右心房血管肉瘤1例

患者,男,40岁。因活动后胸闷气急2个多月就诊。体检心界向左扩大。外院超声提示:右心房侧壁5.6cm×1.0cm略强回声团块影。CT示(图1、2):右心房侧壁可见局限性增厚,并形成软组织肿块,肿块内密度均匀,CT值约35HU,肿块突向心腔内,与心包膜之间关系较密切,增强后肿块中度强化,约75H     

Radiation-associated angiosarcoma of the breast with initial presentation as non-mass enhancement on MRI

Radiation-associated angiosarcoma of the breast (RAASB) is a rare and aggressive malignancy occurring after radiation therapy as part of breast cancer treatment. RAASB usually presents several years after prior radiation and typically involves the skin with or without involvement of the parenchyma. Most RAASB are detected as cutaneous changes on physical exam. Herein, we present a unique case of a clinically occult RAASB diagnosed as non-mass enhancement on annual surveillance breast MRI.     

乳腺根治术继发淋巴水肿后上臂皮肤血管肉瘤1例及文献复习

目的探讨乳腺根治术继发淋巴水肿后的上臂皮肤血管肉瘤,即STS的临床病理学特征及诊断、鉴别诊断要点。方法对1例STS进行病理组织学及免疫组织化学染色观察,并复习相关文献。结果镜下以充满红细胞的大小不等的囊腔,相互沟通的不规则的管腔,部分由梭形和上皮样细胞构成的实性区域为特征。不规则的管腔内衬覆异型瘤细胞,可见单个或多个瘤细胞形成空腔内含红细胞。免疫组织染色肿瘤细胞CD34阳性、CD31阳性,Ki-67部分阳性,AE1/AE3为阴性表达。结论 STS是乳腺血管肉瘤的一种特殊类型,免疫组织化学检测有助于诊断和鉴别诊断。     

Predictors of survival in malignant aortic tumors

ObjectiveMalignant aortic tumors (MATs) are exceedingly rare, and a comprehensive review of clinical and therapeutic aspects is lacking in the literature. The aim of this study was to analyze all known cases of MATs and to identify predictors of patients' survival.MethodsAll patients diagnosed with an aortic tumor treated in a single center along with all case reports and reviews available in the literature through a specific PubMed search using keywords such as “malignant” and “aorta” or “aortic,” “tumor,” or “sarcoma” or “angiosarcoma” were analyzed. The tumor's primary location, clinical presentation, histologic subtype, and treatment choice were examined. Survival at 1 year, 3 years, and 5 years and the possible preoperative and operative outcome predictors were evaluated using Kaplan-Meier analysis with a log-rank test and by Cox regression for multivariate analysis.ResultsIn addition to the 5 cases treated in our center, 218 other cases of MAT were reported in the literature from 1873 to 2017. The mean age of the patients was 60.1 ± 11.9 years, and the male to female ratio was 1.59:1. The median overall survival from diagnosis was 8 (7-9) months; 1-, 3-, and 5-year survival rates were 26%, 7.6%, and 3.5%, respectively. Chronic hypertension (P = .03), fever (P = .03), back pain (P = .01), asthenia (P = .04), and signs of peripheral embolization (P = .007) were significant predictors of a poor result. Histologic subtypes had a different impact on survival, with no statistical significance. Compared with other treatment strategies, combined surgical-medical therapy had the best impact on the median survival rate (surgical-medical, 12 [8-24] months; medical, 8 [5-10] months; surgical 7 [2-16] months; no treatment, 2 [0.5-15] months; P = .001). Analyzing exclusively medical approaches, chemotherapy and radiotherapy had the best impact on median survival rate compared with untreated patients (chemotherapy-radiotherapy, 18 [10-26] months; radiotherapy, 16 [8-20] months; chemotherapy, 10 [7-24] months; no medical treatment, 6 [2-16] months; P = .005); these data were not sustained by multivariate analysis.ConclusionsAortic tumors are a malignant pathologic condition with a short survival rate after initial diagnosis. Survival is further diminished in the presence of clinical factors such as hypertension, fever, back pain, asthenia, and signs of peripheral embolization. Combined surgical and medical treatment, particularly with chemotherapy and radiotherapy, has shown the highest survival rate.     

Mutant c-Ki-ras p21 protein in chemical carcinogenesis in humans exposed to vinyl chloride

Mutations inras oncogenes and expression of their encoded p21 protein products are believed to play an important role in carcinogenesis in humans. Detection of mutant p21 proteins in serum may be a useful molecular epidemiologic biomarker with which to study this process, and workers with heavy exposure to vinyl chloride (VC) represent a model population for such study. We studied the occurrence of a specificras mutation (Asp 13 c-Ki-ras) by oligonucleotide hybridization and the expression of the corresponding p21 protein in tumor tissue and serum by immunohistochemistry and immunoblotting with monoclonal antibodies in five individuals with heavy exposure to VC and resultant angiosarcomas of the liver (ASL). Four of five (80 percent) of the cases of ASL were found to contain the mutation and to express the corresponding mutant protein in their tumor tissue and serum. Serum expression of the mutant protein also was examined in nine VC-exposed workers with liver angiomas and 45 VC-exposed workers with no evidence of liver neoplasia; eight of nine (89 percent) of the former and 22 of 45 (49 percent) of the latter were also positive for the mutant p21 in their serum. However, serum immunoblotting results for 28 age-gender-race matched, unexposed controls were all negative. Stratification by years of VC exposure showed a significant linear trend (P<10^–5) for the occurrence of the serum mutant p21 protein with increasing duration of exposure. These results suggest that detection of serum mutant p21 protein can be a valid surrogate forras gene expression at the tissue level. Further, serum mutant p21 may be a useful molecular epidemiologic biomarker for the study of chemical carcinogenesis in humans exposed to VC and possibly for the study of other mutantras-related human cancers.This work was supported by the US National Institute for Occupational Safety and Health, US Environmental Protection Agency, US National Cancer Institute, E.I. DuPont de Nemours & Co., American Cancer Society, Lucille P. Markey Charitable Trust, US National Institutes of Health, Association pour le Recherche sur le Cancer, and le Groupment de Enterprises Francaises pour les Lutte contre le Cancer.     

Angiosarcoma after revision total knee arthroplasty

BackgroundHemarthrosis after total knee arthroplasty (TKA) is a relatively rare complication. Although most cases are effectively treated with conservative therapy, some cases require angiographic embolization or surgical intervention. Angiosarcoma is a rare malignant tumor derived from the vascular endothelium with neovascular hyperplasia and mainly arises in the skin and superficial soft tissue, and less frequently in deep soft tissue and bone. Although malignant neoplasms such as angiosarcoma in the vicinity of orthopedic implants were reported, the causal relationship between development of the malignant tumor and the orthopedic implant is widely debated in the literature.Case presentationWe report the case of a 68-year-old female with angiosarcoma that developed in the knee joint 2 years after revision TKA. The patient exhibited severe persistent bleeding, which reached 1000–1400 ml per day for 4 months. Histological analysis of the synovial tissue in the knee joint showed large cells with nuclear atypia. Immunohistochemical staining showed cells that were positive for CD31, CD34, and D2-40, and she was diagnosed with angiosarcoma. The patient underwent an amputation at the level of the thigh, and her general condition immediately improved after the operation. The patient did not exhibit bleeding from the site of amputation, and no local recurrence or distant metastases were detected 1 year after the amputation.ConclusionsTo the best of our knowledge, this represents the first report of angiosarcoma 2 years after revision TKA. Further careful follow up is needed, given the high-grade malignancy.     

Other vascular tumors

Vascular tumors are rare in children and adults. Classification of these tumors has been difficult, especially in the pediatric population, due to the rarity of these lesions, the unusual morphologic appearance, their diverse clinical behavior, and no independent stratification for pediatric tumors. In 2013, The World Health Organization updated the classification of soft tissue vascular tumors. Pediatric tumors were not independently stratified and the terminology was mostly left unchanged, but the intermediate category of tumors was divided into locally aggressive and rarely metastasizing. These tumors are treated with multimodality therapy and therefore need the guidance of an interdisciplinary team for best care.     

Hypoxia accelerates the progression of angiosarcoma through the regulation of angiosarcoma cells and tumor microenvironment

Background

Angiosarcoma is a rare malignant tumor with a poor prognosis. It is known that hypoxic condition activates tumor progression in several cancers. Additionally, hypoxic tumor microenvironment accelerates immune escape. However, the presence and significance of hypoxia in angiosarcoma has not been adequately investigated.