磷酸盐尿性间叶肿瘤12例临床病理分析

目的：探讨磷酸盐尿性间叶肿瘤的临床病理学特征。方法回顾性分析12例磷酸盐尿性间叶肿瘤的临床资料、组织学及免疫表型,并复习相关文献。结果12例中男性8例,女性4例,年龄23~63岁,平均40．5岁;病程1~14年,平均5．6年;患者均有不同程度的骨痛、关节痛和活动受限伴低血磷、高尿磷;肿瘤最大径1~7．5 cm,平均2．7 cm;肿瘤主要由梭形细胞构成,部分病例伴有破骨样多核巨细胞,血管丰富,可见簇状厚壁畸形血管、薄壁血管、散在脂肪岛及软骨样细胞;7例伴有不规则钙盐沉积,2例伴条索状上皮,其中CK(AE1/AE3)阳性;10例核分裂象少见,2例核分裂象多见,其中1例肿瘤细胞异性明显;瘤细胞均表达vimentin、CD56(其中2例分别为弱阳性和灶阳性),11例NSE阳性,8例CD99阳性,7例BCL-2阳性,4例CD34和6例SMA呈不同程度阳性,10例Ki-67增殖指数≤5%,2例分别为10%、25%。随访时间2~108个月,其中2例分别于术后72、84个月复发,其余病例均未见复发。结论磷酸盐尿性间叶肿瘤多表现为良性或低度恶性,其组织学形态多变且缺乏特异性,掌握其共性特征并紧密结合临床才能正确诊断。     

胃肠道肿瘤诊断中彩色多普勒超声的应用价值

目的了解彩色多普勒超声在诊断胃肠道肿瘤中的应用价值，提高术前正确诊断率。方法临床彩色多普勒超声所见腹腔或盆腔占位，后经手术或超声引导下活检病理诊断进一步证实。结果临床彩超所见腹腔或盆腔占位，后经手术或超声引导下活检病理证实来源为胃、小肠或结肠良恶性肿瘤27例，其中小肠及胃平滑肌肉瘤各3例，空结肠恶性间质瘤5例，胃及结肠腺癌14例，胃淋巴瘤2例。27例病人中26例行纤维内窥镜检查，15例病理活检为恶性肿瘤（15／27），1例病理回报炎症（1／27），10例未见明显占位（10／27）。结论临床彩超在诊断腹腔或盆腔占位中的价值应得到临床医师的重视，在某些病例中甚至优于经典的纤维内窥镜和钡餐及气钡剂双重造影。     

软组织磷酸盐尿性间叶肿瘤的临床病理分析

目的研究磷酸盐尿性间叶肿瘤（混合结缔组织亚型）的临床与病理学特点。方法对1例骨软化症患者的肿块切除标本进行光镜、电镜和免疫组化SP法检测。结合临床资料,并复习相关文献。结果患者表现为顽固性骨软化症、低磷酸盐血症,高磷酸盐尿,高血清碱性磷酸酶,外周血单核细胞增多,血钙浓度正常。经99mTc—OCT（奥曲肽）检查指导临床发现左胭窝区小肿块并切除之。术后3天,血磷恢复正常。光镜下肿瘤主要由肥胖的梭形细胞和破骨细胞样多核巨细胞构成,具有丰富的血管,可见血管外皮瘤样血管、散在脂肪岛、明显的出血及含铁血黄素沉积,肿瘤边缘有不完整的膜状骨样或软骨样组织。电镜下见梭形细胞内含数量不等的细颗粒状电子致密物,其中部分为结晶样高电子致密度颗粒。免疫组化显示多核细胞和单核间质细胞CD68阳性。结论该例为肿瘤源性骨软化症,其病理类型为磷酸盐尿性间叶肿瘤,混合结缔组织亚型。外科切除后治愈,99mTc-OCT对于指导临床发现小的隐蔽病灶很有价值。     

上皮间叶转化及其在间叶源性肿瘤中的研究进展

上皮间叶转化(epithelial mesenchymal transition,EMT)出现在各种生理和病理情况下,参与胚胎发生与器官发育、纤维化过程以及肿瘤的形成和转移.目前的研究使人们对EMT的分子机制有了一定的认识,EMT与肿瘤发生和转移间关系的研究对象较多集中于上皮源性的肿瘤和细胞系,而近年研究发现EMT也存在于间叶源性的肿瘤中,其分子调控机制与在上皮源性肿瘤中可能不同.本文就EMT的分子机制及其在间叶源性肿瘤中的研究进展做一综述.     

脾脏胃肠道间质瘤型间叶源性肿瘤1例

患者女 ,72岁。因外伤后急腹症、昏迷入院。患者不慎从楼梯滚下 ,3ｈ后出现腹痛、昏迷 ,行急症手术见腹腔内大量血液及凝血块 ,吸尽血液 ,压住脾蒂 ,分次缝扎脾蒂和切断 ,切下脾见一肿块与脾门粘边 ,质韧 ,脾脏可见破裂口 ,探察肝脏、胃、小肠无殊。临床诊断 :脾破裂出血及肿瘤 ?脾脏连同肿块送病理检查。眼观 :脾脏 8ｃｍ× 5 5ｃｍ× 2ｃｍ ,脾门有一椭圆形肿物粘附 ,8ｃｍ× 6ｃｍ× 4 5ｃｍ ,有包膜 ,切面灰白灰红 ,实性 ,质软 ,无出血囊性变等继发改变。脾脏上有一破裂口 ,3ｃｍ× 2ｃｍ。镜检 :肿瘤与脾脏边缘粘连 (图 1) ,与脾实质…     

磷酸盐尿性间叶组织肿瘤七例临床病理分析北大核心CSCD

磷酸盐尿性间叶组织肿瘤（phosphaturic mesenchymal tumor,PMT）是一种罕见的肿瘤,与肿瘤性骨软化症密切相关。组织学表现复杂多样且缺乏明确的特征性,常在临床或病理诊断中被误诊,我们收集7例PMT病例。并结合文献探讨其临床特点、影像学表现、病理特征及鉴别诊断。     

21例胃肠道神经源肿瘤临床病理及免疫组化观察

目的：观察胃肠道神经源肿瘤的临床及病理形态学特点并探讨其组织学发生。方法：２１例胃肠道神经源肿瘤进行光镜观察，部分病例作组化及免疫组化染色。结果：２１例胃肠道神经源肿瘤中，发生于胃１４例、小肠６例、直肠１例，发病年龄３０￣５０岁。镜下食 性神经鞘病１２例、神经纤维瘤４例、恶性神经鞘膜４例、节细胞神经瘤１例。恶性神经鞘瘤４例中１例伴有横纹肌肉瘤分化，免疫组化染色Ｓ－１００弱阳性，Ｍｙｏｇｌｏｂｉｎ部     

胃肠道问质瘤的诊断及治疗

胃肠道间质瘤（GIST）是胃肠道最常见的间叶组织性肿瘤，具有非定向分化的特性，由于组织分化程度的不同，有着不同的组织学、形态学和预后特征。20世纪90年代以前所报道的所谓平滑肌瘤、神经内分泌肿瘤经再次免疫组化证实85％以上为间质瘤。为了使临床医师对GIST有一个总体认识，本文仅对GIST的发生机制诊断及治疗现状作一简要概述。     

胃肠道钙化性纤维性肿瘤临床病理分析

目的探讨胃肠道钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的临床病理学特征、免疫表型、诊断及鉴别诊断。方法回顾性分析7例胃肠道CFT的临床病理学及免疫表型特征,并复习相关文献。结果 7例患者中,男性2例,女性5例。发病部位:胃4例、小肠2例、直肠1例。临床表现:发生于胃的患者主要为上腹痛,发生于小肠的1例患者出现肠套叠,其余为体检或手术偶然发现,大体为黏膜下直径0.7~4 cm包块。镜下见肿瘤边界清晰,主要由丰富的透明变性的胶原纤维构成,呈漩涡状、束状及席纹状排列,肿瘤细胞呈梭形,无细胞异型性及核分裂象;7例均可见散在分布的砂砾体及多少不一的炎细胞浸润,2例可见营养不良性钙化。免疫表型:瘤细胞vimentin阳性,1例CD34局部阳性,1例可见CD117散在阳性细胞,desmin、DOG1、S-100、SMA、H-caldesmon和ALK均阴性,β-catenin胞核阴性,Ki-67增殖指数1%~3%。7例患者6例无复发,1例失访。结论 CFT是一种少见的间叶源性良性肿瘤,发生于胃肠道者临床罕见,临床表现及检查无特异性,特征性的组织学形态较易诊断,但需与其他间叶源性疾病鉴别。     

少见子宫间叶源性肿瘤病理诊断的新进展

<正>随着分子病理检测技术的不断发展,研究人员对各种子宫间叶源性肿瘤的认识进一步深化。WHO(2020)女性生殖系统肿瘤（简称第五版WHO）对子宫间叶源性肿瘤的分类、分级进行更新和补充,本文根据第五版WHO分类并结合最新研究进展,对原发于子宫和近年新发现的几种少见间叶源性肿瘤的特点进行归纳和总结,旨在为临床、病理医师的诊断和鉴别诊断提供新思路。1平滑肌源性肿瘤平滑肌源性肿瘤的亚型繁多,本文重点介绍新近引起重视的几种特殊类型的肿瘤。     

Multifocal granular cell tumors of the gastrointestinal tract: Immunohistochemical findings compared with those of solitary tumors

Granular cell tumors (GCT) are infrequently found in the gastrointestinal tract (GIT), and only four previous reports have described lesions occurring simultaneously in different sites. The present case of 11 GCT, located in the esophagus, stomach, colon and pericolic adipose tissue, occurred in a 50-year-old Japanese woman. All GCT appeared histologically benign and there was no sign of recurrence at 3 years after surgery. Immunohistochemical analysis and comparison between this case of multifocal GCT and six cases of solitary benign GCT of the GIT, which were taken from the files of the Department of Pathology at Kitasato University (1986–2000), demonstrated the follow-ing: (1) all diffusely expressed S-100, DCC and bcl-2, and (2) median labeling indices for Ki-67, cyclin D1, p53 (Pab1801), and p21^WAF1/CIP1 of 4%, 24%, 1% and 28%, respectively, for the multifocal tumors, and 3.5%, 23%, 1% and 29%, respectively, for the solitary lesions, with no significant difference between the two groups. Thus, the expression of cyclin D1 and p21^WAF1/CIP1 may be involved in the tumorigenesis of both types of GCT. The present case emphasizes the need to evaluate the entire GIT when a single GCT is identified. Multifocal lesions should be treated conservatively by local excision because, as with the solitary tumors, they exhibit a benign biological behavior, consistent with their low Ki-67 immunoreactivity.     

胃肠道间质瘤的误诊因素分析及鉴别

目的:分析胃肠道间质瘤（GIST）的影像误诊因素及鉴别要点。 方法:搜集术前误诊为GIST患者11例,GIST患者术前未正确诊断2例作为此次研究的对象,对所有患者的影像资料进行回顾分析。13例患者均有手术病理,进行MSCT扫描,观察CT影像特征,分析误诊或未正确诊断GIST的影像学因素及鉴别价值。 结果:13例误诊患者中,2例为腹膜后神经鞘瘤、2例为低分化腺癌、2例为异位胰腺、1例为类癌、1例为平滑肌瘤、1例为未分化肉瘤、2例为转移性癌、另外2例小肠间质瘤术前未正确诊断。误诊与肿瘤发生部位、大小、血供来源、生长趋势、观察方法等因素有关。 结论:GIST有一定的影像特点,降低胃肠道间质瘤的误诊,三维重建-肿块准确定位是重中之重,其次应仔细观察肿块细微关键征象,注意肿块血供来源及生长趋势也是鉴别要点。     

超声胃镜联合腹腔镜在胃间质瘤手术中的应用分析

目的探讨超声胃镜联合腹腔镜在胃间质瘤手术中的应用及疗效,分析其可行性和临床价值。方法回顾性分析我院2015年1月至2019年6月78例行超声胃镜联合腹腔镜下胃间质瘤手术患者的临床资料,统计手术时间、恢复时间、住院时间、并发症等临床指标,并总结临床经验。结果本组78例患者均在超声胃镜引导及定位下顺利完成手术,无患者中转开腹。内镜操作时间5~15 min,平均(10.3±4.7)min;手术时间35~120 min,平均(72.8±18.2)min;恢复时间15~37 h,平均(23.2±6.5)h;住院时间6~16 d,平均(8.5±2.3)d。术后均顺利出院,无胃腔出血、梗阻、穿孔、吻合口瘘、严重心脑血管意外等并发症。结论超声胃镜能弥补腹腔镜和影像学检查的不足,双镜联合治疗胃间质瘤具有层次清晰、定位精准、创伤小、术后并发症少的优点,手术安全可行。     

Diagnostic findings of ultrasound-guided fine-needle aspiration cytology for gastrointestinal stromal tumors: Proposal of a combined cytology with newly defined features and histology diagnosis

Recently, endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA) has been applied for diagnosis of gastrointestinal submucosal tumors. There have been no definite criteria, however, for the adequate cytological diagnosis of gastrointestinal stromal tumor (GIST) in practice. To facilitate this a novel method is proposed that combines cytology and histology. For 49 cases of submucosal tumor of gastrointestinal tract, EUS-FNA was performed. The aspirated materials were processed for cytology and histology. Both cytological and histological findings were examined on immunocytochemical and immunohistochemical staining of c-kit. Of 49 cases, 40 (81.6%) proved adequate for cytological and/or histological examination. On cytology, cluster types were classified into type A (piled clusters with high cellularity showing a fascicular pattern), type B (thin layered clusters with high cellularity showing a fascicular pattern), and type C (mono-layered clusters or scattered cells). Types A and B were strongly associated with histological diagnosis of GIST. Type C clusters needed confirmation on c-kit positivity and histology. Thus, the combined cytology with newly defined features, and classification and histological diagnostic method for EUS-FNA materials can contribute to improved routine diagnosis for GIST.     

Cytologic diagnosis of gastrointestinal stromal tumors of the stomach by endoscopic ultrasound-guided fine-needle aspiration biopsy: cytomorphologic and immunohistochemical study of 12 cases.

Gastrointestinal stromal tumor (GIST) is an uncommon tumor, which was usually diagnosed by endoscopic biopsy or surgical resection. This study evaluated the efficacy and accuracy of endoscopic ultrasound (EUS) -guided fine-needle aspiration (FNA) biopsy in the diagnosis of GIST and reported its cytomorphologic features. Twelve patients with gastric GIST were diagnosed through EUS-guided FNA. Immediate on-site evaluation and cytologic diagnoses were given in nine cases (75.0%) with an average of three passes. Cell blocks provided diagnostic material in three cases (25.0%). Spindle cells were present in the cytologic material in all cases. Two patients had subsequent surgical resections. Immunohistochemical (IHC) studies performed in cell blocks and two surgical specimens all supported the original diagnoses. In the two cases with surgical resections, IHC results in cell blocks were similar to that in the resected specimens. This study demonstrated that when combining smears and cell blocks, EUS-guided FNA is accurate and efficient in the diagnosis of GIST. IHC reactivity in cell blocks correlated with that of the main tumors.     

Expression of HSP90 in gastrointestinal stromal tumours and mesenchymal tumours

Kang G H, Lee E J, Jang K T, Kim K‐M, Park C K, Lee C‐S, Kang D Y, Lee S H, Sohn T S & Kim S
(2010) Histopathology 56, 694–701
Expression of HSP90 in gastrointestinal stromal tumours and mesenchymal tumours Aims: Heat shock proteins (HSP) are up‐regulated under conditions of increased stress, including cancer. Recently, HSP90 has been shown to be crucial to the expression and activation of the KIT oncoprotein. The aim was to explore the role of HSP90 expression as a prognostic marker and therapeutic target in gastrointestinal stromal tumours (GISTs) and other mesenchymal tumours. Methods and results: The expression of HSP90 was evaluated by immunohistochemistry in 92 GISTs, 47 mesenteric fibromatoses, six schwannomas, leiomyomas, melanomas, malignant peripheral nerve sheath tumours and leiomyosarcomas. Western blotting was performed in 22 selected cases. HSP90 overexpression was found in 33.7% of GISTs and was correlated with non‐gastric location, mixed histological subtype, high mitotic index, high risk grades, and specific mutation genotypes. In mesenchymal tumours, HSP90 overexpression was found in 66.7% of malignant peripheral nerve sheath tumours, 83.3% of leiomyosarcomas, and 100% of melanomas. HSP90 expression by Western blotting correlated with the results of immunohistochemistry. The Cox proportional hazards model showed that HSP90 expression is an independent predictor of recurrence in GISTs (P = 0.003). Conclusions: Overexpression of HSP90 is predictive of adverse behaviour in GISTs and may provide a therapeutic solution to the challenge of imatinib‐resistant GISTs and other mesenchymal sarcomas.     

Cyclooxygenase-2 expression in stromal tumors of the gastrointestinal tract

Cyclooxygenase (COX) is a key enzyme in the conversion of arachidonic acid to prostaglandins, prostacyclin, and thromboxane. COX-2 is expressed in many epithelial malignancies, particularly those of the gastrointestinal (GI) tract. COX-2 has been implicated in the pathogenesis of cancers and has a significant negative effect on survival. To date, little is known about the expression of COX-2 in nonepithelial tumors. The objective of this study was to evaluate the expression of COX-2 in GI stromal tumors (GISTs). We evaluated 15 GISTs using tissue microarray. Tissue blocks were retrieved and stained with hematoxylin and eosin to evaluate the histological tumor type. In addition, immunohistochemistry was performed for COX-2, the macrophage marker, CD68 (KP-1), and KIT (CD117). Two pathologists then evaluated the tissues to determine the extent and intensity of COX-2 expression. The location of CD68-positive cells, and whether these cells were COX-2 positive, was also evaluated. The results showed that 80% (12 of 15) of the tumors expressed COX-2. Expression was noted in the cytoplasm of the tumor cells, with variable intensity of staining among the tumors. COX-2 was expressed in both epithelial cell and spindle cell tumors, but appeared stronger in epithelial lesions. In mixed lesions, COX-2 was expressed to a greater extent in epithelial areas. There was a greater extent of COX-2 expression in malignant tumors and tumors located within the stomach. Tumor-infiltrating macrophages (CD68-positive cells) were identified in all of the lesions; in 80% of cases, those macrophages also expressed COX-2. This study is the first to demonstrate COX-2 expression in stromal lesions of the GI tract. The enzyme may play a role in the proliferation of these lesions, suggesting the potential use of nonsteroidal anti-inflammatory drugs in treatment.