乳腺典型髓样癌与不典型髓样癌临床病理分析

目的探讨乳腺典型与不典型髓样癌的临床病理特征和生物学行为差异。方法对乳腺典型髓样癌及不典型髓样癌各20例临床病理资料进行分析,并采用S-P法检测nm23、E-cad、p53、ER、PR、c—erbB—2、CD45RO和CD20的表达。结果典型髓样癌组,癌细胞合体性结构〉75％,无腺管结构,间质见弥漫性淋巴细胞浸润;不典型髓样癌组,可见腺管结构,间质无或少量淋巴细胞浸润。免疫组化检测典型髓样癌nm23、E—cad阳性表达均高于不典型髓样癌。典型组随访12～84月（平均37个月）,均无腋下淋巴结转移,均健在;不典型组随访9～84月（平均29．5个月）,腋下淋巴结转移4／20例,其中4例分别于术后1—3年内死亡。结论①要严格掌握乳腺典型髓样癌与不典型髓样癌的病理诊断标准。②不典型髓样癌不完全等同“乳腺浸润性导管癌伴髓样特点。”③乳腺典型与不典型髓样癌的预后不同,前者明显优于后者。     

甲状腺髓样癌81例临床病理分析

目的探讨甲状腺髓样癌(medullary thyroid carcinoma, MTC)的临床病理特征、诊断及鉴别诊断、治疗及预后。方法对81例MTC行免疫组化、BRAF V600E基因检测,分析其临床病理特征及免疫表型等,并复习相关文献。结果女性45例,男性36例,年龄20～77岁,平均42.5岁。临床表现:颈部肿物、无明显症状或体检发现。肿块直径0.3～15 cm,平均6.5 cm。镜检:瘤细胞呈圆形、多角形或梭形,大小较一致,核圆形或椭圆形,核仁不明显,未见核分裂象。瘤细胞呈器官样、片状、巢状、梁状排列,8例瘤细胞围绕血管和纤维形成乳头状结构。间质见多少不等的淀粉样物质沉积(67/81)。免疫表型:瘤细胞Calcitonin(81/81)、Syn(80/81)、CgA(63/81)、CEA(25/38)和TTF-1(81/81)均阳性,刚果红染色阳性(67/81)。Ki-67增殖指数3%～30%。BRAF V600E基因检测1例(1/36)阳性。结论 MTC确诊主要依赖病理学形态和免疫表型,MTC预后较好,易复发和转移,手术完整切除后,应注意随访。     

30例Tg免疫反应的甲状腺髓样癌临床病理及预后分析

分析30例MTC,根据对Tg免疫反应有无分成两组:Tg阴性组12例,Tg阳性组18例进行对比观察。发现Tg的免疫表达不仅出现在瘤性滤泡上皮,同样可出现于无滤泡的MTC实质区和转移淋巴结内,与CEA、CT、NSE呈同步的免疫阳性反应。并发现在年龄、瘤体大小、术后5、10年的生存率方面Tg阴性组和Tg阳性组的比较存在明显的差异,显示统计学上的意义(P<0.05)。由此支持MTC中Tg可能来自神经内分泌肿瘤中瘤性干细胞的多方向分化的论点,并提示Tg的表达可能预示较好的预后。     

甲状腺微小髓样癌8例临床病理分析

目的探讨甲状腺微小髓样癌的临床病理特征、诊断及治疗。方法收集8例甲状腺微小髓样癌,分析其临床病理学特征并复习相关文献。结果甲状腺微小髓样癌病灶小,癌细胞起源于甲状腺滤泡旁C细胞,间质丰富,癌细胞具有多形性,核仁不清晰,核分裂象较少见,被纤维血管和(或)淀粉样物分隔。免疫组化标记Calcitonin(+)可作为诊断指标。结论甲状腺微小髓样癌的术后组织病理检查是明确诊断的金标准,甲状腺全切术配合中央区及侧区淋巴结清扫是当前患者的最优治疗方案。     

八例甲状腺髓样癌的组织学和免疫组织化学研究

Histological review and immunohistochemical studies of 8 cases of medullary carcinoma were carried out by using ABC technique. The results showed 8 calcitonin positive cases, 3 Somatostatin positive cases, 7 NSE positive cases, 5 CEA positive cases and 8 keratin positive cases. In addition, histogenesis, histological characteristics and the evaluation of immunohistochemistry in diagnosis of thyroid medullary carcinoma are discussed.     

降钙素在甲状腺髓样癌诊治中的意义及甲状腺髓样癌临床特点分析

探讨降钙素 (CT)在甲状腺髓样癌 (MCT)诊断和治疗中的意义 ,并回顾分析MCT的临床特点。以手术和病理证实的 31例MTC为对象 ,起病年龄 (4 2 8± 6 7) (2 1～ 6 7)岁 ,以年龄和性别相匹配的骨质疏松症患者为对照。血清CT和甲状旁腺素采用放射免疫法检测。血钙、磷、碱性磷酸酶采用自动生化分析仪测定。 31例患者中男性 19例 ,女性 12例 ;散发型 2 2例 (71% ) ,合并多发性内分泌腺瘤病 (MEN) 9例 (2 9% ) ;多于中青年起病。首发症状主要是逐渐长大的颈前肿物 ,临床症状为不同程度的消耗症状、腹泻、腹痛、多汗、声音改变等。合并MEN的尚有高儿茶酚胺的表现。甲状腺均触及肿大 ,质硬、活动度差 ,7例有颈淋巴结肿大。患者人均手术 (1 4± 0 7) (1～ 4 )次 ,病理以甲状腺滤泡旁细胞增生为特点 ,降钙素免疫组织化学染色均阳性 (6例 ,10 0 % )。肿瘤易于复发 (4 1 9% )和转移(5 4 8% )。术前和复发时CT水平为 (14 6 5 2 3± 1314 0 1)ng/L ,明显高于正常和骨质疏松对照组 (P <0 0 0 1) ,术后为(388 99± 374 95 )ng/L ,明显低于术前水平 (P <0 0 1) ,CT与血钙、磷无明显相关性。对于逐渐增大、质硬、伴颈淋巴结肿大的甲状腺肿物 ,应谨惕MCT的可能。血清CT是敏感而特异的MCT标志物 ,对于MCT的早期诊断、     

甲状腺混合性髓样-滤泡状细胞癌的临床病理分析

目的探讨甲状腺混合性髓样-滤泡癌（MMFTC）临床病理特征、免疫组织化学表型及诊断与鉴别诊断。方法对2例甲状腺混合性髓样-滤泡癌临床资料进行分析,免疫组化（SP法）检测CT、Tg、CgA、vimentin、Galectin-3、CD56、CD57、CK19等,并结合文献进行复习。结果肿瘤组织由胖瘦不等的梭形细胞及甲状腺滤泡构成,二者互相穿插、分割排列,并侵犯被膜血管。免疫组化CT梭形细胞弥漫（＋）,个别滤泡上皮（＋）;Tg滤泡上皮（＋）,梭形细胞灶性（＋）;CgA部分滤泡上皮（＋）,部分梭形细胞（＋）;vimentin梭形细胞及滤泡旁细胞（＋）,个别滤泡上皮细胞（＋）;Galectin-3部分梭形细胞及滤泡上皮细胞（＋）。结论MMFTC临床罕见,依据梭形细胞和甲状腺滤泡组织二者同时侵犯被膜和（或）血管,结合免疫组化表型可确诊。     

甲状腺乳头状癌合并髓样癌1例

患者女性,35岁,因发现右侧颈包块2年、颈前包块2个月余入院。2年前无明显诱因下,右侧颈耳后触及一花生米大小质硬无痛性包块,2个月前发现颈前包块并明显增大。查体:颈软,气管居中,右侧甲状腺Ⅱ度,质硬,可随吞咽上下移动,未闻及明显血管杂音,右耳下颈后三角触及3枚肿大质硬淋巴结。颈部CT示:右侧甲状腺内见一类圆形软组织密度影,密度不均匀,边界模糊,与周围结构分界欠清,向下延伸至胸廓入口处,颈部见数枚肿大的淋巴结,初步诊断为甲状腺癌伴颈部淋巴结转移。次日于颈丛麻醉下手术,术中见右甲状腺下极有5.5cm×4cm质硬包块,境界不清,与气管粘…     

小儿甲状腺髓样癌并中枢性尿崩症1例

病例报告患儿,男,7岁半,因颈前肿物三年,烦渴多饮多尿二个月于1993年3月3日入院.家长于3年前发现患孩颈前肿物,缓慢逐渐增大,无多食、多汗、易饥、性情暴躁等症状,亦无明显消瘦;二个月前见肿物较前明显增大,伴有烦渴多饮,每日饮水     

RET原癌基因与遗传性甲状腺髓样癌关系的研究进展

RET原癌基因的突变会引起 MEN2 A、MEN2 B和 FMTC。甲状腺髓样癌 (MTC)是这三种遗传性综合征共同的临床特征。在 MEN2 A、MEN2 B和 FMTC中 ,遗传性的 RET突变仅通过点突变即可导致甲状腺髓样癌的发生。目前 ,从基因的角度来研究 MTC的最新进展已使我们提高了诊断 MTC的能力 ,促进了家族检测计划的发展。本文将主要就 RET基因突变在遗传性和散发性 MTC中所起的作用以及在患有遗传性 MTC家族中确认 RET突变的方法及临床意义作一综述     

Gastric medullary carcinoma, a distinct entity associated with microsatellite instability-H, prominent intraepithelial lymphocytes and improved prognosis

AIMS: To determine the clinicopathological and molecular features of gastric medullary cancer. METHODS AND RESULTS: Clinicopathological review and microsatellite instability (MSI) analysis were carried out on 17 gastric medullary and 64 non-medullary cancers. In addition to characteristic histopathology, gastric medullary cancers had certain prominent features: (i) the average survival time was longer in medullary and low-grade non-medullary cancers than in high-grade (P = 0.004); (ii) serosal involvement was less common in medullary cancers (29.4%, 5/17) than in non-medullary cancers (9.4%, 6/64) (P < 0.05) while pushing borders were more common in medullary cancers (70.6%, 12/17 versus 17.2%, 11/64, P = 0); (iii) the presence of intraepithelial lymphocytes (IELs) in medullary and non-medullary cancers was 2380/10 high-power field (HPF) and 147/10 HPF (P = 0), respectively. Both peritumoural infiltrating lymphocytes (pTIL) and a Crohn's-like reaction were more common in medullary cancers than in non-medullary (pTIL 35.3%, 6/17 versus 3.1%, 2/64; a Crohn's-like reaction 70.6%, 12/17 versus 32.8%, 21/64; P < 0.05); (iv) medullary and high-grade non-medullary cancers were more associated with reduced ECD expression in comparison with low-grade cancers (P < 0.05); (v) higher MSI-H (Bat26+) rate was observed in medullary cancers (41.2%, 7/17) than in non-medullary (1.6%, 1/64) (P = 0). CONCLUSIONS: Gastric medullary cancer has distinct clinicopathological features and genetic alterations. Two subtypes of gastric medullary cancers, Bat26+ and Bat26-, might have prognostic implications, thus analysis of Bat26 may be of clinical value.     

Urothelial carcinoma following augmentation cystoplasty: an aggressive variant with distinct clinicopathological characteristics and molecular genetic alterations

Aims:  Patients who have undergone intestinal augmentation cystoplasty are at risk for developing latent vesicle malignancy. The aim was to evaluate the histological and immunohistochemical characteristics and molecular genetic alterations in these neoplasms.
Methods and results:  Four patients developing urothelial neoplasms after augmentation cystoplasty were included in the current study. The mean age of the patients, including two men and two women, was 37 years. The latency from bladder augmentation to developing malignancy ranged from 17 to 21 years (mean 19 years). All patients died of cancer shortly after diagnosis (mean 5 months). In the morphological evaluation, all tumours were high-grade (grade 3) invasive urothelial carcinoma comprising various architectural patterns with brisk mitoses and tumour necrosis. Three harboured glandular differentiation and the remaining one showed squamous differentiation. All cases revealed abnormal decreasing β-catenin expression. Two tumours showed nuclear expression of CDX2. On UroVysion fluorescence in situ hybridization (FISH) analysis, all tumours displayed characteristic chromosomal abnormalities. Point mutations of both FGFR3 and p53 genes were identified in one case.
Conclusions:  Urothelial carcinomas developed after augmentation cystoplasty are extremely aggressive and exhibit distinct morphological, immunohistochemical and genetic characteristics. UroVysion FISH analysis may offer a surveillance strategy in patients who undergo augmentation cystoplasty.     

胆管癌基因变异与临床病理学特征的关系

目的：了解肝内胆管癌（ICC）在发生和发展过程中基因变异特点及其与临床病理学特征的关系。方法：采用微量组织切割法,从22份ICC石蜡包埋组织块中提取基因组DNA,经PCR扩增,琼脂糖凝胶电泳分离和DNA直接测序,检测6种肿瘤抑制基因（APC,MCC,DCC,OGG1,p53和RB1）的杂合性缺失和Ki-ras-2癌基因点突变的状况,分析其与临床病理学指标的关系。结果：7 肿瘤基因变民的总检出率为86．4％（19／22）,根据变异基因类型与临床病理学持征的关系。将19例有基因变异的病例分为两组,I型患者9例,47．4％,具有APC,MCC,DCC和Ki-ras-2基因变异,平均年龄57．2岁,II型患者10例,52．6％,具有P53,OGG1和RB1基因变异,平均年龄69．1岁（P＜0．05）,I型患者的术后3年生存率为88．9％（8例）,明显高于II型患者的30．0％（3例,P＜0．05）,结论：ICC的发生和发展与多基因变异的长期蓄积和协同作用密切相关,I型基因变异（APC,MCC,DCC和Ki-ras-2)主要在ICC的启动和早期学进阶段起作用,II型基因变异P53,OGG1和RB1）则在促进ICC的晚期演进过程中发挥作用,对ICC基因变异谱系的检测有助于了解肿瘤演进状态和判断预后。     

甲状腺髓样癌原发及转移灶细针穿刺细胞学诊断

目的：提高甲状腺髓样癌（medullary thyroid carcinoma,MTC)细针穿刺（fine needle aspiration,FNA)细胞学诊断的准确性。方法：回顾分析14例组织学证实的MTC FNA标本及免疫细胞化学结果。总结10项细胞形态学特征;涂片背景、胶质、淀粉样物质,细胞数量,细胞排列结构,细胞异型性,细胞形状,有无双或多核细胞,核染色质以及有无核仁。结果：14例MTC主要细胞学特点为：（1）细胞呈浆细胞样和（或）梭形,前者更多见;（2）“盐和胡椒”样核染色质;（3）细胞轻至中度多形性,间有散在大细胞;（4）常见双核/多核细胞;（5）细胞呈散在或疏松团状排列;（6）核仁少见;（7）背景中基本无胶质,淀粉样物质并非多见,3例降钙素标记为阳性。结论：FNA可有效地术前诊断MTC,瘤细胞呈浆细胞样和（或）梭形,具神经内分泌肿瘤特点（包括“盐和胡椒”样核染色质,细胞散在分布倾向,细胞轻至中度多形性并间有少量大细胞及双核/多核细胞）,核仁和胶质少见,可作为诊断依据。淀粉样物质非诊断必须,有选择地应用免疫细胞化学可提高FNA的诊断准确性。     

Ultrastructural and electron immunohistochemical features of medullary thyroid carcinoma

Summary An ultrastructural study, both morphological and immunohistochemical, has been carried out on eight thyroglobulin-positive and nine thyroglobulin-negative medullary carcinomas of the thyroid. The morphometric analysis of granule size showed that all tumours contained cells with small granules and cells with medium size granules, whereas eight tumours had additional cells with large granules. The small granules had an electron dense core, while the medium and large sized granules were both pale-cored and dense-cored. The cells with small, medium or large secretory granules were all immunoreactive for calcitonin and CGRP. No ultrastructural differences were observed between thyroglobulin-positive and thyroglobulin-negative cases of medullary carcinoma of the thyroid.     

肺癌p53蛋白表达和基因突变与临床病理的相关研究

目的 检测肺癌中ｐ５３蛋白表达和基因突变状况及其与临床病理和预后的相关性。方法：应用免疫组织化学（ＬＳＡＢ法）和聚合酶链反应－单链构象多态性分析（ＰＣＲ－ＳＳＣＰ）二种方法。结果 检测肺鳞癌４６例，共９５例。免疫组化ｐ５３蛋白总阳性率为５０．５％（４８／９５例），肺鳞癌阳性率为５６．５％（２６／４６例）、肺腺癌为４４．７％（２２／４９例）。ＰＣＲ－ＳＳＣＰ检测ｐ５３基因突变率为４１．１％（３９／９     

Ovarian carcinomas: five distinct diseases with different origins,genetic alterations,and clinicopathological features

Malignant epithelial tumors (carcinomas) are the most common ovarian cancers and also the most lethal gynecological malignancies. Based on histopathology and molecular genetic alterations, ovarian carcinomas are divided into five main types (high-grade serous (70%), endometrioid (10%), clear cell (10%), mucinous (3%), and low-grade serous carcinomas (<5%)) that account for over 95% of cases. These types are essentially distinct diseases, as indicated by differences in epidemiological and genetic risk factors, precursor lesions, patterns of spread, and molecular events during oncogenesis, response to chemotherapy, and prognosis. For a successful specific treatment, reproducible histopathological diagnosis of the tumor cell type is critical. The five tumor types are morphologically diverse and resemble carcinomas of the uterus. Actually, recent investigations have demonstrated that a significant number of cancers, traditionally thought to be primary ovarian tumors (particularly serous, endometrioid, and clear cell carcinomas), originate in the fallopian tube and the endometrium and involve the ovary secondarily. This review summarizes recent advances in the molecular pathology which have greatly improved our understanding of the biology of ovarian carcinoma and are also relevant to patient management.