肾黏液小管状和梭形细胞癌1例报告并文献复习

目的：探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断。方法：对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析。结果：肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚。镜下观察：肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见。免疫组化染色AE1／AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67（3％）。结论：肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义。     

肾黏液小管状和梭形细胞癌1例报告并文献复习

目的:探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断.方法:对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析.结果:肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚.镜下观察:肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见.免疫组化染色AE1/AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67(3%).结论:肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义.     

肾黏液样小管状和梭形细胞癌临床病理观察

目的:探讨肾黏液样小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)的临床病理特征、诊断、鉴别诊断及组织起源。方法:对3例肾黏液样小管状和梭形细胞癌进行临床病理学特征及免疫组化分析。结果:2例患者女性,年龄分别49岁和56岁,1例患者男性,51岁。3例患者均无临床特异症状。体检发现肾占位性病变。巨检:肿瘤大小3.5cm×3cm×2.5cm-7cm×6cm×5cm,切面灰白、浅褐色,质地均匀。镜下见瘤细胞主要呈两种结构:由单层立方细胞排列呈大小不一管状结构、缎带样及类似于平滑肌瘤的梭形细胞结构,立方状细胞及梭形细胞均形态温和。背景中见大量黏液,并可见灶状泡沫细胞、淋巴细胞浸润。免疫组化:CK、AE1/AE3、Vim、EMA阳性表达;CD10、SMA、HMB45、SYN均阴性表达。结论:MTSCC是一种少见的低级别恶性肿瘤,预后较好,可能来源于远端肾单位。     

肾脏黏液性管状和梭形细胞癌的临床病理学探讨

目的探讨肾脏黏液性管状和梭形细胞癌的临床病理学特征及诊断、鉴别诊断要点。方法对2例肾脏黏液性管状和梭形细胞癌进行临床病理学及免疫组织化学分析。结果2例肾脏黏液性管状和梭形细胞癌患者，临床症状无特异性。肿瘤与周嗣组织分界清楚。镜下肿瘤呈不规则管状结构伴黏液样间质，瘤细胞呈立方形或梭形，胞浆嗜酸，核呈低级别。无核分裂或少见，无病理性核分裂。免疫组化染色EMA、AE1／AE3、Vim均阳性表达，3413E12弱或灶区阳性，S-100、SMA、HMB45、CD15、CgA及SYN均阴性，NSE在1例中有弱的表达。结论肾脏黏液性管状和梭形细胞癌是极为罕见的，明确该肿瘤的组织起源及病理特征，对于诊断及鉴别诊断有重要意义。     

肾脏黏液样小管状和梭形细胞癌1例并文献复习

目的探讨肾脏黏液样小管状和梭形细胞癌的临床特点、病理组织学特征、鉴别诊断、治疗与预后。方法分析重庆医科大学附属江津区中心医院1例肾脏黏液样小管状和梭形细胞癌的临床表现、病理组织学特点及免疫表型, 并复习国内外相关文献。结果该患者肿瘤呈膨胀性生长, 与周围肾脏组织界限清楚, 肿瘤由管状结构、梭形细胞及黏液样的间质构成, 间质出现出血、灶性坏死、泡沫细胞聚集。肿瘤细胞核级别低, 核分裂象罕见。免疫组织化学表型:CKpan、EMA、Vimentin、CK7、CK19、P504s和CK18均为阳性;RCC、CD10、CD117、CD15、CD56、Syn、CgA和Villin均为阴性。患者术后随访1年, 无复发和转移。结论黏液样小管状和梭形细胞癌是一种罕见的低级别多形性肾上皮肿瘤, 具有独特的病理组织学特点, 预后较好。     

肾脏黏液样小管状和梭形细胞癌1例临床病理观察并文献复习

目的：探讨肾脏黏液样小管状和梭形细胞癌（mucinous tubular and spindle cell carcinoma,MTSCCa）的临床病理特征和鉴别诊断要点及生物学行为。方法：对1例肾脏黏液样小管状和梭形细胞癌进行临床病理学观察和免疫表型研究并复习相关文献。结果：组织学显示肿瘤与周围肾组织分界清楚,切面实性、灰白色,肿瘤细胞多呈分支小管状、条索状排列,瘤细胞呈立方形、短梭形或低柱状,核圆形,大小相对一致。其他组织学表现：泡沫样巨噬细胞聚集、典型的透明细胞。免疫组化：肿瘤细胞CK（pan）、CK7、EMA、S-100、Vimen—tin阳性,SMA、Desmin、HCK（3413E12）阴性。结论：MTSCCa是一种罕见的低级别多形性肿瘤,组织学谱系在不断扩大。须与其他类型肾细胞癌鉴别。     

左肾黏液样小管状和梭形细胞癌1例并文献复习

目的:探讨肾黏液样小管状和梭形细胞癌患者的临床病理特征、治疗方法、疗效评价及预后。方法:回顾性分析我们收治的1例左肾黏液样小管状和梭形细胞癌患者的临床病理资料,并复习相关文献。患者女,54岁。因左侧腰部疼痛就诊,CT检查提示左肾下极见大小约44 mm×37 mm×31 mm团块样影。结果:行左肾部分切除术,术后病理诊断:左肾黏液样小管状和梭形细胞癌。免疫组化:肿瘤细胞P-CK（+）,EMA（+）,CD68（PGM1）（-）,CK7（+）,CAM5.2（-）,PAX-8（+）,CK20（-）,Inhibin-a（-）,RCC（-）,Vimentin（+）,CD10（-）。术后患者恢复良好,康复出院,随访至今未见复发及转移。结论:肾黏液样小管状和梭形细胞癌是一类罕见的肾细胞癌,恶性程度相对较低,预后相对较好,治疗方法目前主张手术治疗,但术后仍需注意规律随访。     

肾脏黏液样小管状和梭形细胞癌2例报道及文献复习

目的探讨黏液样小管状和梭形细胞癌（MTSC）的临床病理特征、组织发生、鉴别诊断及预后。方法通过HE、免疫组织化学染色及免疫组织化学观察2例MTSC,随访39及83月,并复习相关文献。结果2例MTSC均为女性,发病年龄分别为60及73岁,肿瘤界限清楚,组织学示紧密排列的、小而狭长的小管,其间为淡染黏液样间质。瘤细胞呈立方形至梭形,嗜酸性胞浆,核级别低,核分裂相很少见。2例CK34βE12、E-cadherin、CK7、CK19、vimentin均为阳性,1例部分表达NSE及Syn,Ki-67增殖指数<5%,随访期内患者病情稳定。结论MTSC是一种罕见的低级别肾上皮性肿瘤,免疫组织化学支持MTSC起源于远端小管的假设,与乳头状肾细胞癌相似,可有神经内分泌分化。单独全肾切除术预后较好,但需密切随访。     

子宫颈孤立性纤维性肿瘤一例并文献复习

目的 探讨子宫颈孤立性纤维性肿瘤的临床病理特征、诊断、鉴别诊断、组织起源及预后.方法 观察1例子宫颈孤立性纤维性肿瘤患者的临床病理情况,并结合相关文献进行讨论.结果 该例患者肿瘤边界清楚,显微镜下肿瘤由梭形纤维母细胞样细胞排列成鱼骨样、条束状及席纹状,细胞形态较一致,部分区域细胞疏松,间质黏液样变性;部分区域细胞密集,部分区域纤维化,呈胶原化、瘢痕疙瘩样;核分裂象未见;瘤内血管丰富,管腔大小不一.免疫组织化学染色结果：Vim^＋,CD34^＋,bcl-2^＋,CD99^弱＋,PCK^-,SMA^-,Calponin^-,S-100^-,ALK^-,CD31^-,CD117^-.结论 子宫颈孤立性纤维性肿瘤是一种较罕见的子宫颈梭形细胞肿瘤,临床病理学特点、免疫组织化学染色有助于明确诊断.     

低度恶性纤维黏液样肉瘤的临床病理特征

目的：了解低度恶性纤维黏液样肉瘤的临床病理特征。方法：收集2例低度恶性纤维黏液样肉瘤的临床资料,手术切除组织光镜切片观察,另作9项免疫组织化学标记,CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68。结果：2例男女各1例,肿瘤由梭形细胞区和星形细胞黏液样区构成,1例有典型的巨形菊形团样结构。肿瘤呈浸润性生长,易复发。免疫组织化学标记瘤组织cKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68阴性,Vimentin阳性。结论：低度恶性纤维黏液样肉瘤的诊断靠病理组织学和免疫组织化学标记,手术切除为主要的治疗方法。     

Mucinous tubular and spindle cell carcinoma of kidney: a clinicopathological study

Mucinous tubular and spindle cell carcinoma is a rare tumor described in the 2004 WHO classification as a new entity with a relatively indolent behavior. We report a case of 53-year-old woman presenting a well-circumscribed tumor confined in the right kidney. The histologic findings consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumor cells were cuboidal to spindled with eosinophilic cytoplasm and lower nuclear grade. The tumor was immunoreactive for cytokeratin CK 7 and CK19, and had negative staining for CD10. The morphological and immunohistochemical features were consistent with mucinous tubular and spindle cell carcinoma of kidney. Because of the favorable prognosis with this type of tumor, mucinous tubular and spindle cell carcinoma must be differentiated from papillary renal cell carcinoma, especially the variant with sarcomatoid dedifferentiation.     

Preliminary study on clinicopathological features of renal mucinous tubular and spindle cell carcinoma北大核心CSCD

Objective: To explore the clinicopathological features and prognosis of mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney in order to improve the understanding of the tumor. Methods: The clinical and pathological information of 15 patients with MTSCC in 7 hospitals were retrospectively collected and analyzed from July 2010 to July 2018. The sections were reviewed by two high-seniority pathologists. The EnVision two-step immunohistochemical staining technique was used to detect the expressions of villin, cytokeratin 7 (CK7), epithelial membrane antigen (EMA), alpha-methylacyl-CoA racemase (AMACR), transducin-like enhancer of split 1 (TLE1), hepatocyte nuclear factor-1p (HNF-1p)and kidney specific calcium binding protein (Ksp-cadherin). The fluorescence in situ hybridization (FISH) was used to detect the synovial sarcoma translocation (SYT)-synovial sarcoma X chromosome breakpoint (SSX) fusion gene in the patients with sarcomatoid changes and positive immunohistochemical staining of TLE1. Finally, the prognostic data of all patients and the relevant literature were reviewed. Results: Among 15 patients with MTSCC, seven of the patients were male and the other eight were female, with an average age of 62 years (ranging from 48 to 75 years). The tumors were found by chance in 12 patients during physical examination, and the other 3 patients developed clinical symptoms such as frequent urine pain or hematuria, including 1 patient with a history of renal calculi for 15 years. The cut surface of tumor is firm and grey or yellow. Except for 2 cases, the majority of tumors were well-circumscribed. Microscopically, 1 case showed neoplastic necrosis, 13 cases showed a mixture of mucinous stroma, tubules and spindle cells, 1 case was mainly composed of spindle cells and mucus, and 1 case was mainly composed of tubule and mucus. Some tumors were with obvious clear cytoplasmic changes, and two cases were accompanied by sarcomatoid differentiation. The immunohistochemical results showed that the positive rates of villin, CK7, EMA, AMACR, TLE1, HNF-1 p and Ksp-cadherin were 20.0% (3/15), 80.0% (12/15), 93.3% (14/15), 80.0% (12/15), 20.0% (3/15), 20.0% (3/15), 93.3% (14/15) and 13.3% (2/15), respectively; the result of FISH excluded synovial sarcoma. Eight patients were followed up wihout other treatment after operation. Bone metastasis occurred in half a year after operation in one patient with follow-up information, while no evidence of local recurrence or distant metastases was identified in the other 7 patients until now. Conclusion: A few cases of MTSCC can metastasize and belong to malignant tumors. The positive expressions of AMACR, CK7 and villin in some cases suggests that the tumor has both proximal and distal renal tubular origins. The positive expression of HNF-ip is correlated with the histological characteristics of MTSCC clear cytoplasm. © 2019 by TUMOR. All rights reserved.     

肾脏黏液性小管状和梭形细胞癌研究进展

肾脏黏液性小管状和梭形细胞癌(mucinous tubular and spindlecell carcinoma,MTSCCa)是新确定一种罕见的低度恶性肾上皮性肿瘤。多见于青年女性,临床上无明显症状。以往常被诊断为梭形细胞(肉瘤样)肾细胞癌或不能分类的肾细胞癌。大体肿物界限清楚,切面实性、灰白色。组织学特点是肿瘤细胞排列成管状和实性梁索状漂浮于黏液性基质中,Alcianblue染色阳性。免疫组化显示复合性免疫表型。临床预后好,可复发并具有潜在远处转移的可能,应重视与其他肾脏良恶性肿瘤(后肾腺瘤、肉瘤样癌和集合管癌等)相鉴别。     

肾黏液性小管状和梭形细胞癌2例报道并文献复习

We reported the clinical and pathologic features of two different types of renal mucinous tubular and spindle cell carcinoma (MTSCC). The first patient was incidentally discovered by health examination, with lower nuclear grade, no part and distant metastasis. The second patient presented with persistence hyperpyrexia, part and distant metastasis, and high nuclear grade. Surgery were both performed successfully. The first patient had no recurrences and no distant metastases. The second patient died of multiple organ failure 3 months postoperatively. Although MTSCC is usually a low potential malignancy carcinoma, high malignancy may occur and lead to a fatal course. So it needs a proper management and prognostication.     

25例肾血管平滑肌脂肪瘤的临床病理观察

目的:探讨肾血管平滑肌脂肪瘤(RAML)的临床病理特点及诊断与鉴别诊断。方法:回顾性分析经术后病理证实的25例RAML患者的临床表现、形态特征及免疫表型特点。结果:肿瘤长径2-20cm,平均8.5cm,除去1例伴发对侧肾脂肪瘤外其余24例均为单病灶。18例有症状,其中14例腰腹胀痛,4例有血尿,7例无症状者于体检或因其他疾病影像学检查时偶然发现。病理检查经典型者24例,由血管、平滑肌和脂肪组织呈不同比例构成。术前76.2%(16/21)的患者B超提示RAML,78.3%(18/23)患者CT扫描提示RAML。术中冰冻9例,8例明确诊断RAML,1例延迟诊断,术后病理诊断为上皮样型RAML。结论:影像学检查对于RAML有较高的特征性。术前进行影像学检查,绝大多数患者可被提示或者明确诊断为RAML。病理切片辅以免疫组化检查对RAML的诊断和鉴别诊断有决定性意义。     

透明细胞乳头状肾细胞癌临床病理特征分析

目的:探讨透明细胞乳头状肾细胞癌（CCPRCC）的临床及病理学特征。方法:回顾性分析6例CCPRCC的临床特征、组织学特点及免疫表型,并复习相关文献。结果:6例肿瘤均位于肾皮质内,肿瘤边界清晰,有纤维性包膜,切面灰红或灰黄色,部分呈囊性改变。镜下肿瘤呈乳头状、管状、囊性及实性混合性生长,胞浆透明,细胞核远离基底膜,世界卫生组织（World Health Organization,WHO）/国际泌尿病理协会（International Society of Urological Pathology,ISUP)分级为1级或2级。免疫表型:6例肿瘤组织均表达CK7、PAX-8和34βE12,CAIX呈“杯状”或完全膜阳性表达。1例CD10部分阳性,其余5例CD10阴性。所有病例AMACR和TFE3均为阴性。结论:透明细胞乳头状肾细胞癌是一种少见的肾肿瘤,呈惰性生物学行为。形态上应与具有透明细胞和乳头状结构的肾细胞癌鉴别,可借助免疫组织化学和分子遗传学检测予以鉴别。     

Primary Large Cell Neuroendocrine Carcinoma of the Kidney

We report a case of a 56-year-old male with a primary large cell neuroendocrine renal carcinoma. Grossly, the left kidney was enlarged by a solid tumor that measured 145 × 125 × 100 mm. Histologically, the tumor consisted of large cells with a moderate to abundant amount of eosinophilic cytoplasm. The nuclei were irregular, some of them with finely or coarsely granular chromatin, others with vesicular chromatin and prominent nucleoli. The tumor cells showed multiple mitotic figures (up to 32 mitoses/10 HPF). In some areas, the tumor cells were arranged in solid sheets; however, the predominant pattern was solid-alveolar, trabecular and cribriform. Large areas of tumor necrosis were found. Immunohistochemically, the tumor cells were positive for synaptophysin, CD56 and CD57. Cytokeratin AE1/AE3, vimentin and CD10 were positive only focally. Chromogranin showed weak cytoplasmic positivity in rare tumor cells. Cytokeratin CAM5.2, cytokeratin 34βE12, BerEP 4, EMA, TTF-1, cytokeratin 7, cytokeratin 20, calretinin, serotonin, somatostatin, gastrin, calcitonin, glukagon and insulin were negative. Primary large cell neuroendocrine carcinoma of the kidney is a rare tumor. To the best of our knowledge, only 3 cases of a tumor of this type have been reported to date.