含有鳞状细胞癌成分的乳腺化生性癌临床病理特征

目的探讨含有鳞状细胞癌成分的乳腺化生性癌(metaplastic carcinoma with squamous cell component,MCSC)的临床病理特征和免疫表型特点。方法复习20例MCSC的临床病理资料,总结其巨检、组织学、免疫表型和预后特点。结果本组肿瘤中纯鳞状细胞癌(squamous cell carcinoma,SCC)6例、腺鳞癌(adenosquamous carcinoma,ASC)13例、癌肉瘤1例。全组平均发病年龄52·4岁,多表现为无痛性乳腺肿块。SCC和ASC腋淋巴结转移率分别为60%和66·7%。巨检45%病例呈囊性。镜下角化型SCC2例、棘层松解型1例、角化与棘层松解混合型2例、角化与梭形细胞混合型1例。ASC中的SCC成分均为角化型,腺癌成分则为浸润性导管癌,仅1例混有少量黏液癌。1例癌肉瘤含有鳞癌、腺癌和软骨肉瘤成分。进行免疫组化染色病例的SCC成分均呈CK34βE12、CK5/6、CK14和p63阳性,ER、PR阴性。5例腺癌成分呈CK34βE12灶性阳性,各有1例腺癌成分灶性表达CK14和CK5/6。CK8阳性见于所有腺癌成分,仅1例SCC成分呈灶性阳性。SMA在本组肿瘤的腺鳞两种成分均呈阴性。结论MCSC以ASC最为常见,眼观呈囊性是其特点之一。SCC成分的组织学类型和分化程度存在不同程度变异。CK14、p63和CK8作为一种组合可能有助于SCC的鉴别诊断。规范诊断标准的大样本研究将会提供更有价值的临床病理资料。     

低度恶性中央性骨肉瘤

目的探讨低度恶性中央性骨肉瘤的临床及病理特征。方法对2例低度恶性中央性骨肉瘤进行病理形态学观察和免疫组化分析。结果2例肿瘤均为复发病例，发病年龄分别为46岁和57岁。影像学上肿瘤为发生于骨髓腔内的体积较大、边界清楚的肿物。组织学上肿瘤由纤维及骨组织构成，细胞分化良好，核分裂象少见。免疫表型示Vim阳性，SMA局灶阳性。结论低度恶性中央性骨肉瘤是骨肉瘤的一种特殊类型，组织学和影像学检查对本病的诊断具有重要意义。     

伴神经内分泌分化的胃原发性癌肉瘤临床病理观察

目的 探讨伴神经内分泌分化的胃原发性癌肉瘤(gastric primary carcinosarcoma with neuroendocrine differentiation)的临床病理特征,诊断及鉴别诊断.方法 对1例伴神经内分泌分化的胃原发性癌肉瘤进行光镜观察、免疫组化标记和W-S染色,并复习相关文献.结果 患者因烧心、黑便40天就诊.胃镜发现胃贲门处有1巨大菜花状肿物.光镜下肿瘤主要由两种成分构成.一种为不规则腺管结构,免疫表型CK和EMA阳性,vimentin阴性,多数腺管Syn阳性,少数腺上皮细胞CgA阳性;另一种为弥漫分布的间质细胞,免疫表型为vimentin广泛阳性,actin、CK、EMA、Syn、S-100局灶性阳性,CgA阴性.瘤组织中可见局灶性软骨肉瘤、骨肉瘤及平滑肌肉瘤成分.肿瘤旁胃黏膜未查见幽门螺杆菌.结论 伴神经内分泌分化的胃原发性癌肉瘤是一种极其罕见,恶性程度较高,预后较差的肿瘤.其诊断与鉴别诊断主要依靠组织病理学及免疫组织化学.     

恶性间皮瘤的肿瘤相关成纤维细胞的特点

目的:通过观察恶性间皮瘤的肿瘤相关成纤维细胞(tumor-associated fibroblasts,TAF)的免疫表型特点,探讨其在诊断治疗中的意义.方法:收集恶性间皮瘤10例,食管癌、乳腺癌、结肠癌标本各10例,观察这些肿瘤TAF的CK,CK19,Calretinin,HBME-1,Vimentin,Sny,S-100等表达情况.结果:10例恶性间皮瘤、食管鳞状细胞癌、乳腺浸润性导管癌、结肠腺癌的TAF都阳性表达Vimentin,均灶状表达Syn,S-100.10例恶性间皮瘤的TAF阳性表达CK,CK19,Calretinin,弱阳性表达HBME-1;10例食管鳞状细胞癌、乳腺浸润性导管癌、结肠腺癌的TAFs均局灶弱阳性表达CK,Calretinin,不表达HMBE-1及CK19;恶性间皮瘤组的TAF表达Calretinin,CK19,CK,HBME-1的积分光密度值(integrated optical density,IOD)显高于其余3组(P＜0.05).结论:恶性间皮瘤的TrAF与一般常见的上皮性肿瘤的TAF免疫表型既有共性又有不同,深入了解其免疫表型及分子生物学特性对研究恶性间皮瘤的诊断及治疗有重大意义.     

肾管状囊性癌的临床病理观察

目的探讨肾管状囊性癌(tubulocystic carcinoma,TCC)的临床病理特点、组织起源及其诊断和鉴别诊断。方法对1例TCC行HE、免疫组化染色,并复习相关文献。结果患者男性,77岁,体检发现左肾占位4年。眼观:肿瘤位于肾皮质,边界清楚,实性,散在大小不等囊腔,部分呈泡状。镜检:肿瘤完全由密集的管状和大小不等的囊腔构成,小管和囊腔内衬扁平至柱状嗜酸性上皮细胞,部分细胞呈鞋钉样,核圆形至卵圆形,核仁明显,Fuhrman核分级3级,核分裂少见。免疫表型:瘤细胞呈vimentin、CKpan、CD10、P504S、CK18和CK19弥漫强阳性,EMA局部强阳性,CK7、34βE12局灶阳性,KSP-cad局灶弱阳性,PAX-2弱阳性,Ki-67增殖指数<5%。结论 TCC是一种具有独特临床病理特征的罕见肾细胞癌,组织起源目前尚存在争议。诊断时需与其他肾囊性病变鉴别。     

卵巢甲状腺肿类癌临床病理分析

目的 探讨卵巢甲状腺肿类癌(strumal carcinoid of the ovary)的临床病理特征、鉴别诊断、治疗及预后.方法 对2例卵巢甲状腺肿类癌进行临床资料分析、光镜观察及免疫组化标记,并复习相关文献.结果 2例患者均以盆腔包块为主要症状.镜下肿瘤由甲状腺滤泡及类癌两种成分构成.免疫表型:甲状腺滤泡上皮CK7、EMA、Tg、TTF-1均阳性,类癌成分Syn、CgA、Calcitonin、TTF-1均阳性.结论 卵巢甲状腺肿类癌是一种罕见的低度恶性卵巢单胚层起源的畸胎瘤,其诊断及鉴别诊断主要依靠组织病理学特征及免疫表型.     

卵巢成熟性囊性畸胎瘤合并甲状腺乳头状癌和类癌1例并文献复习

目的探讨卵巢成熟性囊性畸胎瘤合并甲状腺乳头状癌和类癌的临床病理学特征及鉴别诊断。方法采用免疫组化法对1例卵巢成熟性囊性畸胎瘤合并甲状腺乳头状癌和类癌进行检测,并复习相关文献。结果卵巢肿物表面光滑,切开囊实性,部分区域可见脂质及毛发,部分区域呈灰白、灰黄色。镜下除可见成熟性畸胎瘤成分外,还可见甲状腺乳头状癌和类癌等成分。免疫表型:甲状腺乳头状癌成分CK19、HBME-1、Galectin-3均阳性,CD56阴性;类癌成分CD56、CgA、Syn阳性。结论合并甲状腺乳头状癌和类癌的成熟性囊性畸胎瘤是一种罕见的低度恶性肿瘤,其诊断需结合组织学形态及免疫表型。     

原发性鼻咽部甲状腺样低级别乳头状腺癌3例临床病理特征

目的 探讨原发性鼻咽部甲状腺样低级别乳头状腺癌(thyroid-like low-grade nasopharyngeal papillary adenocarcinoma, TL-LGNPPA)的临床病理学特征。方法 回顾性分析3例鼻咽部TL-LGNPPA的临床病理学特征、免疫表型、分子检测及随访，行HE及免疫组化染色，并复习相关文献。结果 3例患者年龄28～45岁，肿瘤均位于鼻咽顶后壁，呈息肉样或乳头状生长，均行手术切除。镜检：肿瘤呈乳头状或管状排列，胞质嗜酸性，核圆形或卵圆形，核仁不明显，核分裂象不易见，未见坏死及神经、脉管侵犯。乳头纤维间质血管玻璃样变性，局灶砂砾体形成。例2肿瘤细胞部分呈梭形，与乳头状结构相延续，局灶可见鳞状上皮化生。免疫表型：TTF-1、CK7、CK19和vimentin均弥漫阳性，CEA局灶阳性，TG、Napsin A、S-100、GFAP和CD117均阴性。例2梭形区域TTF-1、CK7、CK19阳性，鳞化区域CK5/6、p63和p53阳性。Ki-67增殖指数为1%～2%。BRAF V600E基因突变检测：例2阳性，突变类型为Exon15 V600E,其...     

子宫内膜桑葚样化生14例临床病理分析

目的 探讨子宫内膜活检标本中桑葚样化生临床病理特征、诊断及鉴别诊断。方法 收集14例伴桑葚样化生的子宫内膜活检标本的临床资料，采用免疫组化EnVision两步法检测β-catenin、p16、SATB2、CD10、p63、ER、PR、CK7、Ki-67的表达，分析其临床病理特征并复习相关文献。结果 14例患者年龄21～54岁，其中1例子宫内膜样癌、2例子宫内膜不典型增生以及11例良性子宫内膜病变。镜下见桑葚样化生呈实性巢团状或漩涡状排列，边界清楚，细胞无异型性，核分裂象罕见，巢中央可见坏死。免疫表型：β-catenin、p16弥漫核质阳性，CD10弥漫浆膜阳性，ER散在或局灶阳性，71.4%的病例SATB2弥漫或局灶核阳性，CK7均阴性，PR、p63阴性或个别细胞阳性，Ki-67增殖指数为1%～3%。2例患者行手术治疗，8例患者行孕激素治疗，4例患者活检术后未作特殊处理，预后均良好。结论 子宫内膜桑葚样化生具有独特的组织学特征及免疫表型，预后良好，病理医师应熟悉其临床病理特征，避免误诊及过度诊断。     

伴ALK融合的甲状腺乳头状癌3例临床病理分析

目的 探讨伴ALK融合的甲状腺乳头状癌(ALK-papillary thyroid carcinoma, ALK-PTC)的临床病理学特征。方法 收集3例ALK-PTC的临床病理资料，行HE、免疫组化、FISH及二代测序，并复习相关文献。结果 3例ALK-PTC中，女性2例，男性1例，年龄38～53岁，平均年龄44岁。肿块最大径0.6～2.5 cm。镜下见肿瘤以滤泡状排列为主，局灶区呈乳头状排列。免疫表型：肿瘤细胞CK19、Galectin-3、HBME-1、TTF-1和ALK(D5F3)均阳性，TPO、CD56、BRAF、NTRK和ROS1均阴性。FISH检测示ALK基因融合均阳性。例1 RNA测序结果示HIF1A-ALK融合，例2和例3 RNA测序结果示EML4-ALK融合。结论 ALK-PTC相对少见，组织学形态及免疫表型具有特征性，其准确诊断对治疗和判断预后具有重要意义。     

Dual carcinoid/epithelial neoplasia of the appendix

We report a series of 13 lesions of the human vermiform appendix in which a carcinoid component was associated with a separate non-carcinoid epithelial component that included an adenoma-like lesion of the mucosal epithelium. We use the term dual carcinoid/epithelial neoplasia to describe this phenomenon. The carcinoid component was insular/trabecular in nine cases, tubular in one case and of goblet cell type in three. The epithelial component was a mucinous cystadenoma in four, a mucinous tumour of uncertain malignant potential in three, and a mucinous cystadenocarcinoma in six. No intermediate cell population was seen and in three cases the carcinoid and epithelial components were in different parts of the appendix, leading us to suggest that these lesions may be true 'collision' tumours in which two neoplasms have arisen in the same organ. The prognosis appears to be no worse than for either of the components alone, but conclusions regarding these lesions must be guarded on account of their rarity and the small numbers available for study.     

Different beta-catenin immunoexpression in carcinoid tumors of the appendix in comparison to other gastrointestinal carcinoid tumors

Carcinoid tumor of the appendix is an endocrine tumor that is histologically similar to, but biologically less aggressive than carcinoids arising from other parts of the gastrointestinal tract. In this study, we examined E-cadherin, beta-catenin, DCC, p53 and Ki67 immunoexpression in cases of carcinoid of the appendix and made a comparison with non-appendiceal carcinoid tumors. Nine cases of appendiceal carcinoid and 11 biopsies of carcinoid of other parts of the gastrointestinal tract, five cases of the small intestine and six of the stomach were immunohistochemically evaluated for Ki67, p53, DCC, E-cadherin and beta-catenin. Two main patterns of beta-catenin staining were observed. The first pattern was characterized as membranous and cytoplasmic, and was seen mainly in the peripheral cells of the nests. The second pattern was diffuse, predominantly membranous. Most (five of seven) appendiceal carcinoids and only three of 11 non-appendiceal cases showed the first staining pattern (p < 0.05). Immunoexpression of E-cadherin and DCC was similar in both groups. p53 and Ki-67 immunostaining revealed stronger nuclear positivity in the non-appendiceal carcinoid tumors (statistically not significant). We found a pattern of beta-catenin immunostaining in typical carcinoid tumors of the appendix that was different from the pattern seen in non-appendiceal carcinoid tumors. This alteration suggests that carcinoid of the appendix may represent a different subtype of carcinoid tumors with different immunohistochemical and biological behavior.     

Neuroendocrine tumors of the appendix

The vast majority of neuroendocrine neoplasms of the appendix are carcinoid tumors. Most are of enterochromaffin (EC) cell type, although rare examples are of L cell type. EC cell carcinoids of the appendix differ from those encountered elsewhere in the gastrointestinal system. For example, they are remarkably common given the small size of the appendix, are usually benign, occur in younger patients, and typically contain sustentacular cells that express S-100. Origin from subepithelial neuroendocrine cells could explain these characteristics. It has also been suggested that most appendiceal carcinoids are hyperplastic rather than neoplastic, although this hypothesis requires further study. Nevertheless, truly neoplastic EC cell carcinoids of the appendix undoubtedly occur, and those greater than 2 cm in diameter have a significant risk of producing distant metastases. Carcinoid syndrome is a very rare presentation. Tubular carcinoids are unusual benign neoplasms; it has been proposed that they represent L cell carcinoids with a predominant tubular pattern of growth. Goblet cell carcinoids tend not to produce a grossly visible tumor mass but diffusely infiltrate the wall. They typically exhibit tight clusters of goblet cells, usually with scattered neuroendocrine cells and sometimes with Paneth cells, sometimes surrounding a small lumen. They may behave as a low-grade malignancy. The distinction between goblet cell carcinoid and other types of tumor is of great importance because of the implications for treatment and prognosis. Frank adenocarcinoma can arise from goblet cell carcinoids, and tumors with both components are classified as mixed goblet cell carcinoid-adenocarcinoma. The carcinoma component of the latter determines their prognosis, which would be worse than for a goblet cell carcinoid alone.     

Mixed carcinoid and adenocarcinoma of the appendix: report of 4 cases with immunohistochemical studies and a review of the literature.

Four cases of mixed carcinoid and adenocarcinoma of the appendix were reported. All cases presented with a dominant cecal-appendiceal tumor mass and local metastasis. Two patients had multiple peritoneal implants mimicking primary peritoneal serous adenocarcinoma or carcinomatosis. Histopathologic features of the tumors are similar, with infiltrating microglandular and cribriform patterns of tumor nests, and variable numbers of goblet cells. A literature review of "goblet cell carcinoid" that included nonlocalized cases revealed a significant percentage (>14%) of tumor-associated death, in contrast to the classic carcinoid tumor. Immunohistochemical stains were helpful to separate these tumors from carcinoid tumors and primary peritoneal serous adenocarcinoma. Mixed carcinoid and adenocarcinomas were cytokeratin (CK)-20 positive, and negative or weakly positive for chromogranin A and synaptophysin. Carcinoid tumors were CK20 negative and diffusely positive for chromogranin A and synaptophysin. Peritoneal serous adenocarcinomas were CK20 negative. These cases were clinically aggressive, and 1 patient had multiple recurrences and responded partially to chemotherapy.     

The demonstration of a subset of carcinoid tumours of the appendix by in situ hybridization using synthetic probes to proglucagon mRNA.

Previous studies using immunohistochemistry have shown variable hormone production by carcinoid tumours of the appendix. In order to confirm the existence of a specific subset of these tumours, in situ hybridization using synthetic oligonucleotide probes to detect pre-proglucagon and pre-proinsulin mRNA was performed in formalin-fixed, paraffin-embedded material from eight tubular carcinoids, 12 insulin carcinoids, and two mucinous carcinoids. The results were correlated with standard silver and mucin stains. All tubular carcinoids but none of the insular or mucinous carcinoids contained proglucagon mRNA. Proinsulin mRNA was not detected in any of the tumours. Tubular carcinoids of the appendix constitute a definable subset of appendiceal carcinoids which have a similar distribution and prognosis to typical insular carcinoids and can be diagnosed on haematoxylin and eosin-stained sections confirmed by routine special stains. The main need for recognition is to avoid confusion with mucinous carcinoids, which have a worse prognosis and may require more aggressive treatment.     

Combined goblet cell carcinoid and mucinous cystadenoma of the appendix.

Two cases of combined goblet cell carcinoid and mucinous cystadenoma occurring in the appendix are reported. The histogenesis of the goblet cell carcinoid remains one of its most controversial aspects and the occurrence of both of these relatively uncommon tumours in the same organ may lend support to the unitary stem cell hypothesis on the origin of this tumour. Alternatively, this occurrence may represent an example of the adenoma/carcinoma sequence.     

Goblet-cell carcinoid of the colon

The authors describe a case of goblet-cell carcinoid of the appendix subject to additional immunohistological study. Goblet-cell carcinoid is a rare colonic tumor comprises the morphological signs of adenocarcinoma and carcinoid.     

Goblet cell carcinoid tumor of the appendix. Report of five cases and review of the literature.

Five cases of goblet cell carcinoid tumor of the appendix showed characteristic histologic features that justified classification of these lesions as mucinous variants of carcinoid tumor. The tumor has low-grade malignancy, and metastases are uncommon. Resemblance to mucinous adenocarcinoma of the appendix is striking, and the features that help to differentiate the two lesions are delineated.     

Multiple carcinoid tumors of the rectum: Report of two cases suggesting the origin of carcinoid tumors

Two cases of multiple carcinoid tumors of the rectum with numerous micronests of carcinoid tumors are reported. The patients were 51‐ and 58‐year‐old males. Many carcinoid tumors and numerous carcinoid micronests were found in the resected rectum; the total number of carcinoid tumors, groups of micronests, and solitary micronests was 69 in the first case and 62 in the second case. The micronests, consisting of a few to many endocrine cells, were observed in the lamina propria, muscularis mucosa, and/or submucosa. Micronests increased in number, gathered and formed carcinoid tumors, which were up to 8 mm in diameter. It was found that a nest of the carcinoid tumors in the lamina propria showed continuity with the endocrine cells of a crypt in the different carcinoid tumors in both cases. The carcinoid tumor and micronest infiltrated the nerves and ganglions in the muscularis mucosa and submucosa. Nests of the carcinoid tumors and micronests were surrounded by S‐100‐positive cells. Lymph node metastases of the carcinoid tumor were found in both cases. Rectal carcinoid tumors may originate from endocrine cells of the crypts, and multiple carcinoid tumors may occur heterogeneously.     

Myxoglobulosis of the appendix associated with a proximal carcinoid and a pseudodiverticulum

A 37-year-old woman presented with acute right lower abdominal pain. Intraoperatively, the appendix was enlarged and distended. The lumen of the appendix was tightly filled with pearl-like globules, diagnostic of myxoglobulosis, a rare variant of mucocele of the appendix. A carcinoid of 2.0 cm diameter was found in the proximal region of the appendix. The appendiceal mucosa showed hyperplastic-adenomatous changes. A pseudodiverticulum interpreted as evidence of increased intraluminal pressure was detected. The latter may be an adjunct to the proximal partial obstruction of the appendiceal lumen by the carcinoid in the development of the spheroids of myxoglobulosis.