近端型上皮样肉瘤3例临床病理分析

目的探讨近端型上皮样肉瘤的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析3例近端型上皮样肉瘤的临床病理特征、免疫表型等,并复习相关文献。结果 3例均为男性,发病年龄23～83岁,分别位于左足、右侧锁骨上窝和睾丸。临床均表现为无痛性生长的结节状肿块。镜下肿瘤组织呈多结节性、巢团状或片状浸润性生长,均可见坏死。肿瘤细胞体积较大,呈圆形、卵圆形上皮样,异型性明显,染色质空泡状,可见明显核仁。部分区域,瘤细胞呈横纹肌样。免疫表型:3例瘤细胞均表达vimentin、CKpan和EMA,1例表达CD34。2例近端型上皮样肉瘤均行肿物局部切除或扩大切除,其中1例辅以化疗;1例行肿块和肠黏膜活检术。例1在3年内复发5次,发生淋巴结转移后仍存活;例2确诊后拒绝治疗,3个月后死亡,例3手术切除后随访3年无瘤生存。结论近端型上皮样肉瘤是一种较罕见的恶性肿瘤,临床表现无特异性,确诊需依赖病理形态及免疫表型。近端型上皮样肉瘤切除后局部复发率高,易发生淋巴结和肺部转移。     

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.     

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.     

脊柱上皮样肉瘤一例

患者男 ,2 5岁。 1 998年 3月无诱因出现右上肢感觉及运动障碍。核磁共振 :示颈 4～胸 1椎体骨质破坏 ,在外院两次手术刮除病灶 ,术后病理诊断均为“脊柱嗜酸性肉芽肿” ,术后症状未见明显好转且逐渐加重 ,于 1 999年 6月转入我院 ,核磁共振 :示颈 4～胸 1多处病损 ,原有病灶有继续扩大趋势 ,且腰 1椎体亦出现相似的病灶。体检 :胸腹各脏器及四肢未见肿块。术中见肿物位于椎管内硬膜外 ,由内向外生长 ,侵蚀骨质。病理检查 :送检组织 5粒 ,直径 0 .6～ 1 .5ｃｍ ,切面灰白、灰褐相间 ,有出血点和小黄点 ,质中。镜下观察 :瘤细胞排列成结节状 …     

扁桃体上皮样血管肉瘤一例

患者女,３８岁。２０天前无明显诱因感咽部不适,伴有堵塞感,１０天前自觉口内有腥臭味,痰中带血,门诊以“慢性扁桃体炎”于１９９６年４月３０日收入院。病史中无发热、鼻衄、声音嘶哑及呼吸困难,吞咽正常。体检：全身状况良好,左咽腭弓充血、左侧扁桃体Ⅲ度肿大,充血明显,表面见点状、片状溃疡及出血点,右侧扁桃体不大。行左侧扁桃体扩大切除术,术后经ＰＶＦ方案化疗一疗程,患者不能忍受化疗反应,拒绝进一步化疗出院,失访。病理检查：大体所见,灰红灰褐色椭圆形组织一块,大小为３ｃｍ×２ｃｍ×２ｃｍ,无包膜,表面见溃疡…     

乳腺癌术后上皮样血管肉瘤一例

患者女,70岁。因左上臂肿物8个月,于2005年7月15日入院。患者于1995年11月曾行左乳腺癌根治术,术后应用直线加速器放射治疗5周,化疗4个疗程。1999年春天开始左上臂肿胀,8个月前发现左上臂出现鸡蛋大肿物,无疼痛不适感,后肿物逐渐增大。体检:左上臂尺侧有一6cm×6cm×5cm 大小肿物,质韧,边界较清,表面光滑,活动度差。B 超:左上臂内侧软组织内探及一7cm×6cm×5cm 的实性低回声团块,边界清晰,内回声不均质,并可见多个小囊状液性回声区,肿块内及周边血流较丰富。术中见肿物约10cm×8cm×6cm 大小,张力大,切面呈蜂窝样,有血性液体溢出。     

上皮样血管肉瘤的临床病理学

上皮样血管肉瘤(epithelioid angiosarcoma,EAS)是近年来发现的一种恶性肿瘤,其病理学改变十分特殊,以具有明显的上皮样血管内皮细胞为特点,是血管肉瘤的一种特殊类型,最早由Enzinger和Weiss于1986年首先描述。如未充分认识其特点或诊断手段不全,常造成诊断上的困难,易误诊为原发或转移性癌,恶性黑色素瘤等,故引起了人们的关注。明确诊断对指导临床治疗及判断预     

伴脑膜上皮样旋涡的去分化脂肪肉瘤一例

患者男,56岁。因阵发性左下腹疼痛半个月,于2004年6月11日入院。体检：腹部稍丰满,可扪及包块位于左下及右上腹部,质硬,轻压痛,不活动,表面尚光滑。CT示：腹部巨大包块(畸胎瘤可能性大)。发现包块3d后进行手术,术中见包块位于小肠系膜,与周围无粘连。     

女性生殖系统上皮样肉瘤1例

<正>患者女性,53岁,绝经4年,性生活后阴道流血约10天。MRI检查提示右侧壁近内膜处病灶,肌瘤可能,未除外其他,既往无肿瘤病史。患者遂行全子宫切除+附件切除术,术后标本送病理检查。病理检查 眼观：全切子宫一个,大小9 cm×7 cm×4 cm, 子宫颈管长1.0 cm, 外口径2.0 cm, 子宫腔右侧见一灰白色质韧区域,大小2 cm×1 cm×1 cm, 肌壁厚2～4 cm, 肌壁间见结节数个,最大径0.5～3 cm, 切面灰白色,     

甲状腺上皮样血管肉瘤1例

患者女性,62岁,发现右侧颈部无痛性肿块1年余,因肿块增大、疼痛1个月余入院。查体:右侧颈部下段可触及一6 cm×4 cm大小肿块,表面光滑,边界清楚,质硬,活动度差,有压痛;心、肺、腹部未见明显异常。     

额顶部头皮内上皮样肉瘤一例

患者男,26岁。发现额顶部肿物并迅速增大3个月于2006年4月26日入院,头颅MRI示：头颅额骨、顶骨可见骨质早虫蚀样破坏,骨皮质不连续,周围可见多个软组织肿块影,内见长条形致密钙化影（图1）。术中见肿瘤位于额顶部头皮同,向下穿透颅骨及硬脑膜侵入颅内,     

近端型上皮样肉瘤的临床病理学观察

目的探讨近端型上皮样肉瘤的临床病理特点、免疫表型及诊断和鉴别诊断。方法分析了6例近端型上皮样肉瘤的临床表现、影像学、组织学及免疫组化表达特点。免疫组化染色采用SP法。所用一抗包括CK(AE1/AE3)、EMA、vimentin、desmin、S-100、CA125、β-catenin、HMB45、CD117、CD99、CD34、MSA等。结果男性5例女性1例,年龄范围32～74岁(平均56岁)。临床上表现为近期迅速增大的软组织肿块并伴有疼痛及相应的功能障碍,肿块位于深筋膜或肌肉内。CT显示为软组织肿块影,密度与肌肉相近并有明显的不均匀强化;超声检查为实性低回声肿物,肿块内可见血流信号;MRI-T1加权显示等信号,MRI-T2加权显示高信号。肿块呈多结节状,缺乏包膜,直径3～25cm(平均7cm);切面灰白色及灰红色,质地偏软呈鱼肉样,灰白色区域质地偏韧。在组织学上,肿瘤主要由明显异型的上皮样细胞组成,上皮样细胞呈中等大小,或卵圆形,核仁明显,核分裂象易见,胞质嗜酸性,丰富,细胞界限不清,少部分细胞呈梭形并与上皮样细胞间有移行,此外还有部分瘤细胞为横纹肌样细胞,表现为核偏位,核形不规则,核仁不明显,胞质丰富,嗜酸性,并有核内包涵体;肿瘤坏死明显并侵及横纹肌、骨等周围组织。随访结果:3例病人术后复发,2例患者死于广泛转移。免疫组化染色显示主要表达EMA(6/6)、vimentin(6/6)、AE1/AE3(5/6)、CD99(5/6)、β-catenin(6/6)、CD34(4/6),而HMB45、S-100、CD117、CD31等均为阴性。结论近端型上皮样肉瘤是一中度恶性肿瘤,其组织学形态主要表现为上皮样细胞和横纹肌样细胞,需与具有类似细胞形态的多种肿瘤鉴别;结合免疫表型,对诊断很有帮助。     

Primary adrenal gland epithelioid sarcoma: A case report and literature review

Epithelioid sarcoma is a malignant mesenchymal neoplasm with morphologic and immunophenotypic epithelioid differentiation, which rarely arises in solid organs. We report a case of primary epithelioid sarcoma in the adrenal gland of a 31‐year‐old female. The patient initially presented with nausea and rectal bleeding, and subsequent imaging studies revealed a 4.4 cm left adrenal gland mass and left retroperitoneal lymphadenopathy. Clinical and radiological studies did not reveal tumor elsewhere in the patient. Histologic features were those of epithelioid sarcoma, proximal type with cohesive clusters of epithelioid tumor cells harboring frequent mitoses, and areas of necrosis. Immunohistochemical stains showed strong, diffuse expression of epithelial markers (pancytokeratin), and CD34 and Fli‐1. Partial and focal positive staining of CK7 was also noted. Nuclear expression of SMARCB1 (INI‐1) protein was lost. ERG was negative in this case. We believe that this is the second‐case report of a primary adrenal gland epithelioid sarcoma. Fli‐1 positivity was seen in our case, and ERG was negative as shown in some recent publications regarding epithelioid sarcomas.     

肝脏上皮样血管内皮瘤一例

患者男,57岁。因上腹部不适6个月,于2004年3月15日入院。彩色超声波检查:肝右叶膈顶部2cm×2cm低回声光团,界清;彩色多普勒:其内未见明显血流,PD测及少量静脉频谱,诊断为实质占位,良性病变可能;CT:病灶低密度,界限稍模糊,动态增强后未见明显强化,门脉期边缘可见轻度强化,周围见轻度水肿带,印象为占位,炎性假瘤可能性大;MRI:类圆形异常信号,T1WI为低信号,界尚清,T2WI中心明显高信号,周边略高信号的靶征,增强后动脉期无明显强化,门脉期及延迟期呈周边明显强化,印象为异常信号灶,考虑为慢性肝脓肿(图1)。手术见肝脏周围轻度粘连,病变位于…     

Pitfalls in the diagnosis of epithelioid sarcoma presenting in an unusual site: a case report

The cytologic appearance of epithelioid sarcoma in fine-needle aspiration biopsy cytology (FNABC) smears has not been extensively described. We report a case of epithelioid sarcoma in a 55-year-old male who presented with nodular swellings in the abdominal wall and scrotum. The scrotal swelling was subsequently demonstrated to arise from the vas deferens. We highlight the cytomorphologic, immunocytochemical, and histopathologic features of this rare lesion in an unusual site, with particular emphasis on the dilemma encountered in the cytodiagnosis of this lesion.     

肺上皮样血管内皮瘤一例

患者女,50岁.因间断性胸背部疼痛,胸闷气短3年,加重2个月,咳嗽、咯血1周,于2007年3月入院.在当地医院查CT示:右肺下叶后基底段结节样高密度影,性质待查;陈旧性肺结核;右侧少量胸腔积液;两侧胸膜粘连、肥厚;肝脏多发钙化斑.     

Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity

Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.     

子宫上皮样滋养细胞肿瘤伴腹壁转移一例

患者女,41岁。21岁结婚,孕6产0。因阴道不规则出血8d于2003年10月入院。体检：子宫增大如孕50d大小,血β-绒毛膜促性腺激素（β-HCG）在410．8～630．0U／L之间波动,血CA125、癌胚抗原、甲胎蛋白等均在正常范围。B超示子宫后壁一2．5cm×2．6cm×2．3cm中等回声团块,光点分布不均匀,边界清,血流较丰富。行2次诊刮,刮出物为宫内膜组织,未见肿瘤组织。[第一段]     

Fine‐needle‐aspiration cytology of a proximal type epithelioid sarcoma: A case report

Epithelioid sarcoma is a rare mesenchymal neoplasm, with an as yet unidentified cell of origin. Two subtypes of epithelioid sarcoma, distal/classic and proximal/large cell type, are recognized in the literature; with the proximal‐type having a lower incidence amongst the two. Here, we present a case of proximal‐type epithelioid sarcoma in a previously healthy young man. Fine‐needle‐aspiration of a large axillary mass was performed for diagnosis. The cytologic findings included a dispersed population of large epithelioid to polyhedral cells with abundant cytoplasm. Immunohistochemical staining showed coexpression of keratin and vimentin, as well as loss of INI1 staining, consistent with an epithelioid sarcoma, proximal subtype. Diagn. Cytopathol. 2015;43:859–862. © 2015 Wiley Periodicals, Inc.     

Myxoid epithelioid sarcoma: a diagnostic challenge. A report on six cases

Flucke U, Hulsebos T J M, van Krieken J H J M & Mentzel T
(2010) Histopathology 57 , 753–759
Myxoid epithelioid sarcoma: a diagnostic challenge. A report on six cases Aims: Epithelioid sarcoma (ES) is a distinct sarcoma‐type with a specific morphology and immunophenotype. Whereas focal myxoid change does occur, to our knowledge only two cases of ES with diffuse myxoid stroma have been reported previously. To characterize more clearly the myxoid variant of ES, we describe six additional cases and discuss the differential diagnoses. Methods and results: Cases were retrieved from the authors’ files and studied histologically, immunohistochemically and by molecular methods. The age of the patients, four females and two males, ranged from 16 to 74 years (median: 33 years). The neoplasms arose in an extremity (two cases), the abdominal wall, groin, perineum and shoulder (one case each). Histologically, four cases were of the conventional type, and two were of the proximal type and the immunophenotype was typical for ES. The tumour stroma, however, revealed prominent myxoid changes, ranging from 50 to 90% (median: 75%). Only one of the proximal type ES showed a SMARCB1 mutation, whereas the other tumours showed no mutation. Conclusions: The myxoid variant of ES represents a diagnostic challenge and may be confused with other benign and malignant myxoid neoplasms. The main differential diagnosis is myoepithelioma of the skin and soft tissue.