上皮样肉瘤二例临床病理分析

 目的　探讨上皮样肉瘤的临床、病理学特征及鉴别诊断要点。方法　收集2例上皮样肉瘤患者的临床病理资料,光学显微镜下观察和分析其组织学形态特征,并通过免疫组织化学方法染色分析。结果　2例患者中经典型和近端型上皮样肉瘤各1例。2例上皮样肉瘤均由两种细胞组成,一种为圆形或多角形的上皮样细胞,胞质丰富,嗜酸染色,另一种为肥大的梭形细胞,组织结构呈结节状,可见中心性坏死。免疫组织化学染色2例广谱CK、EMA、Vimentin均阳性表达,CK7、CD68、HMB45、Syn、SMA均阴性表达,其中1例少数细胞S-100阳性表达。结论　上皮样肉瘤是一种少见的软组织恶性肿瘤,需要临床特点、组织学特征及免疫组织化学染色结果相结合,才能做出准确的诊断。     

11例上皮样血管肉瘤的临床病理分析

[目的]探讨上皮样血管肉瘤的临床病理学特征及诊断、鉴别诊断要点。[方法]分析1995～2007年我院收治的11例上皮样血管肉瘤临床病理资料。[结果]镜下观察肿瘤细胞弥漫成片或聚集成巢，伴有出血和多灶性坏死。瘤组织内常见互相吻合、大小不等的不规则腔隙，其内衬覆异型瘤细胞，可见单个或多个瘤细胞形成空腔包绕红细胞。瘤细胞9例CD34阳性、10例CD34阳性。术后存活期5周～55个月。[结论]上皮样血管肉瘤是血管肉瘤的一种特殊类型，免疫组织化学检测有助于确定肿瘤细胞的内皮分化。     

上皮样血管肉瘤临床病理分析

利用HE染色、免疫组织化学法观察3例上皮样血管肉瘤。结合文献探讨上皮样血管肉瘤的临床病理学特征及诊断、鉴别诊断要点。     

上皮样肉瘤3例临床病理分析

上皮样肉瘤(epithelioid sarcoma,ES)是一种分类未明的肉瘤,好发于青年人肢端.临床表现多样,ES易沿组织间隙浸润性生长,局部复发和远处转移的发生率高,预后不佳.本文报道3例上皮样肉瘤,并结合文献复习其组织学诊断和鉴别诊断.     

上皮样肉瘤三例

上皮样肉瘤是一种罕见的、好发于四肢的软组织肿瘤 ,属低度恶性 ,但易被误诊。自 1980年以来 ,我国仅报道 4 8例 ,近年来我科收治 3例上皮样肉瘤患者 ,现报道如下。例 1　患儿女 ,2岁半。约 1岁时发现枕部有一个绿豆大小的结节 ,无自觉不适。于 1994年 10月 17日就诊前 2个月 ,结节增大 ,并由原来的 1个增至 3个。检查 :枕后部有 3个 1ｃｍ× 1.5ｃｍ的结节 ,且相互连接成条索状 ,表面色泽发白 ,质地中等 ,结节边界清楚 ,与皮下骨膜黏连。例 2　患者男 ,36岁。发现双侧坐骨结节处肿块半年 ,坐时有疼痛 ,出现大便形状改变 1个月 ,于 1997年 7…     

上皮样肉瘤1例

患者，女性，年龄５２岁。主因右腕部溃疡伴环、小指麻木，活动受限３年余，于２００１年１２月９日入我院治疗。患者曾于１９９７年６月因右腕部长一黄豆大肿物，经手术切除后切口未见愈合，切口溃疡逐渐增大。临床表现：右手大小鱼际肌萎缩及第一骨间肌萎缩，右腕背侧见一２cm×３cm大小溃疡，周围轻度红肿。X线示：右关节骨质结构未见明显异常。行腕部病灶切除，腹部半埋藏皮瓣断蒂术。术后给予天冬氨酸阿奇霉素治疗，患者恢复良好。术后随访未见复发。病理检查：皮肤组织２块，皮肤表面见一溃疡，溃疡大小为２．５cm×２．４cm×０．４…     

上皮样肉瘤1例

上皮样肉瘤(Epithelial sarcoma,ES)是一种少见的软组织肿瘤,在四肢的发生率可达70.6%[1],位于头部文献罕见报道.     

上皮样肉瘤3 例

上皮样肉瘤罕见，1970年由Enzinger首先报告并命名。现将天津市肿瘤医院1991年9月～1998年12月间收治的3例上皮样肉瘤分析报告如下。 例1 男，39岁。因左足内踝肿胀破溃3年于1991年9月4日入院。入院后检查发现破溃区合并绿脓杆菌感染。因感染长期未能控制，全身化疗禁忌，病情继续发展，保留肢体困难，于1991年10月28日行左股骨中下1／3截肢术。大体检查：左腿中下1／3截肢标本长60cm，在内踝处见一9cm×9cm×2cm的溃疡。镜下检查：瘤细胞呈上皮样(短梭形、圆形），呈结节状排列； 瘤细胞大多分布在结节周围，结节中间有变性、坏死，部分可…     

近端型上皮样肉瘤的诊治分析

目的研究近端型上皮样肉瘤的临床特点、治疗方法及愈后分析。方法 回顾性分析2000年1月—2013年5月北京大学第一医院收治的7例近端型上皮样肉瘤病例,对其临床特点、病理学表现、诊治过程及预后进行分析。结果 7例近端型上皮样肉瘤患者临床表现为发生于头颈及躯干部缓慢生长的皮下无痛性结节,均接受手术治疗,术后大部分(5/7)患者接受放射治疗,7例患者的1年、3年及5年生存率分别为100%、80%和60%。结论 近端型上皮样肉瘤临床罕见,易局部复发、淋巴结转移及远处转移,患者预后不佳。明确诊断需病理学检查,手术切除仍是其主要治疗方式。     

上皮样肉瘤14例临床分析

背景与目的:上皮样肉瘤是一种组织起源尚不清楚的罕见软组织肉瘤.其生物学行为独特,容易发生局部复发、淋巴结扩散和/或远处转移.本研究旨在探讨上皮样肉瘤(Epithelioid sarcoma)的临床特点、诊断、治疗和预后.方法:对我院1990年1月至2005年12月间收治的14例上皮样肉瘤临床资料进行回顾性分析,并结合随访资料进行生存分析.全组14例均接受手术治疗.首次手术均在外院进行.再次手术11例在我院进行(9例行扩大切除术,2例行截肢术;4例同时行区域淋巴结清扫术),3例仍在外院行局部切除术或扩大切除术.术后9例接受辅助性治疗,其中单纯放疗6例,放疗 化疗3例.结果:全组在本院病理科复检确诊为上皮样肉瘤.12例(85.7%)出现肿瘤局部复发.4例(28.6%)发现区域淋巴结转移,9例术后3年内死亡.全组总的1、2、5、10年生存率为71.43%、55.56%、27.78%和13.89%.结论:上皮样肉瘤恶性程度并不很高,但局部复发率高,容易发生淋巴结和/或远处转移,预后不佳.广泛性切除或根治性切除合并预防性区域淋巴结清扫术是治疗上皮样肉瘤的有效手段.     

间叶性软骨肉瘤3例临床病理观察

目的：探讨间叶性软骨肉瘤（mesenchymal chondrosarcoma，MC）的临床病理特征、诊断与鉴别诊断。方法：结合临床资料及影像学所见，分析3例MC患者的组织病理特点并复习相关文献。结果：患者均为成人，平均年龄42．3岁。主要临床表现为疼痛、肿胀、局部肿块伴或不伴肿瘤压迫症状。发病部位分别是小脑幕、左胸壁及骶椎。x线显示溶骨性破坏，其内可见斑驳钙化。巨检：肿瘤组织切面呈灰白色，质地软硬不均，点缀着不规则矿化沉积物。镜下肿瘤由密集的未分化细胞与散在的软骨岛构成。在蓝染的小细胞区，细胞较原始，呈圆形或短梭形，胞质稀少，排列成片层状或形成血管外皮瘤样；在软骨成分区，细胞相对成熟，分化良好。两种成分截然分界或逐渐混杂。免疫组织化学显示3例未分化细胞区vimentin呈弥漫阳性，部分CD99呈阳性；软骨分化区S-100呈阳性，而二者均表达Sox-9。结论：MC是一种罕见的高级别骨或软组织软骨源性恶性肿瘤。具有独特的双向组织学表现，预后不佳。     

Epithelial profile of epithelioid sarcoma. An immunohistochemical analysis of eight cases

Eight cases of epithelioid sarcoma were examined immunohistochemically, in order to clarify the adjunct epithelial profile of the tumor and to ascertain the intermediate filaments contained in the tumor cells. All tissues showed a strongly positive immunoreactivity for epithelial membrane antigen (EMA) and tissue polypeptide antigen (TPA). In the case of carcinoembryonic antigen (CEA), one showed a strong reaction, whereas the others were variably less stained. Cytokeratins (45 kd and 54 kd) specific for simple epithelia, including coelomic epithelium, were regularly found in all cases; a small number of cells were positive for high molecular weight cytokeratin (57 kd) and none for 66 kd cytokeratin. Coexpression of both cytokeratin and vimentin was confirmed using fresh-frozen materials. Therefore, both microscopic and immunohistochemical evidence supports the hypothesis that epithelioid sarcoma masquerades as a carcinoma.     

Fine-needle aspiration of epithelioid sarcoma: cytology findings in nine cases

BACKGROUND: Epithelioid sarcoma is a rare tumor with characteristic morphologic and immunohistochemical features. It can be confused histologically and cytologically with a variety of benign and malignant lesions, including a granulomatous process, synovial sarcoma, melanoma, squamous cell carcinoma, and adenocarcinoma. The objective of this study was to define the cytologic features of this rare tumor. METHODS: The cytologic features of nine histologically confirmed epithelioid sarcomas were analyzed. The criteria evaluated included cell size and shape, cell borders, cluster organization, cytoplasmic characteristics, nuclear and nucleolar features, and background characteristics. RESULTS: In most cases, single, dispersed cells represented the predominant pattern, with only a few small clusters present. The cells were mostly round with interspersed spindle cells and mild to moderate pleomorphism. The nuclei were large and eccentrically located, with a plasmacytoid appearance. A pale zone in the perinuclear area was evident in three of nine cases. Well-defined cell borders with intercellular spaces between malignant cells were observed in eight cases. In three cases, a granuloma-like structure was identified. In two cases, the cells were mostly spindle and showed greater cellular pleomorphism. CONCLUSION: Epithelioid sarcoma is an uncommon tumor with a wide range of differential diagnoses, especially in cytology specimens. Awareness of its existence and knowledge of its cytologic features are important for a correct diagnosis.     

Synovial sarcoma: a clinicopathological study of 36 cases.

Thirty-six cases of synovial sarcoma (13 biphasic and 23 monophasic) were subjected to a clinicopathologic study that included electron microscopy and immunohistochemistry. The group consisted of 21 males and 15 females ranging in age from 2 to 63 years. The majority of tumors (27 cases) were found in the hip and lower extremity. Immunohistochemical study revealed that keratin, which was detected in 92% of the biphasic and 57% of the monophasic tumors, was a more sensitive marker of epithelial differentiation than EMA or CEA. The overall 5-year survival of the patients was 64%. Male sex, older age, presence of tumor necrosis, monophasic pattern, and absence of keratin positivity had an unfavourable effect on survival but lacked statistical significance. Survival was significantly lower in patients with tumors exhibiting more than 15 mitoses per 10 HPF (P less than .02) and in those with tumors showing necrosis and a mitotic rate greater than 5 mitoses per 10 HPF (P less than .005).     

Tendosynovial sarcoma: a clinicopathological study of 136 cases.

A series of 136 cases of tendosynovial sarcomas were studied from the histologic point of view. All of the primary, recurrent and metastatic tumors were reclassified according to histologic type, anatomic site and size; age and sex of patients and modality of surgical treatment. The overall 5-year survival rate was 40%. Among the factors which most favorably influenced the prognosis were: small size (74%, 5-year survival), "exposed" primary site (53%, 5-year survival), and histologic type (biphasic form: 55%, 5-year survival and epithelioid sarcoma: 58%, 5-year survival). Children and elderly patients had also better than the average prognosis. Sixty percent of the sarcomas recurred after wide local excision. Twelve percent of the tumors metastasized to regional lymph nodes and 10% to bones. Almost all of the 24 patients who were autopsied died because of cardio-pulmonary insufficiency due to massive pleuropulmonary metastasis. It is hoped that better understanding of the natural history of these tumors will lead to optimal local and systemic therapy and better survival.     

增生性筋膜炎9例临床病理分析

目的:探讨增生性筋膜炎(PF)临床病理特点及鉴别诊断,提高对增生性筋膜炎的认识,避免误诊.方法:回顾性分析9例增生性筋膜炎的临床情况、组织学形态及免疫表型并复习相关文献.结果:PF好发于四肢及头颈部,发病年龄为32 ～ 80岁.临床表现为伴有疼痛,迅速生长浅表孤立性肿块.镜下特点为黏液样背景、增生的纤维母细胞/肌纤维母细胞、间质散在分布具有诊断意义的神经节样大细胞;免疫组化梭形细胞及神经节样大细胞表达Vimentin、SMA和H-Caldesmon.结论:PF是一种较少见的良性病变,手术切除后罕见复发,不转移,其特殊的临床表现及病理形态特征,易被误诊为恶性肿瘤.应加强对该病变的认识,避免误诊.     

子宫上皮样平滑肌瘤临床病理分析

目的 探讨子宫上皮样平滑肌瘤临床病理特征和生物学特性.方法 观察2例子宫上皮样平滑肌瘤的临床、病理组织学及免疫组织化学特点,KRAS基因突变检测分析并复习文献.结果 2例均由胞浆丰富嗜酸的上皮样肿瘤细胞组成.1例体积巨大,大小为20 cm×17 cm×8 cm,镜下未见坏死及核分裂相;1例属多发性子宫平滑肌瘤,其中仅有...     

Epithelioid sarcoma and unclassified sarcoma with epithelioid features: clinicopathological variables, molecular markers, and a new experimental model

Background.

Epithelioid sarcoma (ES) and unclassified sarcoma with epithelioid features (USEF) are clinically and therapeutically unresolved. We compared ES and USEF patients'' clinical behavior, treatment, outcome, and molecular marker expression. Furthermore, preclinical ES study models were developed to enable comprehensive benchside investigations.

Patients and Methods.

A database of ES and USEF patients (n = 116) treated since 1992 was created. A clinically annotated ES–USEF tissue microarray (TMA) was assayed for tumor-related markers. Newly established human and commercially available ES cell lines were characterized and tested in vivo.

Results.

ES and USEF patients presenting with localized disease exhibited 22% and 25% local recurrence rates, 35% and 19% nodal metastasis rates, and 41% and 53% distant metastasis rates (median follow-up, 54 months and 39 months, respectively). The 5- and 10-year disease-specific survival rates were 88% and 43% and 52% and 42% (ES and USEF, respectively). TMA immunohistochemistry identified integrase interactor (INI)-1 loss, cancer antigen 125, and p53 nuclear expression as significantly more common in ES than USEF cases. Both cell lines preserved ES morphological and biochemical characteristics in vitro and in vivo; loss of INI-1 was shown to occur in both lines.

Conclusions.

Enhanced knowledge of ES and USEF clinical behavior, marker expression, and molecular determinants, extended via experimental models, will hopefully accelerate development of urgently needed effective targeted therapies for ES and USEF.     

子宫颈上皮内瘤样病变108例的临床病理分析

目的　探讨子宫颈癌的早期癌前病变 ,诊断要点及早期防治。方法　收集 10 8例子宫颈上皮内瘤样病变 ,其中癌前病变 73例 ,原位癌 3 5例 ,通过临床随访和光镜下的特点进行观察和分级。结果　诊断CINⅠ级 3 3例 ,CIN 2级 40例 ,CINⅢ 3 5例。结论　 73例癌前病变 ,有 8例CINⅡ级患者在一年内手术后发现为原位癌 ,另外 6 5例癌前病变经 3～ 2 0年随访 ,未发现癌变。