富于细胞性神经鞘瘤2例临床病理分析

目的 探讨富于细胞性神经鞘瘤临床病理学特点及其诊断与鉴别诊断.方法 对2例富于细胞性神经鞘瘤进行临床资料分析、组织学形态和免疫表型观察.结果 富于细胞性神经鞘瘤好发于腹膜后,临床上表现为缓慢生长的无痛性肿块.大体肿瘤呈圆形、椭圆形,包膜完整,切面实性,灰白、灰黄色.镜下肿瘤具有完整的包膜,在包膜外或包膜下可见淋巴细胞聚集灶,形成袖套样结构.免疫组化肿瘤细胞S-100、GFAP和CD57均弥漫强阳性,不表达CK(AE1/AE3)、desmin、SMA、CD34、CD117和DOG1.结论 富于细胞性神经鞘瘤是一种少见的假肉瘤性病变,大体表现、组织学特点是其诊断及鉴别诊断的主要依据,免疫组化标记有助于诊断和鉴别诊断.     

会阴部丛状神经鞘瘤临床病理分析

目的 探讨会阴部丛状神经鞘瘤(plexiform neurilem-moma,PN)临床病理特点和鉴别诊断.方法 对1例发生在会阴部的PN进行组织形态学观察、免疫组化标记并复习文献.结果 患者女,31岁,会阴部肿块渐增大4年,无疼痛.边界清楚,切面灰白,实性,呈多结节状,质地中等.镜检:肿物位于皮下,约有30个大小不等的结节组成,直径0.5～2.0cm不等,每个结节均见纤维包膜包绕,大多数结节以细胞致密区(Antoni A)为主,无或少有细胞疏松区(Antoni B).结节内瘤细胞呈长梭形,核呈锥形、仿锤形或波浪状,呈栅栏状和螺旋状排列,可见verocay小体.免疫组化标记显示:瘤细胞vimentin和S-100蛋白弥漫阳性,GFAP与GPG 9.5部分呈阳性.结节外包膜EMA、CD34阳性,瘤细胞阴性.NSE、CD57、ER、PR、CD68、α-SMA、desmin和H-Caldesmon均阴性.结论 丛状神经鞘瘤是一种具有特殊形态学特点的外周神经肿瘤,应与丛状神经纤维瘤、丛状纤维组织细胞和婴儿和儿童丛状恶性外周神经鞘膜瘤等相鉴别.     

黏液型血管瘤样纤维组织细胞瘤的临床病理学观察北大核心CSCD

目的探讨黏液型血管瘤样纤维组织细胞瘤（AFH）的临床病理学特征、诊断及鉴别诊断。方法收集2008-2017年就诊于南京医科大学第一附属医院的3例黏液型AFH患者资料,采用EnVision法检测免疫组织化学表型,采用荧光原位杂交（FISH）法检测EWSRl基因断裂重排情况。结果3例中男性2例,女性1例,年龄分别为13、31和42岁。临床表现主要为偶然发现或缓慢生长的无痛性肿块。肿瘤位于浅表软组织（皮下、黏膜下）2例,深部软组织（腹膜后）1例。大体上肿瘤最大径分别为1、7和2cm,切面灰白质实。组织学上,肿瘤均境界清楚,有纤维性包膜及淋巴细胞鞘。肿瘤呈模糊的多结节状分布,问质广泛黏液变性（≥60％）。黏液丰富区域瘤细胞相对稀疏,呈梭形或星芒状,条索状或丝网状排列,或杂乱无章的分布于间质中;瘤细胞相对丰富区域可见经典AFH的组织／树突样细胞合体样生长,排列呈小片状、短束状、交错状或局部旋涡状。瘤细胞轻．中度异型性,核分裂象可见（≤2／10HPF）。未见肿瘤性坏死。仅1例见裂隙状出血性腔隙。另有1例部分间质出现丰富的硬化性胶原。3例均表达CD68、CDl63,2例表达结蛋白、上皮细胞膜抗原和CD99,1例表达Calponin和平滑肌肌动蛋白。S-100蛋白、CD34、CD31、CD35、CD21、广谱细胞角蛋白均阴性。FISH检测示EWSRl均阳性。2例获随访结果,分别随访6个月和89个月,未见复发或转移。结论黏液型AFH的临床表现、免疫表型、分子遗传学改变及生物学行为与经典型AFH相同．但组织形态学特殊,需要与肌上皮瘤、神经源性肿瘤等富含间质黏液的肿瘤鉴别。     

嗜酸细胞型黏液表皮样癌临床病理学观察

目的探讨嗜酸细胞型黏液表皮样癌(OMEC)的临床病理学特征、免疫学表型、诊断和鉴别诊断。方法对1例OMEC行临床病理学和免疫表型分析,并复习相关文献。结果患者磁共振检查示,肿物位于右颌下腺,界限清楚,大小1 cm×1 cm。患者行全部肿瘤和右颌下腺切除术。大体见结节样肿物1枚,大小1 cm×1 cm×0.8 cm,界限清,切面主要为实性,局部可见小囊腔,灰白灰黄色,质硬,未见明显包膜。镜下肿瘤主要由嗜酸性细胞构成,嗜酸性细胞主要排列成巢状、片状结构和少许的微囊结构;局部可见中间型细胞、黏液细胞和表皮样细胞。免疫表型:嗜酸性细胞弥漫高表达CK(AE1/AE3)、EMA和p63。组织化学染色显示,嗜酸性细胞PTAH染色阳性,黏液细胞PAS染色阳性。结论 OMEC是黏液表皮样癌的罕见变异型,嗜酸性细胞弥漫高表达CK、EMA和p63。OMEC需要和嗜酸细胞瘤、嗜酸细胞癌、伴黏液化生的Warthin瘤、多形性腺瘤、嗜酸细胞性肌上皮瘤和肌上皮癌等鉴别。     

胃神经鞘瘤临床病理分析

目的：探讨胃神经鞘瘤（gastric schwannoma）的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对8例胃神经鞘瘤的临床特点、组织形态学和免疫组织化学结果进行分析,并复习相关文献。结果：患者女性5例,男性3例,平均年龄61岁（30~81岁）。8例胃神经鞘瘤直径1.0~7.0 cm（平均3.0 cm）,无包膜,切面灰白色,镜下,瘤细胞短梭形,束状、栅栏状排列,肿瘤的边缘见淋巴细胞套。免疫组织化学染色：S-100全阳性,胶质纤维酸性蛋白（glial fibrillary acidic protein,GFAP）7例阳性,CD117、CD34、Dog1、a-平滑肌肌动蛋白（a-Smooth muscle actin,a-SMA）、结蛋白（Desmin）阴性。3例发现血小板衍化生长因子受体（platelet-derived growth factor receptor,PDGFR）阳性,但未发现c-kit和PDGFRα的基因突变。结论：胃神经鞘瘤是一种良性肿瘤,需与胃肠道间质瘤鉴别。     

丛状神经鞘瘤2例临床病理分析

目的探讨丛状神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法观察2例丛状神经鞘瘤的临床表现、组织学形态及免疫表型并复习相关文献。结果 2例患者中,男女各1例,年龄分别为16岁和31岁,肿瘤均位于躯干皮肤。镜下见肿瘤在皮下呈多结节状分布,结节内瘤组织以细胞致密区(Antoni A)为主,相对缺乏细胞疏松区(Antoni B)。瘤细胞长梭形或波浪状,呈栅栏状、漩涡状排列,可见verocay小体。免疫表型:肿瘤细胞S-100及vimentin均弥漫阳性,GFAP部分阳性,EMA、CD57及Ki-67均阴性。结论丛状神经鞘瘤是一种较少见的良性周围神经鞘膜瘤,需与丛状神经纤维瘤、丛状纤维组织细胞瘤、丛状恶性外周神经鞘膜瘤等相鉴别。     

异位性错构瘤性胸腺瘤1例报道及文献复习

目的 探讨良性异位性错构瘤性胸腺瘤的临床病理特征及其鉴别诊断.方法 石蜡包埋组织连续切片分别做HE染色和免疫组化染色,光镜观察,分析1例异位性错构瘤性胸腺瘤的临床病理学特征及免疫表型特征,并进行文献复习.结果 肿块位于左颈部,生长缓慢.大体为结节状,包膜完整,切而部分囊性变.镜下显示肿瘤由梭形细胞、上皮样细胞、脂肪细胞和少量淋巴细胞组成.免疫组化染色显示:梭形细胞和上皮样细胞均弥漫性强阳性表达细胞角蛋白CK7、CK8、CK、CK5/6,不表达desmin、TTF-1、GFAP,,此外,梭形细胞阳性表达p63、Calponin、SMA、CD10、CD34,不表达细胞膜抗原EMA.而与之相反的是上皮样细胞阳性表达EMA不表达SMA、CD10、CD34.结论 异位性错构瘤性胸腺瘤是一种罕见的良性肿瘤,好发于中青年男性的颈部下方、锁骨上方及胸骨上方,需要与其它双向分化的肿瘤尤其是高级别肉瘤如滑膜肉瘤或腺样恶性外周神经鞘膜瘤鉴别,故该肿瘤的诊断及鉴别诊断尤为重要.     

骶骨神经源性肿瘤临床病理分析

目的:探讨骶骨神经源性肿瘤的临床病理学特征、诊断及鉴别诊断.方法:收集21例发生在骶骨的神经源性肿瘤,通过光镜观察及免疫组织化学分析其临床、影像学、病理学特征、免疫表型、鉴别诊断及手术预后.结果:21例中女15例,男6例,平均年龄44.9岁.临床上以骶尾部疼痛为主,影像学上表现为骶骨或骶骨及骶前肿块.神经鞘瘤17例,其中经典型神经鞘瘤8例,富于细胞神经鞘瘤9例.神经纤维瘤3例,节细胞神经瘤1例.累及骶骨的肿块,多数有不同程度的骨质破坏.免疫表型:神经鞘瘤均弥漫强阳性表达S-100蛋白,不表达NF.神经纤维瘤和节细胞神经瘤表达NF.富于细胞神经鞘瘤有4例为复发病例,平均复发时间6.5年.经典型神经鞘瘤和神经纤维瘤各有1例为复发病例.结论:骶骨神经源性肿瘤是少见肿瘤,以良性多见.各病理类型及亚型在形态学和生长方式上有一定的差异,故诊断时应明确病理类型及亚型,以供临床随访、治疗.     

浅表肢端纤维黏液瘤的临床病理特征

目的 探讨浅表肢端纤维黏液瘤(SAF)的临床病理学特点、免疫表型和鉴别诊断.方法 对1例发生于左手中指末端SAF的临床表现、组织形态和免疫学表型进行回顾性分析,并复习文献.结果 患者男,62岁.因左手中指背侧末端肿块伴疼痛就诊,曾有外伤史.术中见肿块近甲床,并深达骨膜.大体观察,肿块周界不清,直径约2 cm,切面呈灰白色,实性,质韧.镜下观察,肿瘤位于真皮层内,略呈分叶状.瘤细胞由梭形至星形纤维母细胞样细胞组成,呈杂乱状分布于黏液样基质内,局部区域可呈条束状或疏松的席纹状排列.黏液样基质内含有丰富的纤细血管,并可见较多散在的肥大细胞.瘤细胞异型性不明显或仅显示轻度的异型性,核分裂象罕见.肿瘤内也未见坏死.免疫组织化学标记显示,梭形和星形细胞表达波形蛋白、CD34和CD99,灶性表达CD10,不表达上皮细胞膜抗原、肌动蛋白、结蛋白和S-100蛋白.结论 SAF好发于成年人指趾末端.熟悉其临床病理特点则有助于与其他发生于指趾的软组织黏液性肿瘤相鉴别.临床上宜将SAF作完整性切除,以预防局部复发.     

栅栏状和Verocay小体型隆突性皮肤纤维肉瘤1例及文献复习

目的 探讨栅栏状和Verocay小体型隆突性皮肤纤维肉瘤(DFSP)的病理学特征及鉴别诊断.方法 报道1例栅栏状和Verocay小体型DFSP患者的临床资料、病理学检查和免疫表型结果.结果 眼观:带皮肤结节状肿块2个,皮面和皮内各见0.9 cm×0.7 cm×0.6 cm和0.8 cm×0.5 cm×0.4 cm肿块,切面呈灰白色,胶冻样,无包膜.镜下见普通型、黏液型和肌样型DFSP图像,尤以黏液型DFSP图像更为突出.最独特的形态学改变是部分区域亦见梭形瘤细胞核排列成栅栏状或形成Verocay小体图像.免疫组织化学:各区域瘤细胞均CD34和vimentin弥漫强阳性,S-100蛋白、neurofilament、calretinin、desmin、SMA、HMB45、MART-1、EMA、ALK-1和bcl-2均阴性.Ki-67增殖指数2%.结论 栅栏状和Verocay小体型DFSP是一种罕见而独特的DFSP亚型,提高对此亚型DFSP的认识,可避免病理诊断中的陷阱和误诊.     

Primary adrenal microcystic/reticular schwannoma: clinicopathological and immunohistochemical studies of an extremely rare case

A case of primary adrenal microcystic/reticular schwannoma affecting the right adrenal gland in a 31-year-old female was reported. Histologically, the tumor significantly consisted of strikingly anastomosing strands of spindle cells imparting a microcystic and reticular pattern with a focal transition to epithelioid nests. Immunohistochemically, S-100 and CD56 protein showed a uniform and strong positivity, GFAP and EMA weakly and focally expressed, but AE1/AE3, CK5/6, CD31, CD34, calrentin, D2-40, WT1, CgA, Melan-A and Neu-N were negative. The patient live through a calm period for 4 months after the whole right paranephros was removed with no evidence of relapse. This interesting case showed primary adrenal microcystic/reticular schwannoma is characteristic of a distinctive and infrequent morphology compared to another subtype of schwannoma involved in the paranephros. To be familiar with its significant histological features would prompt us to take it into consideration when facing the mimickers.     

Pigmented microcystic chromophobe renal cell carcinoma

We report a case of a 60-year-old female with a pigmented microcystic chromophobe renal cell carcinoma (PMCRCC). The tumor was 4.5cm in diameter, and was located in the right kidney. Grossly, on cross section, the tumor was light gray with multiple small brown to black pigmented foci up to 0.2cm in diameter. Histologically, the tumor showed a microcystic arrangement with cribriform areas and formation of adenomatous structures. The microcystic and cribriform areas were composed of larger pale cells and smaller eosinophilic cells, with cytological features of conventional chromophobe renal cell carcinoma (CRCC). The cytological features of the cells within the adenomatous structures were different. These cells were mostly columnar with nuclei at the base, and had a variable amount of pale to eosinophilic cytoplasm. There were foci of ample brown pigmentation located in the cytoplasm of the tumor cells and extracellularly. In addition, microscopic calcifications were present. Immunohistochemically, the tumor cells were positive for EMA, E-cadherin, cytokeratin CAM5.2, and cytokeratin AE1/AE3. Cytokeratin 7 was positive only focally. S-100 protein, melan A, HMB 45, vimentin, and CD117 were negative. PMCRCC is a rare tumor. To the best of our knowledge, only one series containing 20 cases of this variant of CRCC has been described to date. The important feature is that PMCRCC seems to have a relatively benign biological behavior, and distant metastases and sarcomatoid transformation are absent.     

Angiosarcoma of the mandibular gingiva

Angiosarcoma of the oral cavity is extremely rare. A 77-year-old woman consulted to our hospital because of polypoid mass of the mandibular gingival. Physical examination showed polypoid reddish mass measuring 1.5 × 1.5 × 1 cm in the mandibular gingival posterior to the front tooth. Enucleation of the tumor was performed. Grossly, the tumor was not encapsulated. Histologically, the tumor consisted of atypical spindle with hyperchromatic nuclei with nucleoli. Mitotic figures were scattered. Vasoformative channels were present in some areas. The surgical margins were positive. Immunohistochemically, the tumor cells were positive for factor VIII-related antigen, CD31, CD34, vimentin, p53 protein, but negative for pancytokeratin (AE1/3 and CAM5.2), S100 protein, α-smooth muscle antigen, and desmin. The Ki-67 labeling was 60%. A pathological diagnosis of angiosarcoma was made. Radical operation is planned now.     

睾丸微囊性间质肿瘤2例临床病理及分子特征分析

目的 观察2例睾丸微囊性间质肿瘤(microcystic stromal tumor,MCST)的临床病理学表现和分子改变.方法 回顾性分析2例睾丸MCST的临床病理学及相关分子特征,运用免疫组化法及高通量基因测序技术检测MCST中的蛋白表达及基因状态.结果 2例患者年龄分别为31岁和34岁,均偶然发现单侧睾丸肿大,无...     

Chimeric antigen receptor-modified T cells in chronic lymphoid leukemia

We designed a lentiviral vector expressing a chimeric antigen receptor with specificity for the B-cell antigen CD19, coupled with CD137 (a costimulatory receptor in T cells [4-1BB]) and CD3-zeta (a signal-transduction component of the T-cell antigen receptor) signaling domains. A low dose (approximately 1.5×10(5) cells per kilogram of body weight) of autologous chimeric antigen receptor-modified T cells reinfused into a patient with refractory chronic lymphocytic leukemia (CLL) expanded to a level that was more than 1000 times as high as the initial engraftment level in vivo, with delayed development of the tumor lysis syndrome and with complete remission. Apart from the tumor lysis syndrome, the only other grade 3/4 toxic effect related to chimeric antigen receptor T cells was lymphopenia. Engineered cells persisted at high levels for 6 months in the blood and bone marrow and continued to express the chimeric antigen receptor. A specific immune response was detected in the bone marrow, accompanied by loss of normal B cells and leukemia cells that express CD19. Remission was ongoing 10 months after treatment. Hypogammaglobulinemia was an expected chronic toxic effect.     

Microcystic/reticular schwannoma of the pancreas: a potential diagnostic pitfall

Schwannomas occurring in the pancreatic head are rare benign non-recurring mesenchymal neoplasms and are reported to show classic morphologic features. Herein we report a case of a 62 year old male presenting with a 5 cm mass in the pancreatic head encasing the portal vein and the truncus coeliacus. Preoperative fine needle aspiration revealed malignant tumour cells consistent with a moderately differentiated adenocarcinoma. A Whipple surgery was performed after palliative chemotherapy. Histological evaluation revealed a multinodular unencapsulated tumour with focal infiltration into pancreas parenchyma and a striking microcystic/reticular growth pattern. Anastomosing and intersecting strands of spindle cells with eosinophilic cytoplasm set in a myxoid partly collagenous stroma were observed. The tumour cell nuclei were round oval and tapered and showed inconspicuous small nucleoli. Degenerative nuclear atypia was seen. Mitotic activity was sparse (1/50 HPF). Pleomorphism or necrosis was absent. The tumour cells showed strong nuclear and cytoplasmic positivity for S-100 protein, and focal positivity for glial fibrillary acidic protein. The diagnosis of a microcystic/reticular schwannoma was made. The awareness of and, to some extent, the knowledge about this rare tumour are needed to achieve the correct diagnosis and to avoid confusion, especially with malignant pancreatic neoplasms.     

Prostatic stromal sarcoma: case report and review of the literature

We report a case of a 65-year-old man with rare prostatic stromal sarcoma in which suprapubital radical prostatectomy was performed, but neither chemotherapy nor radiation therapy were administered before or after the operation. The well-circumscribed tumor, measuring 5 cm in diameter, showed a homogeneous white grayish cut surface with a hard consistency. Histopathologically, the tumor consisted mainly of medium-sized rounded cells with a sarcomatous and epithelioid appearance intermingled with collagen fiber. Hyalinized foci were also noted in the tumor. Immunohistochemistry revealed that the tumor cells were diffusely positive for vimentin and focally positive for progesterone receptor and CD34, but not for EMA, cytokeratin or estrogen receptor. No recurrence or distant metastasis of the tumor has occurred in 8 years of follow up. The tumor was diagnosed as prostatic stromal sarcoma (PSS) showing epithelioid differentiation and of a progesterone-dependent nature. Possible favorable nature of the PSS might be expected after complete resection.     

Adenomatoid tumors of the female and male genital tract. A comparative clinicopathologic and immunohistochemical analysis of 47 cases emphasizing their site-specific morphologic diversity

Adenomatoid tumors (ATs) are uncommon benign mesothelial tumors with a predilection for the genital tract. We reviewed 47 ATs diagnosed at our institutions during 10-year period. Thirty tumors (64%) originated in the female (21 uterine, 8 tubal, and 1 ovarian) and 17 (36%) in the male (9 epididymal and 8 testicular) genital tract. The median age for females and males was 47.5 and 51 years, respectively. While 83% of tumors in females were incidental findings in resections for unrelated diseases, 94% of male lesions presented as clinical masses leading to surgery. The median size was 2, 1, and 0.5 cm for uterine, epididymo-testicular, and tubo-ovarian lesions, respectively. Architecturally, the microcystic/angiomatoid pattern was the most frequent (32/47; 68%), followed by combined microcystic/trabecular (26/47; 55%) and retiform/adenoid (15/47; 32%) pattern. The trabecular/solid (6%) and macrocystic (4%) patterns were uncommon. However, 57% of cases revealed ≥2 growth patterns. Taken by anatomic site, 20 of 21 uterine cases were at least focally microcystic but none was retiform. In contrast, the retiform pattern dominated in male genital tract tumors (12/17; 71%). Immunohistochemistry showed expression of calretinin (36/36) and D2-40 (30/30) and lack of CD34 (0/30) and PAX8 (0/32). GLUT-1 was expressed in 11/11 male genital tract tumors but in none of the microcystic uterine lesions. Estrogen and progesterone receptor expression was weak and focal (two and three uterine cases, respectively). None stained for the androgen receptor. Our study illustrates the great site-specific morphological diversity of ATs emphasizing their wide site-dependant differential diagnosis.