Epithelioid trophoblastic tumor: clinicopathological features with an emphasis on uterine cervical involvement.

We report on the clinical and histological features of five cases of epithelioid trophoblastic tumor, with an emphasis on its involvement of the uterine cervix. All five patients were of reproductive age (median age 38.4 years) and all, except one, presented with vaginal bleeding 3 to 18 years after the most recent pregnancy. One patient presented with amenorrhea. Elevation of serum human chorionic gonadotropin (hCG) was seen in four cases. Pathologically, the tumor involved endocervix in three cases and involved uterine corpus in another two. All five tumors were invasive, nodular lesions consisting of epithelioid intermediate trophoblastic cells that were mononuclear with abundant eosinophilic cytoplasm, along with zones of hyaline material and necrotic debris. In three cases of cervical involvement, the neoplastic cells focally replaced endocervical surface and glandular epithelium, simulating high-grade squamous intraepithelial lesions. Immunohistochemically, all five tumors displayed focal positivity for human placental lactogen and hCG. Positive nuclear staining of p63 was seen in all five cases. All patients received total hysterectomy and various regimes of adjuvant chemotherapy. Three patients survived the tumor with no recurrences or metastases with follow-up periods of 3, 7 and 16 years. One patient is currently alive with lung metastasis 1 month after the surgery. One patient died of tumor metastasis 8 months after the diagnosis. In summary, with its unusual ability to simulate an invasive squamous cell carcinoma and other epithelioid neoplasms, epithelioid trophoblastic tumor frequently poses a diagnostic challenge, especially when involving the uterine cervix. High index of suspicion and an awareness of elevation of serum chorionic gonadotropin are crucial in reaching a correct diagnosis.     

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。     

Primary cutaneous interdigitating dendritic cell sarcoma is a morphologic and phenotypic simulator of poorly differentiated metastatic melanoma: A report of 2 cases and review of the literature

Interdigitating dendritic cell sarcoma (IDS) is a rare form of hematologic malignancy associated with an aggressive clinical course. Only 4 prior cases have been described as originating in the skin. We encountered two male patients ages 47 and 61 years of age who presented with solitary cutaneous neoplasms diagnosed as IDS. Histologic exam showed a coalescing nested and multinodular proliferation of large pleomorphic epithelioid cells. In one case an initial diagnosis of melanoma was rendered. A recurrence 8 months later was then interpreted as a primary cutaneous IDS. This patient died of widespread metastatic disease within 2 years from his initial surgery. The other patient has recently undergone wide excision and radiation without any recurrence or metastatic disease during this short follow up time period. Both patients had a tumor exhibiting the same phenotypic profile comprising leukocyte common antigen, SOX10, S100, CD68, and CD163 positivity. In reviewing the 4 other reported cases, there is a similar older male predominance (mean age of 58 years) although women affected were significantly younger (mean age of 28 years); there was a predilection for the proximal extremities and the face. Patients treated with excision only developed recurrent disease with one patient subsequently dying of metastatic disease. Primary cutaneous IDS is a highly aggressive hematologic malignancy that has many overlapping features with poorly differentiated epithelioid and spindle cell melanoma including SOX10 staining. An aggressive treatment protocol at the beginning could optimize patient survival.     

子宫上皮样滋养细胞肿瘤临床病理分析

目的 探讨子宫上皮样滋养细胞肿瘤(ETT)的临床病理特征及鉴别诊断,及其与预后相关因素.方法 回顾浙江大学附属妇产科医院2000-2007年间共5例ETT病例,分析其临床病理及CK18、p63、α-抑制素、人绒毛膜促性腺激素(HCG)、人胎盘泌乳素(HPL)、胎盘碱性磷酸酶(PLAP)、Ki-67等免疫表型特征.对所有病例随访11～50个月,并简要分析预后相关因素.结果 5例ETT病例均为生育年龄妇女,平均年龄33岁,发生率占同期妊娠滋养细胞疾病0.48%(5/1037).镜下见肿瘤由相对一致的单核滋养细胞排列成结节状浸润生长,肿瘤细胞巢中可见玻璃样物质.肿瘤坏死较常见,典型者可呈地图状坏死.肿瘤细胞CKl8、p63弥漫阳性,HCG、HPL、PLAP灶性阳性或阴性.α-抑制素表现为阳性或阴性.随访期间,死亡2例:1例核分裂象达15/10 HPF,发病1年后死亡,另1例发生肺转移,2年后死亡.结论 ETT是一种罕见的滋养细胞疾病,具有独特的组织形态和免疫表型特征,核分裂象高与发生肺转移的病例预后可能较差.     

Primary peritoneal mesotheliomas in children: a clinicopathological and immunohistochemical study of eight cases

Aims:  To present eight cases of primary diffuse peritoneal malignant mesothelioma in children <15 years old, with a discussion of the pitfalls of this diagnosis in the paediatric age group.
Methods and results:  The cases were selected based on the following criteria: (i) primary peritoneal neoplasms confined grossly or radiographically to the abdominal cavity; (ii) negative history of previous or another associated malignancy; (iii) histopathological confirmation. All patients (five female, three male) presented clinically with symptoms of abdominal pain, distention and ascites. Grossly, the tumours showed multiple, diffuse peritoneal nodules. Histologically, seven cases corresponded to epithelioid mesotheliomas and one case displayed biphasic (epithelioid and spindle) cellular proliferation. Immunohistochemical studies for cytokeratin (CK) 5/6, calretinin and low-molecular-weight CK (CAM5.2) showed strong cytoplasmic positivity in the neoplastic cells. Three patients were treated by chemotherapy. On clinical follow-up, four patients with epithelioid mesotheliomas were alive and well from 12 to 18 months after initial diagnosis; one patient with a mixed (biphasic epithelioid/sarcomatoid) mesothelioma died of tumour 24 months after diagnosis.
Conclusions:  Peritoneal malignant mesothelioma in children is a rare condition that can introduce difficulties in histopathological diagnosis.     

结节性淋巴细胞为主型霍奇金淋巴瘤的诊断与鉴别诊断

目的　探讨结节性淋巴细胞为主型霍奇金淋巴瘤 (NLPHL)的诊断和鉴别诊断特点。方法　对 1980～ 2 0 0 0年 2 4 5例霍奇金淋巴瘤 (HL)进行回顾性分析 ,发现NLPHL4例 ;另外 2例NLPHL为 2 0 0 0年会诊病例 ;复旦大学肿瘤医院提供 1例。 7例均应用链霉菌抗生物素蛋白 过氧化物酶法(SP法 )检测瘤细胞 (CD4 5、CD2 0、CD15、CD30、波形蛋白 )和背景细胞 (CD3、CD2 0、CD4 5RO、CD5 7、CD6 8、TIA 1)的免疫表型 ,并采用WHO1997年淋巴瘤诊断标准重新分类。结果　NLPHL7例 ,男 4例 ,女 3例 ,平均年龄 4 3.8岁 ,中位年龄 4 3岁 ,表现为颈部、锁骨上或腋窝淋巴结肿大 ,临床Ⅰ期。组织学上 ,NLPHL病变淋巴结结构破坏 ,大部分为肿瘤性病变 ,主要呈结节性生长 ,结节内可见L&H细胞浸润 ,其中 1例NLPHL病变淋巴结可见少部分呈生发中心进行性转化 (PTGCs)改变。免疫组织化学染色显示瘤细胞呈CD4 5、CD2 0阳性 ,CD15、CD30、波形蛋白阴性 ,背景细胞中可见大量CD2 0阳性的B小淋巴细胞及较多CD5 7阳性细胞 ,TIA 1阳性细胞极少见。结论　对NLPHL的诊断必需结合免疫表型特征 ,要注意与经典型霍奇金淋巴瘤及B细胞淋巴瘤相鉴别     

肺上皮样血管内皮瘤临床病理观察

目的 探讨肺上皮样血管内皮瘤的临床病理特点。方法 4例肺上皮样血管内皮瘤,3例女性,1例男性,年龄28～40岁,无自觉症状或有轻度咳嗽、气短。肺活检或手术切除标本经甲醛固定,石蜡包埋,常规HE及免疫组织化学(Envision法)染色。所用抗体包括CD31、CD34、细胞角蛋白(AEl／AE3)、TTF-1、波形蛋白和上皮膜抗原。结果 本组肺上皮样血管内皮瘤病例女性多于男性,胸部CT显示双肺多发弥漫性小结节影。病理形态特点为结节周边上皮样肿瘤细胞呈花冠状充填于肺泡腔,病变中心为黏液透明样变间质,肺泡壁结构保留,肿瘤细胞胞质内有空泡形成,空泡内偶见红细胞,肿瘤细胞异型性不明显,核分裂和坏死均少见,免疫组织化学染色示CD31、CD34阳性,AE1／AE3偶见灶状阳性,其他抗体呈阴性。结论 肺上皮样血管内皮瘤是一种具有独特临床病理特点的低度恶性血管来源肿瘤。     

74例胃肠道间质瘤临床病理与生物学行为评价

目的　探讨在胃肠道间质瘤(GIST)的病理诊断和预后分析上采用一种简单实用且重复性好的病理学“标准”,以利于GIST的日常病理诊断和生物学行为评价及指导治疗,并对Fletcher等推荐的GIST生物学行为评价表进行评估。方法　85例消化道间叶组织肿瘤,复习其病理形态学并应用CD117、CD34、平滑肌肌动蛋白(SMA)、结蛋白、S 100等进行免疫组织化学标记,结合 31例随访资料进行分析。结果　85例消化道间叶组织肿瘤中,GIST74例,平滑肌瘤和交界性平滑肌瘤 8例(食管),平滑肌肉瘤 1例(直肠 ),神经鞘瘤 1例 (胃 ),恶性纤维组织细胞瘤 1例 (肠系膜 )。74例GIST中,发生在胃和小肠的分别为 34例和 30例,占 86. 5%,食管 3例,胃肠道外(肠系膜、网膜、后腹膜)7例。年龄 23~80岁,平均 52 5岁, 40岁以上者占 85%,男性 45例,女性 29例。镜下观察:梭型细胞型 48例,上皮样细胞型 10例,混合细胞型 16例。瘤细胞呈长、短梭形和圆形,胞质丰富弱嗜酸性,排列呈旋涡状、栅栏状或弥漫巢状。免疫组织化学: 85例消化道间叶组织肿瘤波形蛋白均阳性,其中 74例表达CD117,诊断为GIST,表达形式有弥漫胞膜 /胞质强阳性、散在阳性、胞质点状着色等,其中 54例同时表达CD34 (阳性率 72. 9% ), 25例表达SMA, 5例表达结蛋白, 5例表达S 100蛋白。在 85例     

Epithelioid hemangioendothelioma of bone--a clinicopathologic and immunohistochemical study of 7 cases

Epithelioid hemangioendothelioma is a rare vascular tumor of bone. Between 1987-1999, seven cases of epithelioid hemangioendothelioma were recorded. The histopathological evaluation was done with hematoxylin and eosin stain and immunohistochemical stains along with the analysis of clinical data and X-ray findings. There were 4 males, 3 females between 15 to 48 years of age with complaints of pain and swelling. Long bones were involved in five of the seven patients and one was multifocal. Radiologically all seven patients showed osteolytic lesions. The histologic hall mark was the presence of eosinophilic, epithelioid cells with intracytoplasmic vacuoles. The tumor may be histologically confused with metastatic carcinoma, chondromyxoid fibroma and osteogenic sarcoma. Immunohistochemical reactivity was as follows: Vimentin (6/6 cases), Factor VIII related antigen (4/6 cases), Ulex Europeaus (5/6 cases), CD31 (3/5 cases), CD34 (3/5 cases), epithelial membrane antigen (1/6 cases), cytokeratin (none). Treatment comprised curettage (4 cases), wide excision (2 cases), below knee amputation (1 case) and post operative radiotherapy (2 cases). At follow-up, (available in 4 patients) two were without disease at 18 months and 120 months. Of the remaining two, one developed local recurrence after 36 months; while the other died of lung metastasis (18 months). A lytic bone shadow, the presence of plump eosinophilic often vacuolated cells on a myxoid background and immunohistochemistry together help in the correct recognition of this tumor.     

BRAF V600E突变的上皮样胶质母细胞瘤临床病理和分子特征

目的探讨BRAF V600E突变的上皮样胶质母细胞瘤(epithelioid glioblastoma,eGBM)的临床病理特征和分子遗传学改变。方法回顾性分析四川大学华西医院2012—2019年确诊的16例BRAF V600E突变的eGBM,总结其临床和病理特点及分子遗传学改变。结果患者年龄7~61岁,中位年龄31.5岁。其中男性4例,女性12例,男女比1∶3。11例为初诊病例,5例为复发病例。肿瘤多位于大脑半球,以额叶多见,平均直径4.6 cm(2.0~8.0 cm)。镜下表现为排列紧密的上皮样肿瘤细胞或横纹肌样肿瘤细胞呈片状或巢团状分布。肿瘤细胞形态较一致,胞膜明显,胞质嗜酸性,核偏位,空泡状,部分细胞可见核仁,核分裂活跃。所有病例均可见栅栏状/凝固性坏死;多数病例可见肾小球样微血管增生,部分病例可见多核瘤巨细胞。2例有局灶肉瘤样形态,3例可见局灶多形性黄色星形细胞瘤样形态。肿瘤细胞不同程度表达胶质纤维酸性蛋白(GFAP)、Olig2和p53,Ki-67阳性指数10%~50%,仅1例ATRX蛋白表达缺失。Sanger测序显示16例均检出BRAF V600E突变,5例同时检出TERT基因启动子第228位点或第250位点突变,所有病例均未检出IDH1(R132)和IDH2(R172)位点突变。患者均行手术切除肿瘤,3例患者进行了术后辅助放化疗。15例获得随访资料,随访时间1~89个月(中位时间10个月),其中7例患者于术后1~37个月死亡,另有5例复发。年龄<35岁的患者总生存期明显长于≥35岁的患者(P=0.014),但无疾病进展生存时间差异无统计学意义(P=0.232)。结论BRAF V600E突变的eGBM好发于中青年女性。镜下以形态较一致的上皮样肿瘤细胞为主,需与横纹肌样脑膜瘤、间变型多形性黄色星形细胞瘤、非典型畸胎样/横纹肌样瘤、转移癌、黑色素瘤等鉴别。部分病例可观察到多形性黄色星形细胞瘤样区,提示二者起源可能相关。此类肿瘤恶性程度较高,大部分患者在短期内复发或死亡,但年轻患者预后相对较好。     

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。     

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。     

Gastrointestinal stromal tumors in Koreans: it's incidence and the clinical, pathologic and immunohistochemical findings

Seven hundred forty seven cases of gastrointestinal stromal tumors (GISTs) in Koreans who were diagnosed between 2001 and 2002 were analyzed to evaluate their occurrence and their clinical, pathologic and immunohistochemical findings. The most frequent location of tumor was in the stomach (63%), followed by the small intestine (30%), the colorectum (5%), and the esophagus (2%). c-kit expression was found in 93.6% of the cases, while CD34, SMA and S-100 protein was positive in 80.1%, 28.2%, and 20.2%, respectively. c-kit positivity was high in the stomach (94.2%) and small intestine (94.6%), while it was relatively low in the colorectum (85.0%), and esophagus (81.2%). The positivity for CD34 was correlated with the higher risk of GISTs (p = 0.04). Follow up of the patients showed that 58 primary GISTs patients died and 20 of these patients were recurrent or metastatic at the time of diagnosis. The pathologic diagnosis to predict the risk of aggressive behavior of GISTs was correlated with the numbers of tumor, clinical stage, epithelioid histologic type, cellularity, cellular atypia, necrosis, and mucosal invasion (p = 0.00). GISTs with a poor prognosis were closely related to the clinical stage at presentation, the locations of the tumor, and the ages of the patients.     

组织细胞肉瘤的临床病理观察

目的 探讨组织细胞肉瘤(histiocytic sarcoma,HS)的病理形态学、免疫表型、鉴别诊断和预后.方法 分析6例HS患者临床病理资料,对标本行光镜观察、免疫组织化学EliVision法染色,并对患者进行6～36个月的随访.结果 6例HS中男女各3例,年龄12～81岁(平均54.6岁).发生部位为淋巴结(2例)、皮肤及软组织(4例).组织形态改变:瘤细胞弥漫性浸润,呈中至大的多角形上皮样细胞,胞质丰富,嗜酸性,核圆形或不规则,空泡状,有1至多个明显核仁,核分裂象多见.细胞境界较清.6例均见双核瘤细胞,2例多形性明显,可见多核瘤巨细胞,3例可见灶状泡沫状胞质的瘤细胞,1例可见灶状肉瘤样梭性细胞区,2例可见噬血细胞现象.6例均见到多少不一的炎细胞背景.均弥漫表达白细胞共同抗原、CD4、CD68、CD163,溶菌酶染色的5例中4例阳性.4例得到随访资料,3例确诊后6～11个月内死亡,1例局限于皮肤及皮下软组织者已存活3年.结论 明确HS诊断需结合形态改变与免疫表型.该病就诊时多处于进展期,对化疗反应差,但少数病变局限的病例不表现为侵袭性过程,预后较好.     

硬化性上皮样纤维肉瘤的临床病理学观察

目的探讨硬化性上皮样纤维肉瘤的临床病理学特征、免疫表型及鉴别诊断.方法应用光镜和免疫组织化学LSAB法对8例硬化性上皮样纤维肉瘤进行临床病理学分析和免疫表型检测.结果患者均为成年人,男性5例,女性3例,年龄35～54岁(平均45岁).临床上表现为深部软组织内缓慢性生长的肿块,其中大腿/臀部3例,右上臂/肩部2例,胸壁和背部2例,小腿1例.大体上,肿块呈结节状,境界相对清楚,直径2.0～10.5 cm(平均6 em),切面呈灰白色,质地坚韧或有弹性.组织学上,肿瘤由形态一致、小至中等大的圆形或多边形上皮样细胞组成,多呈单个或狭窄的条索状排列,夹杂于大量嗜伊红色、玻璃样变的胶原纤维之间.部分区域中也可排列成巢状、片状或腺泡状.高倍镜下,瘤细胞的胞质透亮或嗜伊红色,核异型性不明显,核分裂象也少见(＜1/10HPF).另在2例肿瘤的局部区域,细胞密度明显增加,核有显著的异型性,核分裂象也易见,类似经典的纤维肉瘤.免疫组织化学标记显示,瘤细胞弥漫强阳性表达波形蛋白,灶性或弱阳性表达上皮膜抗原,不表达细胞角蛋白、S-100蛋白、HMB45、肌动蛋白、结蛋白、CD34、bcl-2、CD30和白细胞共同抗原(LCA)等标记.随访6例,3例复发,1例发生肺部转移.结论硬化性上皮样纤维肉瘤是纤维肉瘤的一种少见亚型,尽管瘤细胞异型性不明显,核分裂象也少见,但肿瘤可发生局部复发及远处转移,临床上应视为一种低至中度恶性的软组织肉瘤处理,组织学上则应注意与一些具有上皮样形态和硬化性间质的肿瘤相鉴别.     

上皮样肉瘤样血管内皮瘤的临床病理学观察

目的 探讨上皮样肉瘤样血管内皮瘤的临床病理学特征和鉴别诊断.方法 回顾性分析3例上皮样肉瘤样血管内皮瘤的临床表现、组织学特点和免疫表型.结果 3例均发生于成年男性,分别因左颈部肿块、髂部疼痛和双侧颈肩区复发性肿块就诊.镜下观察肿瘤由梭形和上皮样的细胞组成,两种细胞在形态上有移行.瘤细胞呈片状、模糊结节状或交织条束状排列,间质伴有胶原化.1例于结节中央可见凝固性坏死,形态上类似上皮样肉瘤.3例肿瘤内均无明显的血管形成,但其中1例于局灶区域可见胞质内空泡形成,类似上皮样血管内皮瘤.免疫组织化学标记,瘤细胞同时表达上皮性标记和内皮标记.3例均经手术切除,其中2例患者术后恢复良好,随访18个月和14个月均健在,无局部复发或远处转移,另1例术后6年内复发5次.结论上皮样肉瘤样血管内皮瘤属于一种少见的中间型血管内皮瘤,兼具上皮样肉瘤和上皮样血管内皮瘤的部分形态.仅凭光镜形态有时较难确定其内皮细胞分化,必须借助于免疫组织化学标记.上皮样肉瘤样血管内皮瘤与上皮样血管内皮瘤关系较为密切,可能是后者的一种富于细胞性梭形细胞变型.

Abstract：

Objective To study the clinicopathologic features and differential diagnosis of epithelioid sarcoma-like hemangioendothelioma (ES-H). Methods The clinical, radiologic and pathologic features of three cases of ES-H were analyzed. Results All the 3 cases occurred in male adults. The age ranged from 44 to 53 years. The presentations included left neck mass, iliac pain and bilateral shoulder masses. Histologically, ES-H was composed of a mixture of spindle and epithelioid tumor cells. Transition between the two cell types was demonstrated. The tumor cells were arranged in compact sheets, vague nodules or intersecting fascicles, amongst a collagenous stroma. Central coagulative necrosis was identified in one case, reminiscent the morphology that seen in epithelioid sarcoma. There was no evidence of angiogenesis, though focal presence of intracytoplasmic vacuoles was seen in one case, as in classic examples of epithelioid hemangioendothelioma. Immunohistochemical study showed that the tumor cells expressed both epithelial (AE1/AE3, CAM5.2 and epithelial membrane antigen) and endothelial (CD31,Fli-1 and factor Ⅷ-related antigen ) markers. Two of the cases were also positive for CD34. All of the patients were treated by surgical resection. Two patients remain well at 14-month and 9-month follow up,respectively. The remaining patient had repeated local recurrences during a 6-year period. Conclusions ES-H represents a rare morphologic type of hemangioendothelioma. It has some overlapping histologic features with epithelioid sarcoma and epithelioid hemangioendothelioma. The endothelial nature of ES-H is difficult to be verified on the basis of morphologic examination alone. Confirmation of the diagnosis with immunohistochemistry is necessary. ES-H is likely related to epithelioid hemangioendothelioma and may represent a cellular spindie cell variant of epithelioid hemangioendothelioma.     

C-Kit-positive metastatic malignant pigmented clear-cell epithelioid tumor arising from the kidney in a child without tuberous sclerosis

We report the first pediatric case of malignant pigmented epithelioid clear-cell tumor arising from kidney; the lesion occurred in a 12-year-old girl without tuberous sclerosis. The tumor was widely metastatic to the retroperitoneum and chest, and the patient died of the disease 9 months after diagnosis, despite active chemotherapy. Pigmented epithelioid clear-cell tumor of the kidney is a rare variant of epithelioid angiomyolipoma and a member of the family of perivascular epithelioid cell tumors (PEComas). The tumor demonstrated overlapping features between clear-cell sugar tumor and epithelioid variant of angiomyolipoma. Tumor cells were positive for HMB-45 expression, negative for any evidence of muscular differentiation, and contained melanin pigment and premelanosomes in the cytoplasm. Diffuse C-Kit (CD117) positivity was identified throughout the tumor. This is the first report of C-Kit–positive malignant PEComas.     

Angiosarcoma of the uterus: report of 2 new cases with deviant clinicopathologic features and review of the literature

A few cases of uterine angiosarcoma have been detailed in the literature: 2 new cases are herein described featuring some unusual clinical or phenotypic differences compared with previously published cases, such as occurrence in premenopausal age or a poorly differentiated histology. The patients were 35 and 81 years old, and both presented with extrauterine spread and evidence of distant metastases. Grossly, they were fleshy, hemorrhagic, and necrotic. Microscopically, they were made up of poorly differentiated, epithelioid, or spindle cells. In one case, the neoplastic growth deceitfully recalled a poorly differentiated leiomyosarcoma showing focal rudimentary endothelial differentiation. The other index case was characterized by a more pronounced vascular pattern, the neoplasm was composed of spindle cells arranged in loose channels. A diffuse immunopositivity for CD31, CD34, and factor VIII–related antigen was detected. Tumor cells were negative for other tested antigens including keratins, desmin, actins, and H-caldesmon. One patient died shortly during the follow-up, whereas the other is alive with evidence of disseminated disease. Occurrence in childbearing age or a deviant histologic pattern, as documented in this report, may be added to the clinicopathologic spectrum of uterine angiosarcoma.     

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.     

Pathological characteristics of liver biopsies in eight patients with hepatic epithelioid hemangioendothelioma

We aim to investigate the pathological characteristics of liver biopsies and their implications for the prognosis of hepatic epithelioid hemangioendothelioma (HEHE). Clinical data of eight patients (5 male, 3 female) with HEHE were analyzed retrospectively. Expression of CD34, FVIII, AE1/AE3, Hepa-par1, GPC3, CK19 and the proliferation index marker Ki-67 were determined by immunohistochemical staining. The clinical pathological features and effects of treatment on prognosis were investigated. Among the eight patients, four did not exhibit significant symptoms, while four showed symptoms such as abdominal distension, aversion to greasy food and mild fever. Two patients had single liver lesions, while multiple lesions were observed in six cases, in which the tumor cells exhibited spindle, irregular or epithelioid morphology, with scattered, streaked and nested distribution. Individual luminal cells were also visible, containing red cells and accompanied by mucoid or fibrous stroma. All cases were CD34 positive, one case was FVIII factor negative, two cases were AE1/AE3 positive, Ki-67 staining exceeded 15% in two cases, and nuclear fission was visible in two cases. Patients with nuclear fission and Ki-67 > 15% died within 2 years after artery embolization, liver transplantation without relapse was observed in two cases and one case survived with the tumor. The other patients without cellular atypia, without nuclear fission and with Ki-67 < 10% did not relapse during the 2-5 years of follow-up. HEHE can be diagnosed according to hematoxylin and eosin morphology and immunohistochemical characteristics in biopsies before treatment allowing the selection of different treatment protocols based on pathological characteristics.