异位错构瘤性胸腺瘤的临床病理学观察和免疫组织化学研究

目的探讨异位错构瘤性胸腺瘤的临床病理学特征、免疫学表型和组织发生。方法对2例异位错构瘤性胸腺瘤进行临床病理学分析,采用免疫组织化学LSAB法行AE1/AE3、细胞角蛋白(CK)5、CK7、CK8、CK20、上皮膜抗原(EMA)、波形蛋白、CD5、CD10、α-平滑肌肌动蛋白(SMA)、calponin、结蛋白、CD34、S-100蛋白、CD57、胶质纤维酸性蛋白(GFAP)、甲状腺转录因子(TTF)-1和CD99标记。结果两例均发生于男性,年龄分别为20岁和40岁,因发现胸骨柄上缘肿块6个月和左锁骨上肿块2个多月就诊。肿块呈球形和卵圆形,边界清楚,最大直径分别为5cm和3cm,切面呈灰白色,质地柔软。组织学上,肿瘤由梭形细胞、上皮细胞和成熟的脂肪细胞3种成分混合组成。梭形细胞成分在2例中分别占到85%和70%,形态上类似纤维母细胞,多呈束状、编织状或席纹状排列。上皮细胞成分在两例中均占10%左右,形态上以非角化性鳞状上皮为主,可呈实性的小岛屿状、类似造釉细胞瘤的条索状和扩张的囊肿样排列,部分区域显示腺样分化,可见腺管形成。上皮细胞和梭形细胞在形态上有移行。成熟的脂肪组织呈不规则性分布,在两例肿瘤中所占的比例分别为<5%和20%。免疫组织化学标记显示,上皮细胞表达AE1/AE3、CK5、CK7、CK8和EMA,梭形细胞除CK外还表达波形蛋白、CD10和CD34,并部分表达α-SMA和calponin。两种细胞成分均不表达CK20、CD5、TTF-1、结蛋白、S-100蛋白、CD57、GFAP和CD99。结论异位错构瘤性胸腺瘤是一种好发于中青年男性锁骨上和胸锁关节附近软组织内的良性肿瘤,是一种由上皮和肌上皮组成的混合性肿瘤,可能起自于His颈窦,采用鳃原基混合瘤来命名或许更为合适。     

腹腔上皮样炎性肌纤维母细胞肉瘤临床病理分析并文献复习

目的：通过对上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma, EIMS)的临床病理学特征的分析,提高对其诊断认识能力,减少误诊。方法：分析3例EIMS的临床病理特征并复习相关文献。结果：术后镜下示：肿瘤细胞弥漫分布,呈圆形、上皮样,胞浆丰富、嗜酸性、核偏位,核仁明显,部分可见梭形细胞,背景显著黏液变性伴突出的炎症细胞浸润,以中性粒细胞为主。免疫表型：肿瘤细胞表达ALK、Desmin、CD30、EMA、INI-1;部分表达：CK和SMA;不表达LCA、CD15、MyoD1、S-100、HMB-45、CD20、CD3、CD34、CD117和DOG-1。FISH检测显示3例均有ALK基因相关易位。结论：EIMS是高度恶性肿瘤,发生率比较低,细胞学形态特征、免疫组织化学特点及分子特征对本病的诊断具有重要价值。     

涎腺恶性肌上皮瘤临床病理分析

目的分析涎腺原发性恶性肌上皮瘤临床表现、形态特征和生物学行为。方法对16例涎腺恶性肌上皮瘤组织进行HE染色和免疫组织化学染色,并进行回顾性分析。免疫组织化学采用EnVision法,所用抗体包括肌动蛋白、细胞角蛋白(CK)、上皮膜抗原(EMA)、波形蛋白、S-100、结蛋白、肌特异性肌动蛋白(MSA)、平滑肌肌动蛋白(SMA)、肌红蛋白,增殖细胞核抗原、白细胞共同抗原和胶质纤维酸性蛋白。结果16例中男性6例,女性10例,年龄12～65岁,平均44岁。肿瘤主要发生于腮腺和腭部小涎腺。临床上主要表现为快速生长或生长突然加速的局部包块,可有溃疡形成、骨质破坏和神经侵犯。16例中7例为复发病例,2例有颌下或颈部淋巴结转移。大部分肿物侵犯周围涎腺组织或邻近脂肪、肌肉、骨组织,侵入程度不一。肿瘤细胞形态多样化,有透明、梭形、上皮样、浆细胞样型和混合型,有一定细胞异型性,核分裂象较多。肿瘤以透明细胞型为主,占9例。免疫组织化学示瘤细胞CK、EMA、MSA、结蛋白、S-100阳性。结论涎腺恶性肌上皮瘤为比较少见的低度恶性的肿瘤,不易发生淋巴结和远处转移但容易复发,局部软组织和骨组织易受侵犯,生物学行为不一,在组织学的基础上结合免疫组织化学等其他检查可以确诊。     

近端型上皮样肉瘤1例及文献复习

目的 探讨近端型上皮样肉瘤(proximal-type epitheliod sarcoma,PES)的临床病理特点及鉴别诊断.方法 报道1例PES患者的临床资料,并对其进行组织学观察、免疫组化(EnVision-plus法)标记及电镜观察.结果 患者女性,24岁.临床主要表现右侧大阴唇逐渐增大的无痛性肿块.组织学特征:瘤组织呈结节状生长,瘤细胞椭圆形、多边形,胞质嗜双色或嗜酸性,核空泡状,有明显核仁.常见横纹肌样细胞,未见肉芽肿样改变.免疫表型:瘤细胞vimentin、β-catenin阳性,EMA局灶阳性;而SMA、MyoD1、desmin、CD117、S-100、HMB-45、Melan A、CD99、Syn、CD57、CKAE1/AE3、CK8/18、CK19、CA125、CEA、FⅧRAg、CD31、CD34、Calretinin、 CD45和CD68阴性.电镜:细胞胞质内见较多线粒体、少量粗面内质网及溶酶体,未见黑色素小体、神经内分泌颗粒及肌丝,细胞间连接不明显,未见肿瘤间质.结论 PES为一种罕见的软组织肿瘤,与经典型ES相比,具有独特的组织学特征,并具有更强的侵袭性.     

头皮低分化皮肤血管肉瘤的临床病理分析

目的 探讨头皮低分化皮肤血管肉瘤的临床免疫病理特征.方法 分析8例头皮低分化皮肤血管肉瘤患者的临床特点,观察其组织病理形态和CD31、CD34、第八因子相关抗原、波形蛋白、细胞角蛋白(AEl/AE3和CAM5.2)、上皮细胞膜抗原(EMA)、癌胚抗原(CEA)和S-100蛋白免疫组织化学染色.结果 患者平均发病年龄为69.0岁,男:女为5:3,临床早期常在头面部出现暗红斑,后期发生浸润性暗红斑块,均伴结节,偶发溃疡.组织病理检查示真皮广泛梭形细胞局部或广泛实性浸润,常见细胞异形,未见明确血管腔样结构.梭形细胞强阳性表达CD31、第八因子相关抗原和波形蛋白,弱阳性表达CD34、AEl/AE3和CAM5.2,而EMA、CEA和S-100蛋白阴性.结论 头面部出现淤斑样浸润性斑块和结节的老年患者,应及时做组织病理检查,CD31、CD34、第八因子相关抗原和波形蛋白免疫组织化学标记有助于明确诊断.     

外耳道近端型上皮样肉瘤2例并文献复习

目的 探讨近端型上皮样肉瘤(proximal-type epithelioid sarcoma,PES)的临床病理特点、诊断及鉴别诊断.方法 报道2例外耳道PES,镜下观察组织学形态特征并结合免疫组化染色结果进行分析.结果 镜下见肿瘤由梭形或上皮样细胞组成,可见横纹肌样细胞;呈结节状排列,伴片状坏死;免疫表型:CKpan、vimentin、CD34均呈阳性,CD31、S-100、CD68、HMB-45、ALK、SMA、CK7、CK20、TTF-1、desmin、Syn、CgA均呈阴性.结论 PES是一种少见的软组织恶性肿瘤,形态复杂多样,极易误诊.需临床、组织学及免疫表型等相结合,才能作出准确的诊断.     

泛发性皮肤肌纤维瘤1例并文献复习

报道1例泛发性皮肤肌纤维瘤,并对国内外相关文献进行复习回顾.本例患者以躯干泛发暗红色丘疹、斑块、结节为主要临床表现.病理见真皮内界限清晰的结节,梭形细胞周围充盈嗜伊红染色的胶原纤维.免疫组织化学:波形蛋白(vimentin)和平滑肌肌动蛋白(smooth muscle actin,SMA)均为阳性,CD34局灶性阳性,结蛋白(Desmin)和S-100均阴性.根据临床表现,组织病理和免疫组织化学结果可诊断.     

严重急性呼吸综合征肺部病变发展过程的形态观察

目的探讨严重急性呼吸综合征(SARS)肺部病变发展过程中组织形态和免疫表型的变化.方法对4例SARS的肺标本进行病理形态和1例超微结构观察,并作VG、Masson、网状纤维、地依红、PAS和天狼猩红组织化学染色及采用链霉素抗生物素蛋白-过氧化物酶(SP)法作结蛋白、波形蛋白、细胞角蛋白(CK)、上皮膜抗原(EMA)、平滑肌肌动蛋白(SMA)、HHF-35、CD34、CD68、bel-2、第八因子相关抗原(FⅧRAg)、AE1、Ⅰ型胶原、Ⅲ型胶原免疫组织化学标记.结果依病程及病变损害程度分为3期.急性渗出期2例病程＜20 d.以急性渗出性肺泡炎、肺泡上皮细胞增生,坏变和肺透明膜形成、脱屑性肺炎为主要病变,局部有纤维母细胞增生;肺泡内渗出蛋白PAS阳性,网状纤维增多并断裂,纤维母细胞明显增多,结蛋白、波形蛋白阳性.纤维增殖期1例病程25 d.以增殖性间质肺炎为主,早期纤维化表现为机化性肺炎和血管周围机化性改变.网状纤维呈肾小球样增生,间叶细胞向肌纤维母细胞分化,结蛋白、HHF-35、SMA、波形蛋白呈强阳性;向平滑肌细胞分化结蛋白、SMA呈阳性,波形蛋白阴性;向纤维母细胞分化仅波形蛋白阳性,并见弹力纤维增多,断裂,胶原纤维增生.纤维化期1例75 d.以弥漫性纤维化,以纤维增生为主的伴蜂窝肺形成.天狼猩红染色胶原纤维显红色,波形蛋白和SMA弱阳性,Ⅰ、Ⅳ胶原纤维明显增多.各期巨噬细胞均增多,CD68阳性.结论SARS肺部病变可分为3期,随病程延长,肺纤维化进行性加重;肺泡壁原始间叶细胞,肺泡上皮细胞增生、损伤和增生的巨噬细胞在肺纤维化中起着重要作用.     

20例梭形细胞癌的病理形态及免疫组织化学特征

目的:探讨梭形细胞癌的形态学及免疫组织化学特征及鉴别诊断.方法:收集20例梭形细胞癌病例的临床及病理资料,分析其形态学特征及免疫表型特征.结果:20例梭形细胞癌中男性12例,女性8例,平均年龄62.5岁.肿瘤无器官特异性,肿瘤细胞呈梭形,多数病例异型性明显,少数病例细胞温和,异形性不明显.免疫组织化学示肿瘤细胞均表达vimentin;至少表达一种上皮标志物;CK,34pE12,CK5/6,EMA和P63阳性率较高;常表达desmin,calponin,SMA;不表达CD34,S100,HMB-45,myogenin,MyoD1,ALK.结论:梭形细胞癌是一种具有独特形态学和免疫表型的肿瘤.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

目的 探讨肾混合性上皮间质肿瘤(MEST)和成人囊性肾瘤的临床病理学特点、免疫表型和鉴别诊断.方法 通过HE和免疫组织化学染色(EnVision法)分析5例MEST和4例囊性肾瘤,并复习有关文献.结果 5例MEST均为女性,中位年龄45岁;囊性肾瘤中男性3例,女性1例,中位年龄41岁;临床表现为腰痛或血尿.大体观察:MEST界清无包膜,切面未见明显出血坏死,其中3例旱实性,1例呈囊实性,另1例呈多囊性其间为厚的纤维分隔;囊性肾瘤有包膜,切面呈多囊性,囊壁薄,无实区和坏死厌.镜下观察:MEST由不等量增生、囊性扩张的腺上皮与不同排列方式的梭形间质细胞混合组成,两种细胞成分无明显异形,2例局部衬覆子宫内膜样或输卵管样上皮;囊性肾瘤为多房囊腔组织,囊壁薄,内衬单层上皮.免疫表型:9例上皮细胞CKpan、上皮细胞膜抗原(EMA)均呈阳性表达;MEST间质梭形细胞波形蛋白(5/5)、平滑肌肌动蛋白(SMA,3/5)、结蛋白(4/5)、CD10(5/5)、ER(4/5)和PR(4/5)呈阳性表达,HMB45、CD34、CD117和S-100蛋白呈阴性;囊性肾瘤间质成分波形蛋白(4/4)、SMA(4/4)、结蛋白(1/4)阳性,ER(3/4)和PR(1/4)少量细胞阳性,CDIO、HMB45、CD34、CDll7和S-100蛋白呈阴性.结论 (1)MEST和囊性肾瘤均是少见的肾脏肿瘤,大多为良性.(2)MEST间叶细胞呈不同程度的平滑肌或肌纤维母细胞分化;2例有MaUerian管上皮分化特征.(3)MEST和成人囊性肾瘤在形态学和免疫表型上有很多相似性,可能为位于同一肿瘤谱系两端的肿瘤.     

Parachordoma is not distinguishable from axial chordoma using immunohistochemistry

Parachordoma is a rare soft tissue tumor that morphologically resembles chordoma of the axial skeleton but occurs in a peripheral site. A recent study reported immunohistochemical differences between chordoma and parachordoma. While both tumors were positive for cytokeratin (CK) 8/18 (as recognized by the antibody Cam5.2), S100 and epithelial membrane antigen (EMA), only the chordoma was positive for CK7, CK20, CK 1/5/10/14 (as recognized by the antibody 34betaE12) and carcinoembryonic antigen (CEA). It has since been suggested that tumors indistinguishable from chordoma that involve the periphery should be termed chordoma periphericum and that these tumors are distinct from parachordoma. In the current study, the clinical, radiological, pathological, immunohistochemical and ultrastructural features of a chordoma-like tumor involving the deep soft tissues of the lower leg of a 69-year-old woman are presented. Microscopically, the tumor had a pseudolobulated growth pattern and consisted of sheets, nests and cords of epithelioid cells, some with a physaliferous appearance, separated by abundant myxoid stroma. The tumor cells were positive for CK 8/18, EMA and S100, showed focal staining for CK7, and were negative for CK20, CK 1/5/10/14 and CEA. On the basis of these results a diagnosis of parachordoma was favored. For comparison, an immunohistochemical analysis of five axial chordomas was also performed. The chordomas showed positivity for CK 8/18 (5 of 5 cases), EMA (5 of 5 cases), S100 (5 of 5 cases), CK 1/5/10/14 (1 of 5 cases) and CK7 (1 of 5 cases). Stains for CK20 and CEA were negative in all five chordomas. The results of the present study suggest that the expression of antigens for CK 1/5/10/14, CK7, CK20 and CEA in chordoma might not be as common as what has been previously reported. The results also suggest that parachordoma might not be easily distinguished immunohistochemically from axial chordoma (and therefore also from so-called chordoma periphericum).     

Proximal-type epithelioid sarcoma of the vulva with INI1 diagnostic utility

Proximal epithelioid sarcoma (PES) is an extremely uncommon neoplasm of the vulva with an aggressive behavior. Recently, these authors experienced a case of proximal-type ES in a 41-year-old woman who was admitted for a rapidly growing mass in the right mons pubis. An about-1-cm-sized mass was initially noticed one and a half years earlier. The excised mass, however, was 8 cm in greatest dimension and was relatively well circumscribed. The cut surface was trabeculated, with multifocal hemorrhages and necroses. Microscopically, the tumor consisted of epithelioid rhabdoid cells with vesicular nuclei, large prominent nucleoli, and cytoplasmic eosinophilic globules comparted by thin, fibrous septae. The main differential diagnoses included PES, other sarcomas with epithelioid cells, malignant melanoma, and sarcomatoid carcinoma. The tumor cells were diffusely positive for vimentin and EMA; focally positive for cytokeratin; and negative for CK5/6, CD34, S-100 protein, desmin, and myogenin. INI1 (hSNF5/SMARCB1, a member of the SW1/SNF chromatin remodeling complex located on chromosome 22q11.2) staining clearly showed loss of expression in the tumor cells. Recent studies reported that some ESs also showed INI1 inactivation, as characteristically seen in malignant rhabdoid tumors of infancy. Reported herein is the diagnostic utility of INI-1 on PES and the possible relationship between PES and malignant rhabdoid tumor of the soft tissue, besides a collective review of the reported cases of PES of the vulva and of the current case.     

29例上皮样胃肠间质瘤的临床病理特征

目的:探讨29例上皮样胃肠间质瘤(gastrointestinal stromal tumors,GIST)的组织学形态和免疫组织化学特点以及诊断和鉴别诊断,对临床正确诊断治疗和判断预后具有十分重要的临床意义.方法:回顾性分析安阳市肿瘤医院病理科2009年月3月至2016年8月321例完整切除GIST标本,经筛查并重新阅片诊断上皮样GIST 29例.结果:29例上皮样GIST,发生胃部15例,小肠2例,肠系膜3例,直肠4例,腹腔3例,腹膜后1例,盆腔1例.恶性GIST 25例,良性4例.瘤细胞丰富,胞质嗜酸或透明,部分瘤细胞核质比高,核大小不等具有多形性,核分裂较多,可伴有多灶凝固性坏死,间质多数伴有黏液样变性.组织结构形成器官样、片状、巢状及腺泡状等.免疫组织化学CD34,DOG-1在上皮样GIST均弥漫阳性(阳性率100%),CD117阳性率(86%).结论:上皮样GIST发生部位广泛,形态多变,易误诊其他上皮样分化的肿瘤;免疫组织化学CD34,DOG-1,CD117在上皮样GIST诊断及鉴别诊断中具有重要价值;如肿物较大、细胞丰富、核分裂多、间质黏液样变性;绝大多数要考虑恶性GIST.     

Immunohistochemical profiling of cytokeratin expression by endometrial stroma sarcoma

Endometrial stromal sarcomas can be confused with several neoplasms because of their inconsistent and widely varied morphologic appearance and frequent immunohistochemical expression of a variety of antigens including cytokeratin. The resulting diagnostic challenge becomes problematic particularly in the diagnosis of metastases resulting from such tumors. Because of the sometime epithelioid appearance of the tumor cells and their expression of cytokeratin, the metastases may be misdiagnosed as poorly differentiated carcinoma. We therefore studied the profile of cytokeratin proteins expression in 17 cases of endometrial stromal sarcomas using a panel of antibodies including cytokeratin cocktail antibody (AE1/AE 3), CK5/6, CK7, CK14, CK16, Cam5.2 (CK8), CK19, CK20, and 34Ebeta12 (CK1, 5, 10, and 14). Of the 17 cases, 8 (47%) stained positive with the cytokeratin cocktail antibody (AE1/AE 3). Of the 8 cases with cytokeratin expression, 5 (63%) stained positive with CK19, and 3 of them stained positive with Cam5.2. The 3 cases that stained positive with Cam5.2 also expressed CK19. Of the 5 cases with CK19, 1 was focally positive for CK5/6, CK7, and 34Ebeta12. None of the cases expressed CK14, CK16, or CK20. These results show that CK19 is most commonly expressed cytokeratin in endometrial stromal tumors. Hence, the inclusion of CK19 in the panel of immunostains may help resolve the diagnostic confusion created by keratin expression in endometrial stromal sarcoma and may also help in the correct diagnosis of endometrial stromal sarcoma at extrauterine sites.     

乳腺上皮-肌上皮性肿瘤4例临床病理学分析

目的：探讨乳腺上皮-肌上皮性肿瘤(epithelial-myoepithelial tumor of breast)的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对4例乳腺上皮-肌上皮性肿瘤的临床特点、组织形态学及免疫组织化学结果进行分析,并复习相关文献。结果：患者：男性1例,女性3例,平均年龄51岁(27~63岁)。4例肿瘤直径1.5~3.0 cm(平均2.0 cm),无包膜,切面灰白色。显微镜下可见肿瘤由双相增生的肌上皮细胞和腺上皮细胞构成,肌上皮细胞环绕腺上皮细胞构成特征的套管结构。免疫组织化学染色,腺上皮细胞表达CK8/18、CK7,肌上皮细胞表达p63、Calponin、CK5/6。1例诊断为腺肌上皮瘤(adenomyoepithelioma,AME),3例诊断为伴有癌的腺肌上皮瘤(恶性腺肌上皮瘤, malignant adenomyoepithelioma,MAME)。结论：乳腺上皮–肌上皮性肿瘤是少见的肿瘤类型,需与导管内乳头状瘤、化生性癌等鉴别。     

骨原发性上皮样血管肉瘤2例报道并文献复习

目的提高对骨原发性上皮样血管肉瘤(epithelioid angiosarcoma,EA)的认识,避免误诊。方法对2例原发于骨的EA进行临床病理、组织学及免疫组化分析,并进行文献复习。结果 2例均为男性,为骨组织内的多灶性、溶骨性病变,组织学上肿瘤主要由实性片状排列的上皮样细胞组成,瘤细胞核大,空泡状,含有明显核仁,可见细胞内空泡和血管腔样结构形成。免疫组化标记显示瘤细胞表达CD31、FⅧRAg、CK、vimentin。分别进行单纯化疗和手术治疗。1例失访,1例2个月后死亡。结论骨原发性EA是一种具有上皮样特征的高度恶性血管源性肿瘤,必须与转移癌等鉴别,CD31、CK等血管标记物对鉴别诊断具有重要的意义。     

上皮样血管肉瘤的临床病理分析

目的 分析上皮样血管肉瘤的临床病理特点及需进行鉴别诊断的肿瘤。方法 利用HE染色,免疫组织化学抗生物素蛋白-生物素复合物法（ABC法）观察15例上皮样血管肉瘤,电镜观察其中12例,结果 15例中12例为男性,3例为女性,发生部位;腹膜后3例,四肢软组织3例,肌骨4例,甲状腺2例,眶上缘1例,胸壁软组织1例,颈部软组织1例。临床症状包括局部肿物及其引起的疼痛,消瘦,乏力。影像学检查显示肿瘤多为实性或囊实性,直径可达3-20cm。镜下观察瘤组织或弥漫成片或聚集成巢,伴有出血和多灶坏死,瘤组织内常见大小不等的不规则腔隙,衬附异型瘤细胞,单个瘤细胞或多个瘤细胞形成空腔包绕红细胞。电镜可观察到胞质内空泡,W-P小体,中间丝及细胞基底膜的存在,瘤细胞可表达CD34,CD31,Ⅷ因子相关抗原,细胞角蛋白,上皮膜抗原,血管周细胞表达平滑肌肌动蛋白,临床主要采用手术切除的治疗方法,偶尔辅以放疗。随访/2例存活,术后存活期分别为19个月,7年;12例已死亡,术后存活期最短为2周,最长38个月,平均19个月;1例失访。8例患者发生肺,骨,淋巴结和腹腔转移。结论 上皮样血管肉瘤是高度恶性肉瘤,免疫组织化学检测和电镜观察有助于确定肿瘤的内皮分化,上皮样血管肉瘤需与转移癌,上皮样血管内皮细胞瘤,恶性黑色素瘤,上皮样肉瘤等鉴别。     

炎性肌纤维母细胞肿瘤临床病理学分析

目的探讨炎性肌纤维母细胞肿瘤(inflammatory myofibroblastic tumor,IMT)的临床病理学特征、诊断与鉴别诊断要点。方法收集江苏省人民医院病理科2010年5月至2020年5月诊治的32例IMT,观察其临床及组织病理学、免疫组织化学及分子病理特点,并复习相关文献。结果患者男19例,女13例,年龄5~65岁(平均年龄37岁)。肺及纵隔10例,胃肠道、肠系膜/大网膜12例,膀胱5例,头颈部3例,小腿软组织及腹膜后各1例,其中4例为上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma,EIMS),均位于腹腔。组织学肿瘤以梭形肌纤维母细胞及纤维母细胞增生为主,间质不同程度疏松水肿黏液变至胶原化,伴多少不等的慢性及急性炎性细胞浸润;EIMS以上皮样瘤细胞为主,间质水肿黏液变,浸润炎性细胞主要为中性粒细胞。免疫组织化学:肿瘤细胞表达间变性淋巴瘤激酶(ALK,25/32,78%),除4例EIMS均为核膜阳性外,其余IMT均为胞质阳性;肿瘤细胞还表达广谱细胞角蛋白(8/19)、平滑肌肌动蛋白(24/32,75%)、结蛋白(12/32,38%),其中4例EIMS均为结蛋白强阳性。15例行ALK荧光原位杂交检测显示12例(12/15)可见分离信号,以非经典断裂信号为主。3例行二代测序显示1例小腿IMT出现CLIP2-ALK融合,2例EIMS均示RANBP2-ALK融合。随访29例,22例无瘤生存,4例复发(其中3例克唑替尼治疗并带瘤生存),3例死亡(其中2例为EIMS)。结论IMT形态学谱系广泛,需与多种良恶性肿瘤鉴别,免疫组织化学(抗体ALKp80、ALKD5F3)及荧光原位杂交检测(ALK断裂探针)可辅助IMT的诊断,不典型病例推荐二代测序检测。     

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5–44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis.     

微结节性胸腺瘤伴淋巴样间质3例临床病理观察

目的:探讨微结节性胸腺瘤伴淋巴样间质(micronodular thymoma with lymphoid stroma,MNT)的临床病理特征.方法:通过组织学和免疫组织化学方法观察3例MNT,研究其临床病理特征,并复习文献.结果:肿瘤有纤维性假包膜,肿块内见多发性散在或局部融合的上皮性结节,由丰富淋巴细胞间质分隔,其中可见淋巴滤泡形成.上皮性结节由温和的细长形或卵圆形细胞组成,核仁不明显,结节内淋巴细胞稀少.免疫组织化学:上皮性结节内上皮细胞CKpan,CK5/6,CK19,CK8/18均阳性,Ki67约2%阳性,CD20,EMA阴性;间隔内淋巴细胞CD20,CD3,CD5,CD99,TdT均灶区阳性,p53,CD1α均散在阳性;淋巴细胞背景内CK5/6,C8/18,EMA均阴性.结论:MNT是一种罕见的胸腺肿瘤,目前WHO归于交界性,有特殊的发病部位和形态学表现,组织学及免疫组织化学有助于该肿瘤的诊断和鉴别诊断.