Epithelioid angiosarcoma of bone:a report of two cases and review of literatures

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.     

小肠转移性上皮样血管肉瘤1例临床病理分析

目的探讨小肠上皮样血管肉瘤(epithelioid angiosarcoma,EA)的临床病理特征、免疫表型、诊断、鉴别诊断。方法分析1例小肠转移性EA的临床资料,观察其组织学形态、免疫表型及FISH检测,并复习相关文献。结果患者右大腿皮肤EA术后1年,小肠镜示肠腔内黏膜呈息肉状突起,组织学检查见肿瘤细胞呈上皮样条索状分布,部分形成血管样裂隙,细胞呈乳头状生长,细胞圆形或多边形,空泡状胞质,内含红细胞,其组织形态呈典型的EA。免疫组化标记CD31、ERG、Fli-1、CK均阳性。FISH检测CAMTA1基因断裂阴性。结论小肠原发性或转移性EA临床罕见,预后差,需与低分化癌或转移癌、胃肠道间质瘤等鉴别。     

骨上皮样血管内皮瘤4例及文献复习

目的探讨4例骨上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理特点、诊断和鉴别诊断。方法对4例原发于骨的EHE临床资料、组织学、免疫组化进行观察分析并随访,结合文献讨论。结果 4例骨EHE中男性和女性各2例,中位年龄24岁,发生部位包括右肩胛骨、腰4～5椎体、胫骨下段和左足。临床症状主要为疼痛,影像学资料提示溶骨性改变;镜下示上皮样的肿瘤细胞呈巢状、索状或小梁状排列,肿瘤细胞呈圆形、多边形间或梭形,常见明显的胞质内空泡并伴有周围间质的玻璃样或黏液样变性。免疫表型:瘤细胞CD31、CD34、α-SMA均阳性(100%),CKpan有1例阳性(25%),EMA均阴性(100%),肿瘤增殖标记Ki-67呈低表达。结论原发于骨的EHE是一种少见的低度恶性的血管源性肿瘤,它的诊断要结合临床和影像学资料,依据其病理学特征及相关的免疫组化特点综合判断,需与骨转移性癌、上皮样血管瘤、上皮样血管肉瘤(epithelioid angiosarcoma,EAS)等鉴别。     

Epithelioid hemangioendothelioma of bone: a study of 4 cases and review of literature

目的 探讨4例骨上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理特点、诊断和鉴别诊断.方法 对4例原发于骨的EHE临床资料、组织学、免疫组化进行观察分析并随访,结合文献讨论.结果 4例骨EHE中男性和女性各2例,中位年龄24岁,发生部位包括右肩胛骨、腰4～5椎体、胫骨下段和左足.临床症状主要为疼痛,影像学资料提示溶骨性改变;镜下示上皮样的肿瘤细胞呈巢状、索状或小梁状排列,肿瘤细胞呈圆形、多边形间或梭形,常见明显的胞质内空泡并伴有周围间质的玻璃样或黏液样变性.免疫表型:瘤细胞CD31、CD34、α-SMA均阳性(100%),CKpan有1例阳性(25%),EMA均阴性(100%),肿瘤增殖标记Ki-67呈低表达.结论 原发于骨的EHE是一种少见的低度恶性的血管源性肿瘤,它的诊断要结合临床和影像学资料,依据其病理学特征及相关的免疫组化特点综合判断,需与骨转移性癌、上皮样血管瘤、上皮样血管肉瘤(epithelioid angiosarcoma,EAS)等鉴别.     

肾透明细胞肉瘤的临床病理及免疫表型特征

目的 探讨肾透明细胞肉瘤（clear cell sarcoma of the kidney,CCSK）的临床病理特点、免疫表型特征及鉴别诊断。方法 应用HE和免疫组化vimentin、bcl-2、desmin、S-100蛋白、CD99、CD34、CDll7、CK、EMA染色,观察2例CCSK的病理组织学形态,并复习文献。结果 镜下见瘤细胞为上皮样或短梭形,被分枝状纤维血管间质分隔成巢团状,部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示：瘤细胞vimentin和bcl-2弥漫阳性,余为阴性。结论 CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。     

原发性甲状腺上皮样血管肉瘤1例报告

目的:观察1例原发性甲状腺上皮样血管肉瘤患者的临床病理特征,并结合相关文献进行讨论。方法和结果:本例原发性甲状腺上皮样血管肉瘤患者行术中快速冰冻切片及常规石蜡切片,HE染色和网状纤维染色、并行免疫组化检查。镜下见,肿瘤主要由实性片状或巢状排列的上皮样细胞组成,肿瘤细胞核较大,呈空泡状,核仁明显,部分肿瘤细胞胞质内可见含有红细胞的空泡或小管腔形成;可见迷路状血管,网状纤维染色能清晰显示这些管腔结构。免疫组化染色,肿瘤细胞CD31、CD34、AE1/AE3阳性,TG、TTF-1、calcitonin、CK19、S-100、EMA和HMB45阴性,Ki-67高表达。结论:原发性甲状腺上皮样血管肉瘤是一种少见的恶性肿瘤,致死率高。确诊主要依靠组织学和免疫组化检查,临床要与未分化癌、低分化癌及上皮样血管内皮瘤等鉴别。     

骨原发性上皮样血管肉瘤3例临床病理分析

目的探讨骨原发性上皮样血管肉瘤(epithelioid angiosarcoma,EA)的临床病理特征及鉴别诊断要点。方法对3例骨原发性EA进行HE及免疫组化En Vision两步法染色,并结合文献复习。结果 3例均为男性,年龄39~69岁,平均55岁,病变均为多发。镜检:肿瘤细胞表现为明显的上皮样特点,异型性明显,胞质丰富、微嗜酸,泡状核,核仁明显,可见核分裂及病理性核分裂。肿瘤细胞呈多种方式排列,可实性生长,也可形成网络状、迷路状或乳头状结构。肿瘤性血管腔隙的形成及含有红细胞的胞质内空泡是肿瘤的特征性结构。肿瘤出血、坏死常见,间质中可见多少不等的淋巴细胞浸润。免疫表型:3例肿瘤细胞表达5种或至少3种检测的血管内皮细胞标志物(CD31、CD34、FLI-1、FactorⅧ和ERG),vimentin均(﹢),2例CK局灶(+),Ki-67增殖指数5%~30%。本组中1例行穿刺活检明确诊断后未做手术,行化疗,3个月后死亡;2例行病灶完整切除术,其中1例随访28个月未见复发,另1例失访。结论骨原发性EA是呈上皮样形态的血管源性高度恶性肿瘤,内皮细胞标志物检测在鉴别诊断中具有重要意义。     

淋巴结原发上皮样血管内皮瘤1例及文献复习

目的 探讨淋巴结原发上皮样血管内皮瘤的诊断及鉴别诊断。方法 1例淋巴结原发上皮样血管内皮瘤进行HE切片、组织化学、免疫组化等观察，并复习文献进行讨论。结果 光镜下肿瘤细胞呈多角形或类圆形上皮样，无明显核分裂象，组织结构多种，呈单个细胞条索、实性巢状及管腔样结构排列，瘤细胞形成小的细胞内管腔，见胞质内空泡，并见红细胞，此特征为细胞原始管腔结构。玻璃样间质。网状纤维染色显示血管腔隙结构。肿瘤细胞表达Vim、CD31、FⅧRAg，少数细胞表达CD34。结论 淋巴结原发上皮样血管内皮瘤是极少见的低度恶性血管性肿瘤，可出现多种组织结构，有一定的特征性。应与转移性癌、转移性恶性黑色素瘤、上皮样血管肉瘤、上皮样血管瘤、上皮样肉瘤及硬化性上皮样纤维肉瘤等鉴别。     

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。     

心包原发性恶性间皮瘤的临床病理分析

目的：探讨心包原发性恶性间皮瘤的临床病理特征。诊断与鉴别诊断要点。方法：对4例心包原发性恶性间皮瘤进行临床病理分析。光镜及免疫组化染色观察并复习有关文献。结果：男3例,女1例,平均年龄42岁,3例呈局限型,1例为弥漫浸润型。组织学上可表现为肉瘤样梭形细胞型,上皮样型及双相型,免疫组化染色显示肉瘤样梭形细胞表CK、vimentin;上皮样型瘤细胞表达HBME1、CK。结论：原发于心包的恶性间皮瘤罕见,预后极差。临床常被误诊,其组织形态亦复杂多样,应注意与心包的良性增生性病变,心包转移性腺癌和梭形细胞肿瘤等相鉴别。     

Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity

Two rare cases of intestinal epithelioid angiosarcoma arising in the sigmoid colon and small intestine are reported. The small intestinal tumours were located in the duodenum and upper jejunum. All tumours showed reddish-black protuberant masses with comparatively clear margins. Histology reveals solid and epithelioid growth of large polygonal cells, mimicking undifferentiated carcinoma. Vascular differentiation such as lumina containing red cells and intracytoplasmic vacuolization is noticed. Intense immunoreactivity toUlex europaeus agglutinin I and JC70 (CD31), and sporadic positive reaction to factor VIII related antigen were detected in one case but not in the other. However, both tumours have cells which show intense endothelin-1 immunoreactivity. Positive immunostaining occurred with low molecular weight keratins suggesting epithelial differentiation and suggested epithelioid angiosarcoma as a diagnosis, a specific entity within angiosarcoma. Because of their histological features, epithelioid angiosarcomas may be confused with undifferentiated carcinomas. Endothelin-1-like immunoreactivity seems to be a good marker for this type of angiosarcoma, even when the tumour cells lack usual marker substances for endothelial cells.     

Primary epithelioid angiosarcoma of the pleura: a case report and review of literature

Primary angiosarcoma of the pleura is an extremely rare malignancy. Herein, we report the case of an elderly Chinese patient with primary left pleural epithelioid angiosarcoma. The 76-year-old man presented with a 4-month history of a cough with sputum expectoration and weight loss of 4 kg within one month. A chest scan showed a massive oval-shaped mass in the left pleural cavity. We then performed a left thoracotomy for tumor resection and surgical exploration. Histological examination of the resected specimen showed few viable tumor cells with significant atypia; tumor cells had large nuclei and prominent nucleoli and were arranged in a crack-like, sheeted pattern. Moreover, there was a significant amount of fibrinous exudates, hemorrhage, degeneration, and necrosis. With immunohistochemical analysis, tumor cells had strong expression of CD31, CD34, FLI-1, vimentin. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma.     

Two cases of epithelioid angiosarcoma involving the thyroid and a brief review of non-Alpine epithelioid angiosarcoma of the thyroid

Epithelioid angiosarcoma involving the thyroid is a rare entity, more often described in the Alpine region. Two cases of epithelioid angiosarcoma that affected the thyroid in patients from a non-Alpine location were diagnosed during a 10-year period in our department. The first case occurred in an 89-year-old Chinese man with a history of longstanding goiter, whereas the second case involved a 74-year-old Chinese man with a history of angiosarcoma of the scalp. On histologic examination, both thyroid tumors were composed of plump epithelioid cells with vesicular chromatin and prominent nucleoli, forming vascular structures and solid sheets. Positive staining for CD31 and factor VIII-related antigen confirmed endothelial differentiation in both cases. Both patients died within 5 months following the diagnosis of thyroid disease. The relationship of the scalp angiosarcoma and thyroid disease of the second patient is unclear. A brief review of non-Alpine primary thyroid epithelioid angiosarcoma is presented.     

Multicentric contiguous variant of epithelioid angiosarcoma of the bone. A rare variant showing angiotropic spread

Epithelioid angiosarcoma of the bone is a rare tumor and is a diagnostic challenge. Here we present an autopsy case of a 62-year-old man with multifocal osteolytic lesions in the extremities and the pelvis. The initial diagnosis of a tibial biopsy was poorly differentiated adenocarcinoma. On the occasion of autopsy, a fungating thrombotic nodule was found at the anterior wall of the right atrium, and small hemorrhagic infarcts with tumor thrombi were found in the lung. Histologically, the above lesions were identical to the former tibial biopsy and they showed large eosinophilic epithelioid cells with irregular ovoid nuclei and prominent eosinophilic nucleoli. Rare intracytoplasmic lumina were identified. Immunohistochemically, the tumor cells were positive for cytokeratins (CAM5.2 and AE1/AE3), CD31, factor VIII-related antigen, and vimentin. This case showed angiotropic spread of the tumor only to the right atrium and the lung, with no solid mass in other organs. Multicentric epithelioid angiosarcoma of the bone is a pitfall in pathological diagnoses, especially if a strong radiological impression of metastatic carcinoma is provided. Therefore, pathologists should be aware of this rare variant.     

Epithelioid angiosarcoma at chest wall which needs to be carefully distinguished from malignant mesothelioma: report of a rare case

Angiosarcoma is a malignant soft tissue tumor the cells of which variably recapitulate the morphologic and functional features of normal endothelium. Most lesions are located in the deep muscles of the lower extremities followed by the arm, trunk and head and neck. Herein we present a case of epithelioid angiosarcoma which is a variant of angiosarcoma at chest wall in a 73-year-old female. Morphologically, the tumor cells are arranged predominantly in luminal structures which can be seen in both angiosarcoma and malignant mesothelioma. Most of the tumor cells are large rounded “epithelioid” cells with abundant eosinophilic cytoplasm which can be also seen in both tumors. The epithelioid of cytomorphology and the localization at chest wall of this case may remind of a diagnosis of malignant mesothelioma which should be carefully distinguished from epithelioid angiosarcoma from imaging and morphology. CT scanning of the patient shows a mass at her chest wall, the majority of which is around the rib but not inside the lung which indicates a tumor originates more likely from soft tissues of chest wall but not pleura. Immunohistochemical staining shows that the tumor cells are positive for cytokeratin, CD31, Vimentin and WT1, and negative for CEA, TTF-1, Calretinin, Mesothelial Cell (MC), CD56, CK19, and Hepatocyte. Thus this case is diagnosed as epithelioid angiosarcoma but not malignant mesothelioma. From this case we suggest that carefully reading and understanding of the imaging are a very important clue for appropriate diagnosis. A misdiagnosis may occur on the basis of misunderstanding of tumor localization and a consequent inappropriate immunohistochemical staining programme.     

Postradiation angiosarcoma of the small intestine: a case report and review of literature

Postradiation angiosarcoma is typically a high-grade sarcoma that presents mainly in the skin and superficial tissues. Postradiation angiosarcoma arising in the small intestine is rare with only 11 cases documented in the English-language literature. Herein, we report a postradiation angiosarcoma of the small intestine 9 years after radiotherapy for uterine cervical adenocarcinoma. The patient presented with symptoms of intestinal obstruction. At exploratory laparotomy, tumor nodules involved the small bowel. Microscopically, the neoplasm was composed of spindled and epithelioid cells arranged in solid aggregates and focally forming vascular channels. The diagnosis of angiosarcoma was confirmed immunohistochemically by tumor cell expression of CD31, CD34, and factor VIII-related antigen. The patient died 10 months after laparotomy. The diagnosis of PRA should be entertained for any poorly differentiated neoplasm arising in a previously irradiated site. The correct diagnosis of PRA depends upon histomorphologic identification of vascular differentiation, coupled with immunohistochemical expression of endothelial-related markers.     

Recurring bone epithelioid hemangioma

Bone vascular tumors are very rare. Epithelioid types are described according to their architecture, their degree of vascular differentiation, and their cytonuclear atypia. The include epithelioid hemangioma, epithelioid hemangioendothelioma, and angiosarcoma. We report a case of L4 corpus vertebral bone epithelioid hemangioma. The patient was a 25-year-old man with a tumor that recurred twice. The lesion was characterized by a vascular lumen lined by cells with regular nuclei and inflammatory infiltrates. Capillaries were lined by prominent epithelioid endothelial cells, associated with CD31+ and cytokeratin-.     

Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review

Angiosarcoma developing within a schwannoma is rare, and only 8 cases have been reported in the literature. Its association with a plexiform schwannoma has never been reported. We describe an epithelioid angiosarcoma arising in a plexiform schwannoma of the sciatic nerve in a 73-year-old man not known to have von Recklinghausen disease. Grossly, the tumor forms a long tubular mass with a multinodular growth pattern on the cut surface. Microscopically, these nodules display characteristics of a schwannoma, including Antoni A, Antoni B, and Verocay bodies. Adjacent to these nodules are high-grade anaplastic epithelioid cells growing in solid sheets, cords, and nests with focal vasoformative channels. The anaplastic cells are immunoreactive to CD31, CD34, and factor VIII related antigen, supporting their endothelial differentiation. Local recurrence and multiple pulmonary metastasis occurred 3 months after complete excision of the tumor. This case demonstrates the first occurrence of an epithelioid angiosarcoma in a deep-seated plexiform schwannoma with an aggressive clinical behavior.     

Extracutaneous angiosarcomas metastatic to the lungs: clinical and pathologic features of twenty-one cases.

Angiosarcomas are rare malignant vascular tumors with a high rate of metastasis involving lungs (most commonly), liver, regional lymph nodes, bone, and other sites. In this study, we have reviewed the clinical presentation and histopathology of 21 cases of extracutaneous angiosarcoma metastatic to the lungs. Tumors with exclusively pleural involvement were excluded. Patients presented with dyspnea, chest pain, and/or hemoptysis lasting a few weeks to months. Radiologically, the most common finding comprised multiple peripheral lung nodules (57%), often accompanied by infiltrates. For 11 cases (52%), the primary tumor was not identified at the time of presentation. Vasoformative areas were identified in 15 cases (71%). Nine cases comprised spindle cells (43%), two contained epithelioid cells (9.5%), and 10 consisted of both spindle and epithelioid cells (48%). Nuclear pleomorphism was at least moderate in all cases. However, five tumors contained regions of minimal nuclear atypia. Hemorrhage, siderophages, and fibrosis were commonly present. Immunohistochemical staining (IHS) was performed on 14 cases. Thirteen tumors showed reactivity for vascular markers. Tumor cells reacted for Von Willebrand factor in 13 of 14 cases, and CD31 and CD34 were each positive in 2/2 cases. Two cases (of nine examined) also expressed cytokeratins. Because the tumor often first presented in the lungs before the primary sarcoma was identified, the clinical impression included both benign and malignant entities. For patients with primary cardiac tumors, symptoms referable to the primary tumor complicated the clinical presentation, and radiologic evaluation supported a clinical diagnostic impression of non-neoplastic pericarditis. Thus, angiosarcoma in the lung may elude diagnosis until histopathologic evaluation of the lung biopsy.