乳腺血管肉瘤临床病理分析

目的 分析乳腺血管肉瘤的临床及病理特点.方法 对2例乳腺血管肉瘤进行光镜及免疫组化染色观察.结果 乳腺血管肉瘤组织学形态呈多样性.免疫组化染色显示,血管肉瘤表达血管内皮标记物FⅧRAg、CD31、CD34及淋巴管内皮标记物D240.结论 乳腺血管肉瘤十分罕见,临床表现无特异性,病理形态多样,诊断困难,组织学形态结合免疫组化染色对于确诊有意义.     

隆突性皮肤纤维肉瘤10例临床病理及免疫组化分析

隆突性皮肤纤维肉瘤(Dermatofibrosarcoma protubrans,简称DFSP)是发生于皮肤真皮的低度恶性肿瘤,具有侵袭性生长、容易复发、罕见转移的特点,其组织学来源颇有争议。本文报道10例DFSP临床病理及免疫组化,着重进行DFSP复发病灶与原发病灶的组织学比较,初步揭示两者的差异及其与预后的关系,并根据免疫组化结果,结     

隆突性皮肤纤维肉瘤20例临床病理分析

20例隆突性皮肤纤维肉瘤临床特点为发生于真皮内、病程长、常反复局部复发。组织学特征为梭形瘤细胞围绕小血管排列成车辐状结构且遍及整个瘤体;瘤细胞成分单一、核轻度异型、核分裂象少见;瘤体常包裹较多脂肪细胞和皮肤附件;瘤组织常向皮下脂肪呈树枝状浸润性生长。本文介绍了新近的色素型和纤维肉瘤型两个亚型。     

伴有纤维肉瘤变的隆突性皮肤纤维肉瘤12例临床病理分析北大核心CSCD

目的 探讨伴有纤维肉瘤变的隆突性皮肤纤维肉瘤（FS-DFSP）的临床病理学特征.方法 对1997年1月至2011年12月收集的12例FS-DFSP进行形态学观察,免疫组织化学EnVision法标记,并结合患者的临床资料及相关文献进行讨论.结果 12例患者,男10例,女2例,平均年龄53岁（41 ～70岁）.临床表现为躯干和四肢皮下缓慢性生长的无痛性肿块,其中7例短期内肿块迅速增大,并伴有隐痛.3例为原发性,9例为复发性.镜下表现为经典的DFSP中出现纤维肉瘤样（FS）改变,且FS的区域超过肿瘤的5％.与经典的DFSP不同,FS区域内瘤细胞密度增高,常呈长束状、人字形或鱼骨样排列,而席纹状结构已不明显或完全消失,瘤细胞核异型性较明显,核分裂象（6～25）/10 HPF.免疫组织化学结果显示经典的DFSP区域瘤细胞CD34弥漫强阳性,而FS区域瘤细胞CD34明显减弱.FS区域Ki-67阳性指数约10％ ～40％,显著高于经典DFSP区域的2％ ～5％.12例患者均行手术切除治疗,并有2例行术后辅助放疗.9例获得随访,时间9 ～86个月,6例局部复发,2例患者发生转移,1例患者于术后9个月死亡.结论 FS-DFSP是一种罕见而独特的DFSP亚型,与潜在低度恶性/交界恶性的经典DFSP不同,FS-DFSP在临床、病理和生物学行为上均表现为明确的恶性肿瘤特点,具有较高的复发率及转移率.     

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。     

原发性胸膜上皮样血管肉瘤2例临床病理分析

目的探讨原发性胸膜上皮样血管肉瘤(epithelioid angiosarcoma, EAS)的临床病理学特征、鉴别诊断、治疗及预后。方法收集2例原发性胸膜EAS的临床病理资料,并复习相关文献。结果 2例患者男、女性各1例,年龄分别为47、45岁,均以呼吸系统症状就诊。影像学发现左侧胸膜增厚或包块。镜检见异型上皮样细胞及含红细胞的空泡状瘤细胞,胞质嗜伊红色或透明,胞核圆形空泡状或不规则形,核仁增大,局部见梭形瘤细胞,核分裂象5～12/HPF,坏死多少不等。瘤组织排列呈实性巢片状、条索状、窦隙状脉管样及乳头状。免疫表型:肿瘤细胞vimentin、CD31、ERG弥漫阳性,CD34阴性,Ki-67增殖指数为40%～60%。患者未行放、化疗,均于确诊后短期内死亡。结论原发性胸膜EAS十分罕见,其高度恶性、预后差,临床表现缺乏特征性,易误诊,组织病理学检查可确诊,需与转移癌、恶性间皮瘤、上皮样血管内皮瘤、黑色素瘤及大细胞淋巴瘤鉴别。     

上皮样肉瘤的临床病理和免疫组化观察

上皮样肉瘤是一种比较少见的软组织恶性肿瘤 ,往往误诊为滑膜肉瘤、无色素性恶性黑色素瘤、恶性纤维组织细胞瘤、血管肉瘤 ,为此我们对 3例上皮样肉瘤进行了光镜观察和免疫组化染色 ,以探讨其临床病理特点、诊断、鉴别诊断及组织来源。1　材料和方法收集淄博矿务局中心医院、山东铝业公司医院及淄博铁路医院外科手术切除的软组织上皮样肉瘤 3例 ,标本经10 %福尔马林固定 ,石蜡包埋 ,4μｍ厚切片 ,ＨＥ染色 ,光镜观察。免疫组化采用ＬＳＡＢ法 ,试剂ｖｉｍｅｎｔｉｎ ,ＥＭＡ ,ｃｙｔｏｋ ｅｒａｔｉｎ,ｌｙｓｏｚｙｍｅ ,α1 ＡＣＴ ,Ｓ 10 …     

骨原发性血管肉瘤10例临床病理观察

目的 探讨骨原发性血管肉瘤的临床病理特征、诊断及鉴别诊断。方法 收集10例骨原发性血管肉瘤的临床资料,行HE、免疫组化染色并对患者进行随访。结果 10例血管肉瘤患者中男性7例,女性3例,年龄25～75岁,中位年龄58.5岁。8例发生于骨盆、1例肱骨、1例股骨。镜下可见不同分化程度的血管腔隙,内衬异型肿瘤细胞,其中8例主要由片状或条索状排列的上皮样细胞构成,胞质丰富嗜酸性,核仁明显,诊断为上皮样血管肉瘤。免疫表型：不同程度表达CD31、CD34、ERG、CK等。8例获得随访资料,其中4例存活,另4例术后5～12个月死亡。结论 骨原发性血管肉瘤是少见的恶性肿瘤,术前影像及病理活检易误诊。免疫组化有助于鉴别诊断,多数患者预后较差。     

多形性脂肪肉瘤6例临床病理分析

目的探讨多形性脂肪肉瘤(pleomorphic liposarcoma,PLPS)的临床病理学特征。方法采用免疫组化En Vision法和FISH法检测6例PLPS的临床特点、组织学形态,并复习相关文献。结果男性4例,女性2例;发病年龄27～76岁,平均58.5岁。发病部位是腹膜后(4例)、腋窝(1例)和大腿(1例)。PLPS由梭形细胞、圆形细胞、多形性脂肪母细胞及"筒花状"多核巨细胞组成。2例PLPS为上皮样。4例肿瘤细胞呈S-100阳性。6例肿瘤细胞MDM2基因扩增均阴性。6例PLPS均行手术切除,其中4例发生复发,1例发生2次转移。结论 PLPS是脂肪肉瘤少见亚型,主要由多形性脂肪母细胞构成。治疗以手术切除为主,并密切随诊。肿瘤易复发、易转移且预后较差。患者年龄≥60岁、中心(非肢体)位置、肿瘤≥10 cm和核分裂象≥10/10 HPF可能是PLPS不良预后的独立因素。     

肾透明细胞肉瘤10例临床病理分析

目的探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney, CCSK)的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析10例CCSK的临床特征、病理学改变及免疫表型特征,并复习相关文献。结果 10例患者中男性8例,女性2例,发病年龄1～34岁,平均7岁,其中1例为34岁成人CCSK。临床症状均为腹部包块。7例获得随访,其中2例死亡,5例存活。10例中均有不同比例的经典型CCSK组织学形态,表现为胞质浅染、核染色质细腻、核仁不清楚的片状瘤细胞被呈树枝状分布的纤维血管间质分隔;此外,5例呈黏液样型、3例呈富于细胞型、2例呈硬化型和1例呈上皮样型。免疫表型:vimentin、BCL-2、EGFR、TLE1、Cyclin D1、BCOR、SATB2阳性率依次为100%(10/10)、90%(9/10)、90%(9/10)、90%(9/10)、80%(8/10)、30%(3/10)、10%(1/10);上皮性、肌源性、神经源性标记均为阴性。结论 CCSK是一种少见的儿童期恶性肾肿瘤,因其组织学亚型较多,需与多种肿瘤进行鉴别诊断,使用免疫组化vimentin...     

Fatal poorly differentiated angiosarcoma of the scalp

Cutaneous angiosarcoma is a very rare but aggressive tumor. Angiosarcoma of the scalp is very rare, and a review of the world literature revealed less than 60 cases. Here, the author reports a case of poorly differentiated angiosarcoma of the scalp. The pathological diagnosis was very difficult. A 70-year-old Japanase man was admitted to our hospital complaining of red mass of the scalp. An excisional biopsy was done. The biopsy showed proliferation of malignant spindle cells in the dermis. Apparent differentiation was not recognized. Invasion into the lateral dermis and subcutis was recognized. There were many mitotic figures and a few foci of necrosis. The size was 2 × 2 × 3 cm. Intracytoplasmic vacuoles were recognized in the malignant tumor cells in some places. A few vague vasoformative features were recognized in one very small area. Immunohistochemically, the malignant spindle cells were positive for factor VIII-related antigen (F-VIII-RA), Ulex lectin, CD31, CD34, vimentin, p53 protein. The Ki-67 labeling was 76%. In contrast, the tumor cells were negative for cytokeratins, epithelial membrane antigen, desmin, S100 protein, α-smooth muscle antigen, bcl-2, melanosome, and myoglobin. The intracytoplasmic vacuoles were strongly positive for F-VIII-RA, Ulex lectin, CD31, and CD34, The abortive vasoformative channels were moderately positive for these endothelial markers. A pathologic diagnosis of angiosarcoma of the scalp was made. Chemoradiation and immunotherapy were performed. However, the tumor recurred several times, and ultimately metastasized to the systemic bones and lungs. The patient died of systemic carcinomatosis 33 months after the first manifestation.     

低分化滑膜肉瘤临床病理及分子遗传学研究

目的：研究低分化滑膜肉瘤的临床病理学特点及其分子遗传学表现。方法：收集低分化滑膜肉瘤标本121例，采用形态学观察和免疫组化染色，并用RT－PCR方法在石膜包埋组织中检测SYT－SSX融合mRNA表达。结果：12例低分化滑膜肉瘤中细胞型4例，大细胞型6例，高度恶性梭形细胞型2例。8例有随访资料其中4例死于肿瘤，平均生存时间18个月。免疫组化表现为CK和（或）EMA阳性，以EMA阳性率较高，同时vimentin阳性。S－100蛋白也有较高阳性率，多呈局灶或散在阳性。RT－PCR方法均可检测到SYT－SSXmRNA表达，对照组12例肿瘤包括恶性周围神经 鞘膜瘤、尤因肉瘤和恶性血管外皮瘤SYT－SSX检测全部阴性。结论：低分化滑膜肉瘤有其形态学及免疫表型特点，分子遗传学检测SYS－SSX融合mRNA有助于诊断和鉴别诊断。     

脂蛋白肾小球病的临床病理分析

脂蛋白肾小球病(lipoprotein glomerulopsthy,LPG)是20世纪80年代末才命名的一种新型的独立的肾小球病,属于常染色体隐性遗传疾病,是以肾小球内大量脂蛋白栓子为特有的组织学特征,临床见异常血浆脂蛋白,类似Ⅲ型高脂蛋白血症,伴有明显血清ApoE升高.我们报道3例LPG患者肾活检组织的光镜、免疫组织化学或免疫荧光及超微结构改变,并结合患者的临床特点进行分析,旨在加深对此病的认识.     

Clinicopathologic and immunohistochemical study of breast angiosarcoma

Breast angiosarcoma (AS) is a rare malignancy which can be classified into primary or secondary as a result of breast cancer therapy. On histology, breast AS has a wide spectrum of morphologic presentations, and its diagnosis can be challenging based on morphologic evaluation alone. Here, we studied 10 cases of breast AS diagnosed at our institution during a 20-year period, in which 7 cases were radiation-associated AS (RA-AS) and 3 cases were primary AS (P-AS). The average latency between radiotherapy and RA-AS was 8.1 years. RA-AS mostly occurred in breast skin, while all P-AS involved breast parenchyma. All 10 AS cases were high grade, including 4 RA-AS cases demonstrating epithelioid morphology. Histologic morphologies of AS varied from confluent growth of atypical spindle or epithelioid cells to scattered marked pleomorphic cells. Some cases appeared deceptively bland or low grade, but the presence of areas of haemorrhage (‘blood lake’) or necrosis upgraded them to high grade lesions. Additionally, some epithelioid RA-AS cases with lymphatic differentiation (D2–40 positive) showed pseudopapillary morphology characterized by discohesive cells sloughing off at periphery of vascular cores, resembling papillary breast carcinoma. P-AS did not show prominent vesicular nuclei and/or conspicuous nucleoli, which were features observed in RA-AS. C-MYC immunostain results showed P-AS was completely negative or focal weakly positive in hypercellular areas. In comparison, RA-AS were consistently positive for c-MYC. Epithelioid RA-AS with lymphatic differentiation tended to show stronger and/or more diffuse c-MYC positivity than other AS cases. CD31 and ERG immunostains showed positivity in all cases, while CD34 were negative in some cases with lymphatic differentiation. This study offers a detailed morphologic and immunohistochemical assessment of a rare tumor of the breast that is important to recognize. Common differential diagnosis for breast AS, including post-radiation atypical vascular proliferation (AVP), are also reviewed and discussed.     

Report of an angiosarcoma mimic: cutaneous aneurysmal fibrous histiocytoma

Aneurysmal fibrous histiocytoma is an unusual variant of the spectrum of fibrous histiocytomas with the peculiar morphologic appearance of a benign aneurysmal vasoformative process that ultimately culminates in multiple microhemorrhages within the tumor. It looks strikingly different from the usual cutaneous lesions encountered in clinical dermatology practice. A single report of a cutaneous aneurysmal fibrous histiocytoma in the skin of the back of a 60-year-old male is described with emphasis on the immunostaining pattern and review of the literature. There is a significant potential for confusion of this lesion with other cutaneous lesions, clinically as well as pathologically. In our case, the patient presented with a lesion that clinically resembled a hemangioma, was pathologically interpreted initially to be an angiosarcoma, and finally, the revised pathology was interpreted as an aneurysmal variant of a fibrous histiocytoma. Caution is warranted to avoid misinterpretation of cutaneous fibrohistiocytic tumors.     

乳腺原发性血管肉瘤的临床及病理学特征

目的探讨乳腺原发性血管肉瘤的临床及病理特点。方法复习5例乳腺原发性血管肉瘤切片,依据2003年WHO乳腺肿瘤组织学分类标准诊断和分级。采用第Ⅷ因子相关抗原、CD31、CD34、雌激素受体（ER）、孕激素受体（PR）、细胞角蛋白（cK）及c-erbB-2等抗体进行免疫组织化学SP法染色观察。结果5例患者均以乳腺无痛性肿块为首发症状,术前B超、钼靶检查缺乏特异性诊断。组织学呈多样性表现是乳腺血管肉瘤组织学形态特点。免疫组织化学染色显示,5例血管内皮相关抗原均呈阳性表达,ER、PR、CK及c—erbB-2均阴性。结论乳腺原发性血管肉瘤非常罕见,临床表现缺乏特异性,病理形态多样,诊断困难,血管内皮相关抗原均呈阳性表达对确诊有帮助。     

子宫内膜间质肉瘤55例临床病理特点和预后分析

目的 了解子宫内膜间质肉瘤(ESS)的病理形态特征并分析影响预后的相关指标.方法 收集该院55例ESS患者的临床和病理资料,所有病例重新阅片,参照文献分类为低级别子宫内膜间质肉瘤(LGESS)、不伴核多形的未分化子宫内膜肉瘤(UES-U)、伴有核多形的未分化子宫内膜肉瘤(UES-P);同时观察肿瘤细胞的形态特点,包括纤维样、肌样、黏液样、上皮样分化,并计数核分裂象等.对所有病例进行临床资料的收集并随访.结果 LGESS、UES-U、UES-P型病例分别为39、9、7例.病理形态上,ESS有多种形态分化并存的特点,LGESS、UES-U及UES-P型病例中分别有12.8%(5/39)、5/9及5/7伴有两种以上混合的形态学分化;同一病例的不同区域核分裂象计数和组织学类型亦存在较大差异.临床上肿瘤复发比例分别为51.6%(16/31)、5/6、2/3;LGESS无死亡病例,UES-U和UES-P中各有2例死亡,且UES-U的死亡病例均有局灶UES-P区域.按核分裂象最高计数进行预后分析,≥10/10 HPF的病例复发率显著高于＜10/10 HPF的复发率(P=0.009),在LGESS病例中亦存在这种统计学差异,所有死亡病例的核分裂象最高计数均＞30/10 HPF.结论 ESS常见不同程度分化重叠及多向分化的特点,尤以UES-U和UES-P中更为常见,因此应充分取材以寻找诊断线索.肿瘤中伴有UES-P图像,同时伴核分裂象计数高度活跃可能会增加死亡风险.在LGESS病例中,核分裂象最高计数≥10/10 HPF的肿瘤复发率显著增高,在诊断时应引起重视.

Abstract：

Objective To investigate the clinicopathologic features and the prognostic factors of endometrial stromal sarcoma (ESS). Methods 55 cases of endometrial stromal sarcoma were reviewed and categorized into 3 pathologic types based on the related literatures, i.e. , low grade endometrial stromal sarcoma (LGESS), undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P). Meanwhile, the pathologic features were reviewed, including fibroid, myoid, mucoid, and epithelioid differentiation and mitotic index.Clinical and follow-up data were collected. Results In endometrial stromal sarcoma, two or three pathologic types co-existed in one case, including 12. 8% (5/39) of LGESS, 5/9 of UES-U, and 5/7 of UES-P.Mitotic index varied in different regions of one tumor from rare to high. Multi-differentiation was also commonly seen in ESS. The numbers of cases in LGESS, UES-U and UES-P were 39, 9 and 7, with recurrence rate of 51.6% ( 16/31 ), 5/6 and 2/3, respectively. There was no death case in LGESS, and 2 cases were died in UES-U and UES-P, respectively. In the 2 death cases of UES-U, both had focus of UES-P. There was a significant difference in the recurrence rate between cases with different mitotic index ( ≥ 10/10 HPF and ＜ 10/10 HPF, P = 0. 009), especially in LGESS group. All death cases had high mitotic index ( ＞ 30/10 HPF). Conclusions It is a common phenomenon in ESS that two or three pathologic types may exist in one case, especially in UES-U and UES-P. And multi-differentiation is also commonly seen in ESS. So adequate pathologic sampling is important for pathologists to make a correct diagnosis of ESS in daily work. The recurrence rates are significantly higher in cases with high mitotic index,especially in LGESS. In addition, the presence of UES-P and high mitotic index may increase the risk of death in the patients.     

原发性皮肤间变性大细胞淋巴瘤临床病理分析

目的 探讨原发性皮肤间变性大细胞淋巴瘤(C-ALCL)的临床病理特征、免疫表型及预后.方法 分析8例C-ALCL的临床病理资料,复习HE切片,进行T淋巴细胞、B淋巴细胞、活化淋巴细胞和细胞毒性等16种标记的免疫组织化学染色,原位杂交检测EB病毒.结果 8例中男3例,女5例,中位年龄49.5岁.临床上以皮肤无症状的单个红色结节、肿块为主要表现,组织学上肿瘤细胞在真皮与皮下脂肪内大片状、弥漫性浸润.瘤细胞以大细胞为主,异形性明显.8例C-ALCL的瘤细胞CD30阳性细胞数均大于75%.瘤细胞均表达1～3个T细胞标记(CD3、CD5或CD45RO)及1～3个细胞毒性标记[T细胞内抗原(TIA)-1、颗粒酶B或穿孔素].表达白细胞共同抗原(LCA)为8例、CIM为5例、CD8为1例、间变性淋巴瘤激酶(ALK)-1为1例、上皮细胞膜抗原(EMA)为3例,均不表达CD15、CD20、CK和HMB45.EBER 1/2原位杂交均为阴性.获随访的6例中5例存活,1例死亡(死因不详).结论 C-ALCL有独特的临床病理表现和免疫表型,预后较好.EB病毒与C-ALCL可能无明确的相关性.