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.     

颈部异位错构瘤性胸腺瘤1例

患者女性,61岁.因发现左侧颈前下部肿块3年入院,发病以来肿块无明显变化,既往体健,无恶性肿瘤及相关疾病家族史.专科体检:气管居中,颈前下部稍偏左侧可扪及一4 cm×3 cm大小肿块,不随吞咽移动,质软,边界尚清,活动度可,无压痛及压迫感,表面皮肤颜色正常.颈部B超:颈部胸骨上窝偏左侧软组织内可见不均质等回声,大小37 mm×24 mm,边界欠清,CDFI示内部及周边可见高阻动脉血供(图1).     

异位错构瘤性胸膜瘤

1　临床资料患者男性 ,74岁 ,右锁骨上区肿物逐渐增大 2 0余年。肿物如哑铃状 12cm× 6cm× 6cm ,边界清楚 ,活动。临床诊断 :颈部肿物待查。后转至病理科行细针吸取活检。诊断为软组织良性梭形细胞肿瘤 ,神经鞘瘤可能性大。手术完整切除肿物送检。2　病理观察2 1　眼观　手术切除肿物呈分叶状 ,10cm× 6cm× 6cm ,有完整包膜 (图 1) ;切面灰白色 ,编织状 ,质实。2 2　镜检　肿瘤有完整包膜 ,由梭形细胞束伴分化良好脂肪组织灶及淋巴细胞小灶所构成 (图 2 )。梭形细胞核长梭形 ,呈编织状 (图 3)或车辐状 (图 4 )排列。部分细胞似纤维细胞…     

胸骨异位胸腺瘤1例报告并文献复习

目的：探讨胸骨异位胸腺瘤的临床和病理特征,以提高临床诊断和治疗水平。方法和结果：复习异位胸腺瘤相关的临床和病理文献。手术治疗并经术后病理确诊胸骨异位胸腺瘤1例,术后随访30个月,无局部复发和转移。结论：异位胸腺瘤发生于胸骨非常罕见,目前国内外尚未见有文献报道;一旦确诊即应手术切除,预后取决于病理分型和分期。     

中纵隔异位化生性胸腺瘤1例并文献复习

目的探讨罕见的起源于中纵隔异位化生性胸腺瘤(metaplastic thymoma, MT)的临床病理特点。方法回顾性分析1例中纵隔原发异位MT的临床病理学及免疫表型特征等并复习相关文献。结果患者男性,45岁,CT示左侧中纵隔肿块,临床无重症肌无力表现。镜下肿瘤表现为双相分化特征,岛状的上皮细胞区域与束状的梭形细胞区域交错分布并相互移行。上皮细胞为多角形,核空泡状,核仁小而明显,可见核沟和核内假包涵体。上皮细胞有轻度核异型,上皮岛内可见穿插有嗜酸性细支状透明物质。梭形细胞呈束状或席纹状结构排列。两种区域内均有大量泡沫细胞聚集和散在成熟小淋巴细胞、浆细胞浸润。肿瘤内缺乏未成熟T淋巴细胞。免疫表型:上皮细胞区域CKpan、p63强阳性,vimentin阴性。梭形细胞区域vimentin弥漫强阳性,非常少的梭形细胞CKpan弱阳性,p63阴性。间质淋巴细胞CD3、CD5阳性,TdT和CD1a阴性。EBV原位杂交检测结果为阴性。结论中纵隔异位MT是一类临床罕见的低度恶性肿瘤,明确诊断需依靠其独特的临床病理学特征。     

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。     

甲状腺内化生型胸腺瘤1例并文献复习

目的探讨甲状腺内化生型胸腺瘤(metaplastic thymoma,MT)的临床病理学及免疫表型特征。方法复习1例MT的临床资料,进行组织病理学观察,采用免疫组化EliV ision两步法染色和EBER原位杂交检测,并结合文献进行讨论。结果患者女性,67岁,肿瘤直径4.6 cm,位于右侧甲状腺下极靠近胸骨上窝,境界清楚,包膜完整。镜下表现为双相性结构,由互相盘绕的上皮岛和短束状梭形细胞构成,两种成分在肿瘤的不同区域变化较大,部分区域由梭形细胞占优势的双相性结构构成,部分区域由纯梭形细胞构成。上皮细胞形成网格状或宽梁状吻合岛,形态类似非角化型鳞状细胞,胞核小而深染;部分上皮细胞较成熟,胞核大而空泡状,核内假包涵体易见,胞质嗜酸性。梭形细胞形态类似纤维母细胞,多排列成短束状、漩涡状、编织状或席纹状,可见胸腺小体样结构。背景有较多成熟小淋巴细胞浸润,梭形细胞区域散在较多微砂砾体。免疫表型:两种成分均弥漫表达EMA,BCL-2、CD99均弱阳性,Ki-67增殖指数约2%;上皮成分CKpan、CK5/6、p63均弥漫阳性,vimentin、CD5均阴性;梭形细胞vimentin弥漫阳性,CKpan、CK5/6、p63均阴性;间质小淋巴细胞CD3、CD5、CD20均阳性;TDT、CD1a、CD34、S-100、actin、Calretinin、D2-40等均阴性。原位杂交EBV呈阴性。结论 MT是一种极为罕见的交界性胸腺肿瘤,掌握其独特的组织学及免疫表型特征易于诊断和鉴别诊断,预后较好。     

脾脏错构瘤1例并文献复习

目的：探讨脾脏错构瘤的临床病理特征、诊断及鉴别诊断。方法对1例脾脏错构瘤行HE及免疫组化SP两步法染色,光镜观察,分析其临床病理特征并结合文献复习。结果脾脏错构瘤境界清楚,瘤组织主要由纤维组织构成,其间见巢状分布的裂隙状血窦和内含红细胞的小血管组成,内皮细胞呈胖梭形,未见坏死和核分裂象。免疫表型：内皮细胞CD8、CD31、CD34均阳性;纤维组织MSA、α-SMA、vim-entin均阳性,S-100、BCL-2、CD34均阴性;Ki-67增殖指数<1%。结论脾脏错构瘤属于罕见的良性肿瘤,应与脾脏血管瘤等鉴别,免疫组化检测有助于其鉴别诊断。     

淋巴结血管平滑肌瘤性错构瘤10例临床病理分析

目的探讨淋巴结血管平滑肌瘤性错构瘤的组织学及免疫组化特征。方法对10例淋巴结血管平滑肌瘤性错构瘤进行组织病理学及免疫组化观察,并结合文献进行讨论。结果光镜下见淋巴结部分结构保存,部分间质胶原化,内见厚壁血管及小血管增生,外周为不规则增生的平滑肌束,层次多少不等,平滑肌细胞分化良好,未见核分裂象和坏死。免疫表型显示平滑肌细胞SMA、desmin阳性,HMB45、ER、PR均阴性。结论淋巴结血管平滑肌瘤性错构瘤较为罕见,临床主要表现为腹股沟淋巴结肿大,依据组织病理学及免疫组化染色可作出明确诊断。     

异位错构瘤性胸腺瘤1例并文献复习

目的探讨异位错构瘤性胸腺瘤(ectopic hamartomatous thymoma,EHT)的临床特点、发病部位及病理学特征。方法回顾性分析1例EHT的临床资料、病理学形态、影像学特征,分析、总结EHT的复杂结构及不同细胞的免疫表型,并复习相关文献。结果 (1)EHT具有典型的临床特点:中年女性,发生于右胸骨下窝的无痛、生长缓慢的肿块。(2)具有典型的混合瘤样的组织学形态,可见上皮细胞、梭形细胞及脂肪细胞3种细胞。(3)上皮细胞岛及梭形细胞均表现出复杂的免疫表型。结论 EHT具有典型的临床病理学及免疫表型特征,镜下见混合细胞及双向分化的形态学特点可明确诊断,但仍需与头颈部的其它软组织肿瘤鉴别。     

Ectopic hamartomatous thymoma: report of a case and review of literature

Ectopic hamartomatous thymoma (EHT) is an exceedingly rare lesion that usually arises in the lower neck and mainly affects adult men. We present the clinicopathological features of a case of EHT in a 28-year-old Chinese male, together with a literature review. Ultrasound imaging and a computed tomography (CT) scan of the neck demonstrated a 3.0-cm well-defined nodule of heterogeneous density located within the left sternocleidomastoid muscle. The patient underwent a gross total resection of the tumor. Grossly, the well-demarcated, encapsulated mass had a predominantly solid and gray-white appearance admixed with microcystic foci filled with serous content and yellowish regions. The lesion consisted of an irregular admixture of spindle cells, epithelium, and mature adipose tissue. Immunohistochemistry showed that both the spindle cell and epithelial components were diffuse and had intense nuclear positivity for p63 and cytoplasmic reactivity for pan-cytokeratin, CK7, and CK19. The patient was followed for 18 months without any evidence of metastasis or recurrence.     

Ectopic hamartomatous thymoma: a case report with immunohistochemical and ultrastructural studies

A case of ectopic hamartomatous thymoma (EHT) arising in the supraclavicular region of a 52-year-old male is presented. The well-defined tumor measuring 1.7x1.5x0.7 cm consisted of three components: spindle cell (70%), epithelial (25%), and adipose (5%). The spindle cell component was characterized by sheet-like, haphazard and short fascicular arrangements of bland spindle cells. Neither mitotic figures nor cellular pleomorphism were found. Admixed with, and adjacent to, the spindle cell areas was an obviously epithelial component of variable appearance, ranging from glandular spaces lined by mainly cuboidal clear cells, irregularly anastomosing cords, and strands of epithelial cells to irregular solid nests of squamous epithelium with dark and clear cytoplasm. Myoepithelial cells were also observed. Immunohistochemically, the spindle cells were strongly and diffusely positive for cytokeratins and some of them were positive for BRST2, alpha-smooth muscle actin, and CD10. The tumor was negative for S-100 protein, glial fibrillary acidic protein, and CD34. Ultrastructurally, tonofilaments and desmosomes were observed in the spindle cells. The findings indicate an epithelial origin. The patient was well without recurrence or metastasis 8 months after excision. Pathologists and clinicians should be aware of the existence of ectopic hamartomatous thymoma in the supraclavicular or suprasternal region and should differentiate it from a high-grade sarcoma, such as biphasic synovial sarcoma or glandular malignant peripheral nerve sheath tumor.     

Ectopic hamartomatous thymoma: a case report and review of the literature

We report a case of a rare tumor of the head and neck: ectopic hamartomatous thymoma. The tumor involved the sus-clavicular area of a 48 year-old male. Ectopic hamartomatous thymoma exclusively occurs in the sus-scapular region. Histologically, it is characterized by the association of spindle-cell fascicles, epithelial solid or cystic structures and foci of mature adipose tIssue. Spindle cells express diffusely cytokeratins. The differential diagnosis of ectopic hamartomatous thymoma includes mixed tumor of salivary glands, biphasic synovial sarcoma and sarcomatoid carcinoma. Ectopic hamartomatous thymoma is a benign tumor which does not recur after complete excision.     

Ectopic hamartomatous thymoma in an immunocompromised male

Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm classically occurring in the lower neck of adult males. Here we present a case of EHT occurring in a 43-year-old immunocompromised male and a brief review of existing literature. The patient presented with a palpable mass overlying the left clavicle which, on imaging, showed a solitary nodule possibly eroding the cortical bone. A biopsy predominantly showed spindle cells that were immunopositive for keratin AE1/AE3 as well as weakly positive for CD99, SMA, and CD34. A diagnosis of synovial sarcoma was favored; at which point surgical resection was performed. The resected mass was well-demarcated with a tan-yellow cut surface. Microscopically, the lesion was composed of a mixture of spindle cells, glands, and mature adipose tissue. The spindle cells were plump with bland nuclei, and the epithelial component showed morphology similar to glands of salivary or breast tissue with a bilayered appearance (luminal and basal). No pleomorphism, mitotic figures, or necrosis was present. Immunohistochemical stains were performed and showed the spindle cells to express a myoepithelial phenotype (cytokeratin AE1/AE3, p63, calponin positive). The glands showed SMA and p63 positivity in the basal cells (similar to salivary gland and breast). Overall, given the clinical context, histomorphologic, and immunohistochemical profile, a diagnosis of EHT was made. At 12 months of follow-up there was no evidence of recurrence.     

Ectopic hamartomatous thymoma: a case study and review of the literature

Ectopic hamartomatous thymoma is a rare and distinctive tumor found in the deep soft tissues of the neck, which is characterized histopathologically by a mixture of spindle, epithelial, and adipose cell elements. We present a case of this lesion occurring in a 39-year-old male. The characteristic histochemical and immunohistochemical findings of these tumors are demonstrated. In addition, review of the reported histological, immunohistochemical and ultrastructural findings is presented together with a table of clinical findings in the tumors so far described. A brief discussion of theories of histogenesis and possible differential diagnosis is included.     

肾脏原发性淋巴瘤临床病理分析

目的 :对肾脏原发性淋巴瘤的临床病理特点、组织学起源、诊断及鉴别诊断等进行初步探讨。方法 :对 1例手术切除的肾脏原发性淋巴瘤标本做HE染色和S P免疫组化染色 ,光镜观察。结果 :左肾脏上极见一 7 5cm× 6cm× 4cm界限不清的肿块。镜下见在肾实质内有弥漫大片淋巴瘤细胞浸润。瘤细胞核呈略不规则形 ,染色质呈凝块状。免疫表型肿瘤细胞表达LCA、L2 6、IgA弥漫阳性。病理诊断为弥漫小核裂细胞型。 结论 :肾脏原发性淋巴瘤甚为罕见。结合文献 ,其主要诊断依据为 :①肾脏内有弥漫大片形态一致的淋巴瘤细胞浸润 ;②肿瘤主要位于肾脏实质内 ,肾包膜及其周围脂肪组织内亦可见瘤细胞浸润 ;③患者浅表淋巴结不肿大 ,CT检查未见胸、腹腔内有肿大的淋巴结 ;④骨髓穿刺涂片和活检未见异常细胞。⑤发现肾脏淋巴瘤至少 3月后未发现其它部位的淋巴瘤。发生于肾脏的淋巴瘤应与肾脏的肉瘤样癌、Wilm瘤、慢性炎症等相鉴别。其主要治疗方法为肾切除加化疗和 (或 )放疗。     

脊索瘤样脑膜瘤1例并文献复习

目的:探讨脊索瘤样脑膜瘤(chordoid meningiomas,CM)的临床与病理特点.方法:应用组织病理学、组织化学以及免疫组织化学方法对1例CM进行观察,同时复习相关文献进行讨论.结果:CM病理特点为黏液样基质中有呈分叶状或条索状排列的肿瘤细胞,细胞质呈淡染或嗜酸性染色,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤细胞中亦可见典型脑膜上皮细胞区域.组织化学染色阿尔辛蓝-过碘酸-Schiff反应(alcian blue and peridic acid-schiff's reacyion,AB/PAS)呈阳性反应.免疫组织化学染色显示:肿瘤细胞波形蛋白(vimentin)、上皮膜抗原(epithelial membrane antigen,EMA)表达阳性,孕激素受体(progesterone receptor,PR)灶状阳性,广谱细胞角蛋白(pan cytokeratin,CKpan)、S-100钙结合蛋白(S-100 calcium binding protein,S-100)表达阴性.结论:CM是发生于颅内的较少见的脑膜瘤亚型,需要与脊索瘤、黏液性软骨肉瘤及转移性黏液腺癌等鉴别.     

肾素瘤1例临床病理及文献复习

目的：阐述肾素瘤的病因，病理特征及鉴别诊断要点。方法：对１例肾素瘤进行大体、光交易、免疫组化镜观察，并复习有关文献。结果：肾素瘤包膜完整光下酪似血管外皮瘤；免疫线化，部分细胞Ｖｉｍｅｎｔｉｎ呈阳性反应，瘤组织的腺样及乳头样结构区瘤细胞ｃｙｔｏｋｅｒａｔｉｎ呈阳性反应。电下瘤细胞胞浆内可见多量圆形分泌颗粒及棱莆和梯形结晶样物。结论：肾素瘤是发生于变更的肾小球入球小动脉血管平滑肌细胞的罕见衣性肿瘤，明