全肺硬化性血管瘤）、胰腺或卵巢的实性假乳头状肿瘤及肝脏钙化性巢状间质上皮肿瘤。全文重点阐述β-catenin突变的间叶性肿瘤及组织起源未定的实质脏器肿瘤的临床病理和分子遗传学特征,强调不同β-catenin突变对潜在预后的影响。     

胰腺实性假乳头状肿瘤一例报告及文献复习

为了分析胰腺实性假乳头状肿瘤的临床病理特征及免疫组织化学特点,对胰腺实性假乳头状肿瘤的临床资料、大体观察、镜下观察及免疫组化结果进行分析.胰腺实性假乳头状肿瘤好发于年轻女性,镜下由实性区及假乳头状区组成,免疫组化程度不一的表达上皮性、间叶性、内分泌性标记.初步研究结果提示,胰腺实性假乳头状肿瘤是较少见的、预后较好的交界性肿瘤,其诊断主要依赖于临床资料、组织学形态及免疫组化标记.     

胰腺实性假乳头状瘤研究现状

胰腺实性假乳头状瘤（solid pseudopapillary tumor,SPT）在临床上比较少见,是一种近年来才被逐渐认识的胰腺肿瘤,其组织发生来源尚未确定.Frantz于1959年首先报道了胰腺SPT.该病曾用名称较多,如实性乳头状瘤、乳头状囊性瘤、实性囊性瘤及实性囊性乳头状上皮肿瘤等.1996年WHO将其统一命名为胰腺实性假乳头状瘤.     

胰腺实性假乳头状瘤病理与免疫组化分析

目的:运用免疫组化技术探讨胰腺实性假乳头状瘤(solid pseudopapillary tumors of pancreas,SPT)的病理形态特征及组织起源.方法:免疫组化EnVision法检测β-catenin、E-cad、Fli-1、CD56、 α1-AT、Vim、NSE、CD10、PR、Syn、CgA及CKpan在35例SPT组织中的表达.结果:β-catenin核(质)阳性表达为86%、E-cad 3%、Fli-1 41%、CD56 54%、α1-AT 94%、Vim 100%、NSE 89%、CD10 86%、PR 60%、Syn 51%、CgA 34%及CKpan 31%.β-catenin与E-cad的表达差异有统计学意义,P<0 05.结论:β-catenin异常核表达与E-cad表达缺失存在相关性,两者在SPT特殊的假乳头形态的形成中可能起重要作用;多种抗体表达支持SPT起源于多潜能干细胞的假说.     

胰腺实性-假乳头状肿瘤的诊断和鉴别诊断

胰腺实性-假乳头状肿瘤(solid-pseudopapillary tumors of pancreas, SPTP)是1种罕见的低度恶性潜能胰腺肿瘤,临床上常常误诊.本文回顾性分析6例SPTP,探讨其诊断与鉴别诊断要点、生物学行为和预后.     

胰腺实性假乳头状瘤的临床病理特征

目的 探讨胰腺实性假乳头状瘤的临床病理特征.方法 回顾性分析3例胰腺实性假乳头状瘤的临床资料、组织病理和免疫表型,并复习相关文献.结果 胰腺实性假乳头状瘤多见于女性,可发生于胰腺的任何部位,肿瘤呈囊实性,细胞形态一致,核分裂相罕见,瘤细胞形成特征性假乳头结构.免疫组化结果显示:Vim(+)、CD56(+)、CD10(+...     

胰腺实性假乳头状癌1例

恶性胰腺实性假乳头状肿瘤（SPTP）即胰腺实性假乳头状癌非常罕见，现诊断1例报道如下。     

胰腺实性假乳头状瘤临床病理及超微结构分析

[目的]了解胰腺实性假乳头状瘤的临床病理特点及其超微结构。[方法]采用HE、免疫组化（EnVision法）及电镜技术观察18例胰腺实性假乳头状瘤。[结果]肿瘤平均直径约7cm,有包膜。免疫组化结果：β-eatenin核阳性（13／18）,E-cad阳性（0／18）,α1-AT阳性（18／18）,Vim阳性（18／18）,CD10阳性（14／18）,PR阳性（12／18）,CKpan阳性（5／18）,Syn阳性（3／18）,CgA阳性（4／18）。电镜示肿瘤细胞内发达的粗面内质网及数量不等之酶原颗粒,少数瘤细胞内见神经内分泌颗粒。[结论]β-eatenin异常核表达和E-cad表达缺失可能与胰腺实性假乳头状瘤的发病有关：胰腺实性假乳头状瘤可能起源于胰腺腺泡并具有神经内分泌特征。     

胰腺实性假乳头状瘤:MDCT诊断及鉴别

目的 探讨胰腺实性假乳头状瘤的多排螺旋CT(multi-detector computer tomography,MDCT)诊断价值.方法 回顾性分析11例经手术病理证实的胰腺实性假乳头状瘤的临床资料及MDCT表现.结果 11例临床多表现为上腹部胀痛不适,所有病例均无黄疸.CT表现为胰腺单发囊实性类圆形肿块,最大直径约5.5～13.6 cm,平均8.6 cm;肿瘤位于胰头部3例,胰体部2例,胰尾部6例.平扫肿瘤呈不均匀的较低密度,边界较清,3例肿块内可见钙化;动态增强后肿瘤实性成分呈不均匀的轻至中度进行性强化,弱于正常胰腺组织强化,囊变坏死区无强化,10例见较完整包膜,7例可见肿瘤与胰腺间呈“喇叭口”样界面.所有病例均无胆管及胰管扩张,未见腹腔及腹膜后肿大淋巴结.结论 胰腺实性假乳头状瘤多见于较年轻女性,好发于胰头、尾部,MDCT对其有较大诊断价值.     

胰腺实性假乳头状肿瘤一例报道

胰腺实性假乳头状肿瘤(solid pseudopapillaryneoplasmof pancreas,SPNP)又称囊性实性乳头状上皮性肿瘤、乳头状囊性上皮肿瘤、实性乳头状肿瘤和乳头状囊性肿瘤等,为一种少见的胰腺肿瘤[1],主要见于青春期少女和年轻妇女,可发生于胰腺任何部位,具有独特的临床病理特征和免疫组化表型,除极少数外,手术切除均能治愈,但易与胰腺其他肿瘤相混淆。为提高对SPNP认识,我们报道1例,并结合文献对其临床病理特点、组织发生和免疫组化表型进行分析探讨。1临床资料1.1一般资料患者女,16岁。因右上腹部疼痛不适5d伴恶心、呕吐1d于2002年11月16日收住…     

Tumeur pseudopapillaire et solide du pancréas

Solid pseudopapillary tumors (SPT) of the pancreas are rare exocrine pancreatic tumors. Their etiology is unknown and they occur most commonly in young women. Local invasion and metastatic evolution is rare in these tumors and their prognosis is excellent after complete surgical resection. We report of SPT in a young female revealed by abdominal pain. The diagnosis of the cystic tumor was based on abdominal ultrasound and CT data. The surgical treatment was conservative and consisted of a tumorectomy. Histological study confirmed the diagnosis of SPT of the pancreas.     

Immunohistochemical evaluation of solid pseudopapillary tumors of the pancreas: The expression pattern of CD99 is highly unique

The aim of this study was to investigate CD99 as a new marker to characterize solid pseudopapillary tumors (SPTs), and to determine a specific panel of markers to identify the disease. We analyzed the clinicopathological characteristics and immunohistochemical features of 37 patients with SPT. All 37 tumors displayed intracytoplasmic dot-like immunoreactivity of CD99 in contrast to membranous staining in all pancreatic endocrine tumors and most of acinar cell carcinomas, along with negative immunostaining in ductal carcinomas. In addition, we observed a loss of expression of E-cadherin in all SPTs as well as in some other pancreatic tumors, and aberrant nuclear expression of β-catenin in most SPTs. Our findings demonstrated for the first time that the pattern of CD99 expression was highly specific for distinguishing SPTs from other pancreatic tumors. CD99 combined with E-cadherin/β-catenin and CD10 can be used as a relatively specific expression profile of SPTs.     

胰腺实性假乳头状瘤的临床特点及预后分析

目的 探讨胰腺实性假乳头状瘤(SPT)的临床病理和免疫组化特征、组织起源、生物学行为及临床治疗方法 和预后.方法 对18例SPT组织标本进行HE染色和免疫组化SP法染色,收集患者的临床资料,并对患者进行随访.结果 18例SPT患者中,女性16例,男性2例,发病年龄9～65岁,平均25.3岁.多以腹痛和上腹部包块为主要症状.肿瘤常有包膜,囊实相间,镜下可见肿瘤由假乳头和囊实性区混合组成,瘤细胞围绕纤维血管轴心形成特征性假乳头结构.免疫组化检测结果 显示,多数肿瘤表达α-抗胰蛋白酶(α-AT)、α-抗糜蛋白酶(α-ACT)和波形蛋白(Vim),阳性率均为94.4%.18例SPT患者全部获得随访,随访时间3个月至10年不等,5例术后复发,均予手术切除.18例SPT患者中,死亡4例,中位生存时间为23.0个月,5年生存率为72.2%.患者年龄、肿瘤大小、SPT特殊的病理表现、大血管侵犯和肿瘤转移与胰腺SPT患者生存期有关.结论 SPT为一种低度恶性肿瘤,可能起源于胰腺多潜能干细胞.好发于青少年女性,有独特的临床病理表现,手术切除后预后好.     

胰腺实性假乳头状肿瘤的临床病理学分析

目的：探讨胰腺实性假乳头状肿瘤的临床病理学特点及免疫表型，方法：对4例胰腺实性假乳头状肿瘤临床资料进行回顾性复习。并采用免疫组织化学EnVision法检测肿瘤细胞Vim、CK8、CK18、Chromogranin、Sy、AAT、NSE、S-100的表达，结果：4倒患者均为女性．平均年龄30岁．肿瘤由乳头状和实性区混合构成，肿瘤细胞形态单一．免疫组化标记显示Vim，4例均呈弥漫性强阳性；AAT灶性强阳性；NSE三例阳性，一例阴性；Sy、Chromogranin和S-100 4例均阴性；CK8和CK18 一例散在弱阳性，另三例阴性。结论：胰腺实性假乳头状肿瘤是一种好发于青年女性、具有低度恶性潜能的肿瘤，免疫组织化学对胰腺宴性假乳头状肿瘤的诊断和鉴别诊断具有重要价值。     

Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPT) is an exceptionally rare neoplasm in children. Its origin remains enigmatic. It is of low malignant potential and occurs most frequently in young females. PATIENTS AND METHODS: A cumulative review of the tumor's clinicopathological characteristics from the world's literature is presented. The clinical course, pathohistologic data and outcome of surgery of four Austrian children treated at the general hospital of Vienna are analyzed. RESULTS: Between 1987 and 1999, four girls (age: 12--16 years) with SPT were diagnosed at our institution. All patients presented with an abdominal mass and uncharacteristic abdominal pain. Two tumors were located in the tail, one in the body and tail and one in the head of the pancreas (diameter: 7--15 cm). Surgical procedures included three distal pancreatectomies and one partial duodenopancreatectomy (Whipple procedure). One patient had two recurrences with metastases that could only be partially resected. Chemotherapy was initiated for this patient. In the follow-up period (range: 6 months to 12 years) all patients are alive with no evidence of recurrence. CONCLUSIONS: SPT is a rare differential diagnosis of a pancreatic mass in children. It is mandatory to establish this diagnosis since complete surgical removal of the tumor even in case of metastases or local invasion offers an excellent prognosis.     

胰腺实性假乳头状瘤的诊断与治疗

对近年来国内外胰腺实性假乳头状瘤(SPT)诊断及治疗方面的认识进行了阐述.SPI是一类原发于胰腺的低度恶性肿瘤,多见于年轻女性,临床症状多不明显,生化指标、肿瘤标志物大多正常.影像学呈囊实性混杂表现,CT增强扫描呈高血供表现.SPT组织来源尚不完全明确,病理诊断主要依靠形态学,免疫组织化学对鉴别诊断有一定参考意义.手术切除是SPT最有效的治疗手段,术中不必常规淋巴结清扫.完整切除者预后良好,极少转移或复发.     

Accumulation of beta-catenin protein, mutations in exon-3 of the beta-catenin gene and a loss of heterozygosity of 5q22 in solid pseudopapillary tumor of the pancreas

BACKGROUND: Solid pseudopapillary tumors (SPT) of the pancreas are neoplasms with a low malignant potential. The molecular events contributing to the pathogenesis of SPTs are still unknown. OBJECTIVES: This study was intended to help better understand the early steps of human SPT development. METHODS: We microdissected 20 SPTs and normal pancreatic tissue. In addition, we examined the DNA from each SPT for mutations in exon-3 of beta-catenin and loss of heterozygosity (LOH) on 9 chromosome arms using 10 microsatellite markers. Immunohistochemical staining for beta-catenin was performed. RESULTS: Activating mutations between codons 32 and 37 of beta-catenin exon-3 were present in 16 cases (80%). Allelic loss on chromosome 5q22.1 was present in 10 cases (55.5%), while no allelic loss was found on chromosomes 1p, 6q, 9p, 9q, 11p, 11q, 17p, or 22q. Nuclear accumulation of beta-catenin was found in 20 cases (100%). CONCLUSION: Mutations in exon-3 of the beta-catenin gene, nuclear accumulation of beta-catenin, and LOH on chromosome 5q22.1 in SPT tissue suggest that these mutations are involved in SPT tumorigenesis.     

胰腺实性假乳头状瘤19例临床分析

 目的　探讨胰腺实性假乳头状瘤（SPT）患者的临床特点、诊断及治疗。方法　回顾性分析8年间收治的19例胰腺SPT患者的临床资料,其中男3例,女16例。结果　手术方式依肿瘤的生长部位及大小而定。术后并发症以胰漏较多见,本组19例中9例发生胰漏。随访3～70个月,未发现复发、转移。结论　胰腺SPT是一种少见的胰腺肿瘤,其多见于年轻女性,治疗依赖于手术切除,预后良好。     

β-连环蛋白在妇科肿瘤诊断中的研究进展

β-catenin is a very unusual protein with multiple functions depending on its cellular localization.The β-catenin gene(CTNNB1)encodes for β-catenin and apart from its well-defined role in cellular adhesion,it is also a component of the Wnt signalling pathway.The Wnt/β-catenin pathway is involved in various normal cellular activities,including determination,proliferation,migration and differentiation in embryonic development and adult homeostasis.Deregulation or constitutive activation of the Wnt/β-catenin pathway may lead to cancer formation.Immunohistochemical expression of β-catenin in gynecologic tumor have been reported recently.In normal epithelia,immunoreactivity was strongly observed at the membrane,partially at cytoplasm,nuclear staining of β-catenin was rarely seen in normal cases; In ovarian carcinomas,β-catenin nuclear expression was found more commonly in endometrioid carcinomas,nuclear β-catenin staining seemed to be of prognostic importance; In endometrium carcinomas,β-catenin nuclear expression were more common in pure endometrioid tumors than in unendometrioid tumors,associated with favorable prognosis,the staining pattern was independent of the menopausal status; In synchronous primary cancers of the endometrium and ovary,activating mutations in β-catenin seemed to distinguish synchronous primary tumors from metastatic tumors.