Primary angiosarcoma of the small intestine: radiological appearances

Angiosarcoma of the small intestine is a rare malignancy that is clinically manifested by gastrointestinal bleeding, In the few cases prev iously reported discussion mainly focused on the recent contribution of immunocytochemistry in tumor identification. It seems that the radiologic appearance of the tumor is poorly documented, and its preoperative imaging evaluation ill-recognized. A 75-year-old male with an intestinal angiosarcoma is presented. Emphasis is g veil on the radiologic features of the tumor and the possibility of its preoperative demonstration when usingnteroclysis. Correspondence to: N. C. Gourtsoyiannis     

上皮样血管肉瘤4例临床病理分析

目的 探讨上皮样血管肉瘤的临床病理学特征。方法 对4例上皮样血管肉瘤进行光镜和免疫组化检测，1例做电镜观察。结果 男性3例，女性1例，平均61岁。头颈部2例，膀胱1例，上臂1例。癌细胞上皮样增生呈片状，细胞大，多角形，胞质丰富，嗜酸，核大，核仁明显，核分裂象易见，可见胞质内空泡形成，偶见含有单个红血球，多数病例能找到互相吻合的血管腔隙，其内衬非典型的内皮细胞，间质易见灶性淋巴细胞浸润和含铁血黄素。Vim、CD31、CD344例均（ ）；FⅧAg及低分子角蛋白（CK7）2例（ ）；S-100、HMB45、EMA、HHF35和SMA4例均（-）。电镜观察1例见胞质内含丰富的微线和少许W-P小体。3例随访1-3年，2例有转移，1例转移至局部淋巴结，1例转移至肺，1例术后半年死亡。结论 上皮样血管肉瘤是血管肉瘤的一种特殊类型，熟悉其形态特征，对避免误诊具有重要意义。     

皮下软组织上皮样血管肉瘤2例临床病理观察

目的探讨上皮样血管肉瘤(EAS)的临床病理特点、诊断和鉴别诊断。方法对2例上皮样血管肉瘤的组织病理学、免疫组化结果进行分析,结合文献进行讨论。结果镜下肿瘤由细胞体积大、胞质丰富、具有上皮样特征的瘤细胞构成。瘤细胞呈弥漫片状、腺管状、单排条索状排列。免疫组化:肿瘤细胞CD34和FⅧAg(+),AE1/AE3和EMA部分(+)。结论上皮样血管肉瘤是一种罕见的高度恶性肿瘤,诊断和鉴别诊断主要依赖其病理形态学特点结合免疫表型。     

Pseudoangiomatous stromal hyperplasia of the breast in a 10-year-old girl

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a benign lesion, characterized by a dense proliferation of stromal mesenchymal cells of myofibroblastic origin forming empty, slitlike channels. The development of PASH is subject to hormonal influence and is most commonly seen in premenopausal women. In adolescent patients, PASH is rarely reported. Here we report a case of PASH presenting in a 10-year-old girl. After the presentation and inconclusive radiographic findings, incisional biopsy revealed a soft nodule with histopathologic features characteristic of PASH. Immunoperoxidase studies showed positive immunoreactivity for CD34 and bcl-2 and negative immunoreactivity for CD31 and desmin, supporting the diagnosis of PASH. Because of the benign nature of the lesion, excisional biopsy was deferred to minimize damage to the patient's developing breast. To our knowledge, this case represents the youngest female patient with unequivocal PASH in reported literature.     

Bone marrow metastasis of angiosarcoma

We report a rare finding of bone marrow metastasis from an angiosarcoma. The patient was a 36-year-old man who initially presented with a high-grade angiosarcoma of the spleen and was treated with splenectomy and chemotherapy. He developed leukoerythroblastic anemia three years after splenectomy. Bone marrow biopsy revealed extensive infiltrate by angiosarcoma with typical features of spindle tumor cells and anastomosing vascular channels. The immunohistochemistry showed tumor cells positive for the endothelial markers of CD31, CD34, and von Willebrand factor. Angiosarcomas are rare and aggressive tumors. Although metastases occur commonly, bone marrow findings have been rarely documented. We have found in the literature two other cases of bone marrow metastasis of angiosarcoma, and all these patients had a primary tumor of the spleen. It would seem that splenic angiosarcomas have a virtually unique propensity for infiltration in the bone marrow.     

卵巢原发性血管肉瘤2例临床病理观察

目的探讨卵巢原发性血管肉瘤的临床病理特征及鉴别诊断。方法收集2例卵巢原发性血管肉瘤的临床资料,进行组织病理学观察和免疫组化染色,并复习文献。结果 2例患者年龄为36岁和37岁,平均年龄36.5岁;临床以腹胀、腹部肿块为主要症状。镜下肿瘤组织由上皮样及梭形细胞组成,轻到中度核异型性,瘤细胞呈实片状或形成大小不等的囊腔,部分小腔隙中含有红细胞。免疫组化:CD31、CD34和vimentin呈广泛强(+),上皮标记物CK(AE1/AE3)为(-)。结论卵巢原发性血管肉瘤罕见,预后差,极易误诊。     

Pleural cavity angiosarcoma arising in chronic expanding hematoma after pneumonectomy

A 52-year-old man received a left pneumonectomy for pulmonary squamous cell carcinoma without signs of recurrence after surgery. At age 68 years, a capsulated huge mass developed in the left pleural cavity, which was diagnosed as chronic expanding hematoma. Two years and 8 months after detection, the lesion began to invade the chest wall, and 10 months later, the patient died of active bleeding and direct compression of the heart by the lesion. At autopsy, the left thoracic cavity was occupied by a cystic and hemorrhagic mass infiltrating into the surrounding structures. In addition, scattered tumorous nodules were observed in the right lung. Histologically, angiosarcoma with irregularly anastomosing vessels lined with atypical endothelial cells was noted in the chronic expanding hematoma. The final diagnosis was pleural cavity angiosarcoma arising in chronic expanding hematoma and its metastases to the right lung.     

Combination therapy for scalp angiosarcoma using bevacizumab and chemotherapy: a case report and review of literature

Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a case report and review of the literature using bevacizumab and combination chemotherapy for angiosarcoma. The understanding of the effectiveness of combined therapy of bevacizumab and chemotherapy agents is still limited. The benefits of bevacizumab treatment for angiosarcoma will need to be weighed against the risks of venous thromboembolism in this population.Key Words: Angiosarcoma, chemotherapy, bevacizumab, angiogenesis inhibitor, combination therapy     

Uterine angiosarcoma associated with lymphangioleiomyomatosis in a patient with tuberous sclerosis complex: an autopsy case report with immunohistochemical and genetic analysis

A 41-year-old woman carrying a germline tuberous sclerosis complex 2 (TSC2) mutation, whose regular medical follow-up for tuberous sclerosis complex and tuberous sclerosis complex-associated lymphangioleiomyomatosis had continued for 2 years, had uterine angiosarcoma concomitant with uterine lymphangioleiomyomatosis. Immunohistochemically, the uterine angiosarcoma cells showed an extremely skewed lymphatic differentiation; they were diffusely immunopositive for CD31 but negative for other vascular endothelial markers including factor VIII and CD34 yet strongly immunopositive for lymphatic endothelial markers including D2-40 and Prox-1. Loss of heterozygosity analysis demonstrated that not only lymphangioleiomyomatosis and renal angiomyolipoma but also the uterine angiosarcoma had loss of heterozygosity on TSC2. Furthermore, direct sequencing revealed a TP53 mutation in the uterine angiosarcoma. Collectively, the findings suggest that combined dysfunction of the p53 and TSC2 tumor suppressor proteins may contribute to the development of uterine angiosarcoma in this rare clinical setting.     

CT诊断脾脏血管肉瘤1例

患者女,49岁,因“上腹部不适1个月”就诊.2007年曾接受血管瘤栓塞治疗术.患者一般状态可,左上腹压痛明显,脾脏增大,达肋下2.0 cm,质略硬.实验室检查未见异常.CT平扫示肝内多个类圆形不均匀低密度影,边缘尚清晰;胆囊内可见结石影;脾脏增大,密度不均,实质大部呈囊状改变,囊内可见不规则斑片、结节状软组织影(图1).腹膜后区未见明显肿大淋巴结.增强扫描肝脏内低密度影呈不均匀渐进性强化,由边缘向中央区填充;脾脏囊性病灶内斑片、结节影呈轻度强化改变,并随强化时间延长,密度增加(图2).影像诊断:肝脏多发血管瘤;脾脏恶性占位病变,源于脉管可能性大.行脾脏切除术,术后病理:脾脏血管肉瘤,浸透脾脏全层(图3).