甲状腺上皮样血管肉瘤临床病理观察

目的探讨原发于甲状腺的上皮样血管肉瘤的临床病理特点及鉴别诊断要点。方法报道1例甲状腺上皮样血管肉瘤,并结合文献对其临床病理特点及鉴别诊断和预后进行讨论。结果患者为老年女性,以颈部包块伴声音嘶哑就诊。CT显示颈部巨大软组织肿块伴气管受压。组织学显示,瘤组织大部分排列成实性片状,瘤细胞核大,空泡状,含有明显的核仁,部分瘤细胞胞质空泡化,内含红细胞。除实性区域外,还可见内衬不典型内皮细胞的不规则互相吻合的血管腔。周围淋巴结内见肿瘤转移。免疫组化:瘤细胞Ckpan(AE1/AE3)、CK19、CD31、CD34和FⅧRAg(+),TG、TTF-1、calcitonin、S-100、EMA和HMB45(-)。结论原发于甲状腺的上皮样血管肉瘤是一种罕见的肿瘤,恶性程度较高,确诊主要依靠组织学和免疫组化检查,临床主要应与甲状腺未分化癌鉴别。     

伴有横纹肌样分化胃间质瘤1例的临床病理表现及相关文献复习

目的探讨伴有横纹肌样分化胃间质瘤的临床病理学特点。方法对1例伴有横纹肌分化的胃间质瘤进行组织学观察和免疫组化染色,观察其超微结构及进行基因测序分析,并复习相关文献。结果患者CT示腹腔巨大肿物。肿物位于胃浆膜面,质软,有出血,直径约15cm,肿物表面光滑,活动度差。镜下见部分细胞呈典型胃肠道间质瘤形态:细胞梭形,呈束状排列,纵横交错,少许细胞内核周有空泡形成。部分细胞呈现横纹肌样特征,片状分布,多边形、圆形,核圆形偏位、核仁清楚,胞质红染,偶见核分裂象。超微结构显示:部分肿瘤细胞胞浆内见发育不良的肌原纤维结构。免疫组化结果示:肿瘤细胞CD117、Dog-1、Desmin阳性,Myogenin浆阳性,Ki-67增殖指数2%~3%。基因测序结果显示:c-KIT基因第9,11,13,17外显子均未检测出突变。PDGFRA基因第12外显子检出同义突变(不导致氨基酸改变):NM-006206.4:c.170A>G(p.P567P);第18外显子检出错义突变:NM-006206.4:c.2525A>T(P.D842V)。结论胃肠道间质瘤是一类罕见的发生于特异性间质细胞的潜在恶性肿瘤,具有横纹肌样分化的间质瘤更为罕见,限于病例罕见,横纹肌样分化间质瘤的远期效果和预后有待进一步观察和随访。     

大脑内含有神经毡和菊形团的胚胎性肿瘤伴髓上皮瘤和间叶分化临床病理观察

目的探讨伴髓上皮瘤和间叶分化大脑内含有神经毡和菊形团的胚胎性肿瘤的临床病理特点、诊断与鉴别诊断。方法对1例发生在左侧颞顶叶的含有神经毡和菊形团的胚胎性肿瘤伴髓上皮瘤和间叶分化进行组织学、免疫组化观察,并结合文献复习。结果患儿男性,5岁。头颅CT示左侧颞顶叶肿瘤性病变伴钙化,左侧颞骨骨质破坏,考虑左颞顶叶占位。光镜下瘤组织由疏密相间区域构成,致密区域见原始小圆核或短梭形瘤细胞呈片状密集分布,核染色质深,异型性明显,见核分裂象伴多灶性坏死,可见横纹肌样细胞,部分区域见柱状上皮构成的原始神经管样结构或Homer-Wright菊形团形成伴结缔组织增生;稀疏区见分化良好的胶质神经毡样基质内有小型神经元样瘤细胞或节细胞样瘤细胞散在分布。免疫组化:原始神经管样结构基底部vimentin(+),原始瘤细胞和横纹肌样细胞nestin、INI-1和p53(+),横纹肌样细胞NF、Syn、MAP2和Cg A(+),Ki-67为局灶20%,原始神经管样结构CK和EMA(-),横纹肌样细胞SMA(-),横纹肌样细胞GFAP和NSE灶(+),小型神经元样瘤细胞S-100和Neu N(+),Olig2(-/+),β-catenin和desmin(-)。结论大脑内含有多量神经毡和真性菊形团的胚胎性肿瘤伴髓上皮瘤和间叶分化确诊依赖于影像、组织学和免疫组化,需要与髓母细胞瘤、非典型畸胎样/横纹肌样瘤、室管膜母细胞瘤和脉络丛乳头状癌鉴别。     

甲状腺胸腺样分化癌临床病理观察

目的探讨甲状腺胸腺样分化癌(CASTLE)的临床病理特征。方法对1例甲状腺GASTLE进行临床病理和免疫组化分析,并结合文献讨论其诊断与鉴别诊断。结果甲状腺CASTLE为灰白色实性分叶状肿块;镜下肿瘤由纤维组织条索状分割或呈不同大小的巢块,纤维间隔中有淋巴细胞及浆细胞浸润;肿瘤细胞呈多边形,胞界不清,泡状核,核仁突出,可发生鳞状细胞分化;免疫组化肿瘤细胞表达CD5及CD117。结论甲状腺CASTLE是一种少见的甲状腺恶性肿瘤,发生于中老年人,组织学上与胸腺癌类似,应与甲状腺未分化癌、鳞状细胞癌、转移性淋巴上皮瘤样癌等鉴别,免疫组化示CD5和CD117( )有助于诊断。     

硬化性横纹肌肉瘤临床病理观察

目的探讨硬化性横纹肌肉瘤(SRMS)的临床病理学特征、免疫表型,提高诊断及鉴别诊断水平。方法对1例硬化性横纹肌肉瘤进行组织形态学及免疫组织化学染色观察,并复习相关文献。结果患者男性,年龄28岁,肿瘤位于右侧咬肌,大小3 cm x2. 5 cm x2 cm,边界尚清,质硬。镜下见肿瘤间质显著玻璃样变性,嗜伊红染色,似骨样或软骨样基质;瘤细胞小至中等大小,圆形或梭形,胞质淡嗜酸性,核深染;呈巢状、条索状、梁状、假血管样、微腺泡样排列于粉染硬化基质中,少量球拍样、带状、疟原虫样横纹肌母细胞散在分布于瘤细胞之间,可见横纹。免疫表型:瘤细胞MyoD1弥漫强(+),Desmin局灶逗点样(+),Mygenin局灶弱(+)。结论硬化性横纹肌肉瘤是横纹肌肉瘤的一个罕见亚型,准确诊断依赖组织形态学特征和免疫组化标记。     

伴有横纹肌样特征的低分化胃腺癌临床病理观察

目的探讨伴有横纹肌样特征的低分化胃腺癌的临床病理特征、诊断与鉴别诊断,以提高对本肿瘤的认识。方法应用光镜和免疫组化染色对1例罕见的伴有横纹肌样特征的低分化胃腺癌进行观察,并复习相关文献。结果镜下伴有横纹肌样特征的肿瘤细胞弥漫片状或腺泡状排列,侵犯胃壁全层。肿瘤细胞黏附性差,呈类圆形或不规则形,胞质丰富,强嗜酸性,可见红染均质包涵体,核泡状、偏位,核仁突出,核分裂象易见。免疫组化肿瘤细胞vimentin弥漫(+),CAM5.2部分(+),Muc-5AC灶状(+),Ki-67增殖指数80%。结论伴有横纹肌样特征的低分化胃腺癌是极为罕见的高度恶性肿瘤,特征性的组织学形态及免疫组化标记有助于诊断与鉴别诊断。     

2例肠道横纹肌样未分化癌临床病理分析及文献复习

目的:探讨横纹肌样未分化癌的临床病理特征、免疫表型及鉴别诊断。方法:对2例横纹肌样未分化癌进行分析并复习相关文献,观察其组织学形态及免疫组化标记结果。结果:2例肿瘤分别位于小肠和直肠,均呈浸润性生长,2例肿瘤组织学形态相似,肿瘤细胞异型明显,核大核仁明显,胞浆丰富,嗜酸性,细胞呈上皮样或横纹肌样,细胞间黏附性差。免疫组化,两例肿瘤细胞均表达vimentin,CK,INI1和EMA。结论:横纹肌样未分化癌是一种罕见类型肿瘤,具有高度侵袭性,预后差。对该肿瘤确诊主要依靠组织学形态和免疫组化标记,需要与其他恶性肿瘤进行鉴别。     

横纹肌样脑膜瘤临床病理观察

目的 探讨横纹肌样脑膜瘤的临床病理学特征、免疫表型、超微结构和鉴别诊断要点.方法 对1例横纹肌样脑膜瘤进行组织形态学、免疫组化和超微结构观察.结果 患者男性,37岁.CT示左颞叶巨大肿物,手术发现肿物与硬脑膜相连.组织学显示,肿瘤主要由横纹肌样细胞组成,瘤细胞中等大,圆形或卵圆形,具有丰富的嗜酸性细胞质和偏位的细胞核,部分胞质内有包涵体,核仁易见;局部为梭形细胞型和合体细胞型脑膜瘤形态,与横纹肌样细胞有过渡.可见局灶性出血和坏死,核分裂易见.免疫组化显示肿瘤细胞为vimentin弥漫强（＋）,S-100（＋）,EMA和p53灶性（＋）,Ki-67阳性率为20%,actin、desmin、LCA、HMB45、GFAP、NF、AE1/AE3、CD30、CD79a、CD68、CD138、EGFR和bcl-2均（-）.超微结构显示胞质内有丰富的线粒体、溶酶体,部分细胞线粒体和溶酶体集聚在一起,在核旁形成包涵体样结构.细胞有不同程度的黏附,可见到简单连接和胞质紧密并对.结论 横纹肌样脑膜瘤是一种少见的恶性肿瘤,具有侵袭性和临床进展迅速的特点,组织形态以横纹肌样细胞为主,鉴别诊断需要依靠免疫组化和超微结构检查.     

肌间黏液瘤1例

患者女性 ,49岁。发现右大腿中段肿块 1年 ,抗感染治疗无效。查体 :右大腿中段可扪及 4cm× 3cm肿块 ,质软 ,活动欠佳。行肿块切除术。病理检查　巨检 :不整形组织 1块 ,大小 5cm×2cm× 1cm ,切面灰红、灰白色 ,实性 ,质软 ,无包膜。镜检 :瘤细胞多呈星形或小圆形。胞质呈不规则突起向四周伸出。少数细胞胞质明显 ,核小 ,深染 ,无核仁及核分裂象。瘤细胞稀疏分散在大量黏液样基质中 ,伸入周围的横纹肌和脂肪组织内 ,并见灶性血管丰富区 (图 1 ,2 )。奥辛蓝染色 (+) ,瘤细胞胞质内偶尔存在黏液染色阳性的空泡。免疫组化 :瘤细胞仅波形蛋白 (…     

EBV相关性淋巴上皮瘤样肝内胆管细胞癌的临床病理特征

目的探讨EBV相关性淋巴上皮瘤样肝内胆管细胞癌的临床病理学特点。方法通过HE、免疫组化染色、EBER原位杂交检测,对3例EBV相关性淋巴上皮瘤样肝内胆管细胞癌进行形态学观察,并分析其临床病理特征。结果 2例男性,1例女性。年龄43～67岁,平均年龄57.3岁,肿瘤大部分由分化好的腺癌组成,伴间质显著增生;其中一例伴有未分化的癌,瘤细胞界限不清,呈合体状,瘤细胞胞质丰富,核呈空泡状,核仁明显,核分裂易见。两种肿瘤成分间质内均可见大量淋巴细胞及浆细胞浸润,并浸润肿瘤细胞间。免疫组化:CK7、CK19及Villin(+),CK20、CDX2、Hepatocyte、Arginase-1、AFP、Glypican-3及CD34(-),Ki-67约20%～30%(+);原位杂交检测EBER(+)。结论 EBV相关性淋巴上皮瘤样肝内胆管细胞癌是一种少见的胆管癌亚型,其形态学独具特征,确诊需要结合形态学和免疫组化检测及EBER原位杂交检测。     

甲状腺岛状癌1例报道并文献复习

目的介绍并讨论甲状腺岛状癌的临床病理特征。方法分析1例有8年甲状腺滤泡性腺瘤病史癌变的岛状癌，结合免疫组化进行文献复习。结果岛状癌的肿瘤组织由大小一致的低分化小细胞组成，瘤细胞或细胞核呈轻度不典型性，不易找到核仁和有丝核分裂象，排列成巢状，呈小岛状结构，局灶可见大片明显的坏死，存活的肿瘤细胞围绕小血管排列成放射状，形成“血管外皮瘤样”结构。肿瘤细胞还可呈密集实体状、微滤泡状排列。岛状癌的复发率和10年生存率介于高分化的乳头状癌、滤泡性癌和未分化的间变性癌之间。结论岛状癌为甲状腺癌中的一种独立类型。其临床及病理特征介于高分化癌和未分化的间变性癌之间。     

甲状腺未分化癌靶向治疗研究进展

甲状腺未分化癌是一种非常罕见的甲状腺肿瘤,因肿瘤细胞分化差、高度侵袭性,成为甲状腺癌的主要死亡原因.虽传统手术或者放化疗等治疗方法在甲状腺分化癌方面取得令人鼓舞的效果,但其对未分化癌的治疗效果不明显.靶向治疗是近年来发展较为迅速的一种针对晚期肿瘤的治疗方法,机制是通过药物特异性结合或阻断已明确的致癌位点,在细胞分子水平...     

核素显像随访诊断甲状腺结节癌变（附19例报告）

目的：探讨核素显像在随访诊断甲状腺结节癌变中的价值。方法：回顾性分析19例经手术病理证实为甲状腺结节癌变患者的临床资料和核素显像表现。结果：19例患者，手术前核素显像表现为单发或多发的“冷”结节或“凉”结节，考虑恶变可能性大，手术病理结果显示：19例甲状腺结节恶变中，13例为甲状腺乳头状癌，4例为滤泡癌及2例未分化癌。结论：核素显像随访诊断甲状腺结节癌变具有重要的意义和价值。     

CD105在不同类型甲状腺癌组织中的表达及与淋巴结转移相关性的研究

目的研究甲状腺癌组织中CD105的表达意义及与淋巴结转移的相关性。方法SP法检测105例存档蜡块标本中CD105的表达。结果正常甲状腺组织、良性甲状腺肿瘤组织标本30例，无CD105的表达。甲状腺乳头状癌标本40例中26例阳性，滤泡状癌20例中17例阳性，未分化癌标本15例中14例阳性，上述两两比较其差异均有显著性；甲状腺乳头状癌中有淋巴结转移的标本24例中22例阳性，无淋巴结转移的标本16例中4例阳性，二者比较其差异均有显著性。结论CD105在正常甲状腺组织、良性甲状腺组织的血管内皮细胞膜上无表达，在甲状腺乳头状癌、滤泡状癌和未分化癌中依次升高。CD105可以作为预测甲状腺癌转移的参考指标。     

Medullary thyroid carcinoma and other rare types of thyroid carcinoma

Among 4 major traditional groups of thyroid carcinoma, papillary and follicular carcinomas are most common, and other forms, anaplastic and medullary carcinomas, are relatively rare. The 2003 WHO histological classification of thyroid tumor separated 7 other malignant thyroid tumors into distinct pathological entities, such as poorly differentiated, squamous cell, mucinous carcinomas, carcinoma showing thymus-like differentiation (CASTLE), etc. Although they are also extremely rare, recognition of their clinicopathologic features are very important. In this review, not only diagnostic and therapeutic strategies for the rare forms of thyroid carcinomas, specifically focussed on medullary carcinoma and CASTLE, but also their histogenetic abnormalities were discussed.     

甲状腺结节的超声常见误诊病例分析

目的总结甲状腺结节性病变误诊病例的声像图特征,探讨误诊的原因,提高对此类病变的认识,降低超声检查的误诊率。 方法选取2014年1月至2017年12月期间因超声提示甲状腺恶性肿瘤至上海交通大学附属上海市第六人民医院备行手术治疗的患者资料。所有患者均在我院复行术前甲状腺高频超声检查。选取其中61例超声诊断与病理结果或其他检查结果不符的病例。对这些病例的超声资料及病理等其他检查结果进行分析,对比超声报告中误诊的甲状腺病变类型,总结易混淆的病变在声像图上的差异,探讨诊断、鉴别诊断的思路,并提出适用于日常超声诊断的策略及方法。 结果误诊的病例包括9种病理类型：甲状旁腺病变,食管憩室,甲状腺髓样癌,甲状腺转移癌,颈部恶性肿瘤侵及甲状腺,甲状腺淋巴瘤,甲状腺滤泡癌,甲状腺腺瘤囊性变吸收后改变以及亚急性甲状腺炎。可总结为4种误诊情况：甲状腺乳头状癌（72.1%）,甲状腺腺瘤（19.7%）,甲状腺未分化癌（3.3%）以及桥本甲状腺炎（4.9%）。针对误诊病例的声像图特征,提出了相应诊断策略,包括实验室检查、超声引导下细针穿刺细胞学检查、同位素扫描等。 结论在常规甲状腺高频超声检查中,不同病变的声像图表现有交叉重叠现象,应当通过结合必要的临床资料、辅助检查手段或配合适当的超声扫查技巧进行鉴别,以减少误诊。     

Thyroid cancer review 3: management of medullary and undifferentiated thyroid cancer

This is the last of the three review articles dealing with thyroid cancer. Over 90% of thyroid cancer is of the differentiated type associated with a very good 10-year disease-free survival rate. In contrast, the rare forms of thyroid cancer which comprise medullary thyroid cancer arising from parafollicluar C cells, Hurthle cell carcinoma, anaplastic carcinoma, thyroid lymphoma and squamous cell carcinoma are typically associated with a poorer survival rate. Management is based upon small retrospective cohort studies.     

Chromophobe renal cell carcinoma with rhabdoid differentiation in an adult

Cases of adult renal cell carcinoma with rhabdoid features have been reported in the literature, usually in association with conventional clear cell and papillary tumors. Till date, only one report described chromophobe renal cell carcinoma with rhabdoid differentiation. We report a case of a 47-year-old male patient that underwent a routine medical check-up. Computer tomography scan revealed a large heterogeneous mass in the left kidney, multiple nodular changes in the lungs and multiple enlarged lymph nodes. On microscopic examination of the left nephrectomy specimen, 65 % of the kidney tumor was comprised of rhabdoid foci and the rest of the tumor showed eosinophilic areas consistent with chromophobe renal cell carcinoma. Final diagnosis was chromophobe renal cell carcinoma with rhabdoid differentiation. Behavior of these tumors in adults is more aggressive, demands more aggressive treatment and has poor prognosis. Therefore, rhabdoid differentiation should be recognized in this type of carcinoma and mentioned in the pathohistology report.     

Tumor cytotoxicity and endothelial Rac inhibition induced by TNP-470 in anaplastic thyroid cancer

Anaplastic thyroid carcinoma is an aggressive form of cancer with no treatment. Angiogenesis inhibitors, such as TNP-470, a synthetic derivative of fumagillin, have been shown to reduce tumor size and increase survival in heterotopic animal models of thyroid cancer. Our goals were to determine the effect of TNP-470 on anaplastic thyroid cancer using an orthotopic murine model, to identify the molecular pathways of TNP-470 actions on endothelial cells, and to determine the non-endothelial tumor effects of TNP-470. We injected human anaplastic thyroid carcinoma cells (DRO'90) into the thyroid glands of nude mice. Mice received TNP-470 (30 mg/kg) s.c. for 6 weeks. TNP-470 prolonged survival and reduced liver metastases. TNP-470 had direct cytotoxic effects on anaplastic thyroid carcinoma cells in vitro and in vivo. Paradoxically, TNP-470 increased vascular endothelial growth factor secretion from tumor cells in vitro and in vivo. However, there was no associated increase in tumor microvessel density. In endothelial cells, TNP-470 prevented vascular endothelial growth factor-induced endothelial permeability, intercellular gap formation, and ruffle formation by preventing Rac1 activation.     

High prevalence of mutations of the p53 gene in poorly differentiated human thyroid carcinomas.

The development and progression of thyroid tumors is signaled by phenotype-specific mutations of genes involved in growth control. Molecular events associated with undifferentiated thyroid cancer are not known. We examined normal, benign, and malignant thyroid tissue for structural abnormalities of the p53 tumor suppressor gene. Mutations were detected by single-strand conformation polymorphisms of PCR-amplified DNA, using primers bracketing the known hot spots on either exons 5, 6, 7, or 8. The prevalence of mutations was as follows: normal thyroid 0/6; follicular adenomas 0/31; papillary carcinomas 0/37; medullary carcinomas 0/2; follicular carcinomas 1/11; anaplastic carcinomas 5/6; thyroid carcinoma cell lines 3/4. Positive cases were confirmed by direct sequencing of the PCR products. All five anaplastic carcinoma tissues and the anaplastic carcinoma cell line ARO had G:C to A:T transitions leading to an Arg to His substitution at codon 273. In both tumors and cell lines, examples of heterozygous and homozygous p53 mutations were identified. The only thyroid carcinoma cell line in which p53 mutations were not detected in exons 5-8 had markedly decreased p53 mRNA levels, suggesting the presence of a structural abnormality of either p53 itself or of some factor controlling its expression. The presence of p53 mutations almost exclusively in poorly differentiated thyroid tumors and thyroid cancer cell lines suggests that inactivation of p53 may confer these neoplasms with aggressive properties, and further loss of differentiated function.