hepatic angiosarcoma

肝血管肉瘤
  • 文章类型: Case Reports
    本研究旨在全面了解原发性肝血管肉瘤,一种罕见的侵袭性恶性肿瘤,专注于其诊断挑战和独特的成像特征。目的是通过计算机断层扫描和磁共振成像模式来描绘血管肉瘤的独特特征。有助于提高诊断精度,并为科学文献增加有价值的见解。我们介绍了一个25岁男性原发性肝血管肉瘤的病例,强调将其与其他血管肿瘤区分开来的挑战。
    This study aims to provide a comprehensive understanding of primary hepatic angiosarcoma, a rare and aggressive malignancy, focusing on its diagnostic challenges and unique imaging characteristics. The objective is to delineate the distinctive features of angiosarcoma through computed tomography and magnetic resonance imaging modalities, contributing to improved diagnostic precision and adding valuable insights to the scientific literature. We present the case of a 25-year-old male with primary hepatic angiosarcoma, emphasizing the challenges in distinguishing it from other vascular tumors.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    增强成像技术具有压倒性的优势,即非侵入性且足够灵敏,可以评估病变的微循环,从而使它们在肝脏病变的诊断中准确。不幸的是,关于罕见癌性疾病的影像学特征的研究和知识很少:肝血管肉瘤(HA)。
    在这项研究中,我们回顾性收集了6例同时接受超声造影(CEUS)和CT(CECT)检查的患者的数据,随后获得了明确的HA组织病理学诊断。我们通过比较CEUS和CECT图像来描述HA的成像外观。此外,我们从组织病理学和肿瘤发生的角度分析了这些影像学特征。该研究包括迄今为止接受CEUS检查的组织病理学证实的HA患者的数量最多(6例)。
    通过为读者提供全面的对比成像知识,尤其是CEUS,在HA的诊断中,我们的研究可以减少误诊,进一步改善治疗方案.
    UNASSIGNED: Enhanced imaging techniques have the overwhelming advantages of being noninvasive and sensitive enough to evaluate the microcirculation of lesions, thus making them accurate in the diagnosis of hepatic lesions. Unfortunately, there is very little research on and knowledge of the imaging features of a rare cancerous condition: hepatic angiosarcoma (HA).
    UNASSIGNED: In this study, we retrospectively collected the data of six patients who underwent both contrast-enhanced ultrasound (CEUS) and contrast-enhanced computed tomography (CECT), and subsequently obtained a definitive histopathologic diagnosis of HA. We described the imaging appearances of HA by comparing CEUS and CECT images. Furthermore, we analyzed these imaging characteristics from the perspective of histopathology and tumorigenesis. The study included the largest number (six) of histopathologically confirmed HA patients who had received CEUS examinations to date.
    UNASSIGNED: By offering readers comprehensive knowledge of contrast imaging, especially CEUS, in the diagnosis of HA, our study may reduce misdiagnosis and further improve treatment options.
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  • 文章类型: Case Reports
    一名80岁的日本男子因肝脏肿瘤破裂而出现腹腔内出血。经导管动脉栓塞(TAE)暂时实现止血,但他在4天后再次破裂后死亡。根据尸检结果,肝肿瘤诊断为肝血管肉瘤。在肝小叶间小动脉内发现了栓塞期间使用的栓塞剂。然而,在血管肉瘤中没有发现肿瘤细胞坏死或缺血性改变。在目前的情况下,单独的TAE不会诱导缺血诱导的肿瘤坏死,提示TAE可能不适合治疗肝血管肉瘤。希望对破裂的肝血管肉瘤进行治疗优化。
    An 80-year-old Japanese man presented to our hospital with intra-abdominal hemorrhage due to a ruptured liver tumor. Transcatheter arterial embolization (TAE) temporarily achieved hemostasis, but he died following re-rupture 4 days later. Based on autopsy findings, the liver tumor was diagnosed as hepatic angiosarcoma. Embolic agents used during embolization were identified within the hepatic small interlobular arteries. However, there were no findings of tumor cell necrosis or ischemic change in the angiosarcoma. In the present case, TAE alone did not induce ischemia-induced tumor necrosis, suggesting that TAE might be unsuitable to treat hepatic angiosarcoma. Treatment optimization for ruptured hepatic angiosarcoma is desired.
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  • 文章类型: Case Reports
    原发性肝血管肉瘤(PHA)是一种极为罕见且侵袭性的间充质起源肿瘤。PHA占原发性肝肿瘤的很小一部分,预后较差。症状可能是模糊的,并且通常在出现时模仿原发性肝细胞癌。诊断需要仔细的免疫组织病理学确认。我们介绍了一名患有腹痛和怀疑潜在隐源性肝硬化的患者的PHA病例。
    Primary hepatic angiosarcoma (PHA) is an exceedingly rare and aggressive neoplasm of mesenchymal origin. PHA makes a very small portion of primary liver tumors and conveys a poor prognosis. Symptomatology can be vague and often mimics primary hepatocellular carcinoma upon presentation. Diagnosis requires careful immunohistopathologic confirmation. We present a case of PHA in a patient with abdominal pain and suspected underlying cryptogenic cirrhosis.
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  • 文章类型: Journal Article
    肝脏的间充质肿瘤可能具有诊断挑战性,特别是核心针活检。这里,我讨论了肝脏特有的肿瘤(间充质错构瘤,未分化胚胎肉瘤,钙化巢式间质上皮肿瘤),肝脏血管肿瘤(吻合性血管瘤,肝小血管肿瘤,上皮样血管内皮瘤,血管肉瘤),和其他主要发生在肝脏的肿瘤类型(PEComa/血管平滑肌脂肪瘤,炎性假瘤样滤泡树突状细胞肉瘤,EBV相关平滑肌肿瘤,炎性肌纤维母细胞瘤,恶性横纹肌样瘤)。最后,我讨论了肝脏的转移性肉瘤,以及转移性黑色素瘤和肉瘤样癌带来的陷阱。
    Mesenchymal neoplasms of the liver can be diagnostically challenging, particularly on core needle biopsies. Here, I discuss recent updates in neoplasms that are specific to the liver (mesenchymal hamartoma, undifferentiated embryonal sarcoma, calcifying nested stromal-epithelial tumor), vascular tumors of the liver (anastomosing hemangioma, hepatic small vessel neoplasm, epithelioid hemangioendothelioma, angiosarcoma), and other tumor types that can occur primarily in the liver (PEComa/angiomyolipoma, inflammatory pseudotumor-like follicular dendritic cell sarcoma, EBV-associated smooth muscle tumor, inflammatory myofibroblastic tumor, malignant rhabdoid tumor). Lastly, I discuss metastatic sarcomas to the liver, as well as pitfalls presented by metastatic melanoma and sarcomatoid carcinoma.
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  • 文章类型: Case Reports
    肝血管肉瘤(HA)是肝内皮和成纤维细胞血管组织起源的罕见原发性恶性肿瘤。患者通常表现为疲劳的模糊体质症状,减肥,腹痛,和腹水。腹膜积血是HA的常见临床表现,与较高的死亡率相关,但未得到充分认可。这里,我们报告了一例因腹膜出血而并发的HA患者,其管理,和相关的不良预后。
    Hepatic angiosarcoma (HA) is a rare primary malignancy of hepatic endothelial and fibroblastic vascular tissue origin. Patients typically present with vague constitutional symptoms of fatigue, weight loss, abdominal pain, and ascites. Hemoperitoneum is a frequent clinical manifestation of HA associated with higher mortality and is underrecognized. Here, we report the case of a patient with HA that was complicated by a peritoneal bleed, its management, and associated poor prognosis.
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  • 文章类型: Case Reports
    原发性肝血管肉瘤(PHA),一种罕见的软组织肿瘤,仅占所有肝脏恶性肿瘤的2%。在病理上具有挑战性,用超声很难将PHA与其他恶性肿瘤区分开来,计算机断层扫描(CT)或磁共振成像(MRI)。由于晚期诊断和对传统化疗和/或放疗的抵抗,只有3%的PHA患者在诊断后可以生存长达两年。据我们所知,本病例报告为首例晚期弥漫性PHA伴破裂出血,经TACE和安洛替尼联合卡姆瑞珠单抗有效治疗.到目前为止,患者接受了10个周期的治疗,进展良好。最新的MRI结果表明,肿瘤缩小了56%,可以评估为部分反应(PR)。这个病例报告包括我们治疗这种晚期恶性肿瘤的经验,我们希望对先进的PHA进行更大的研究可以更好地量化潜在的益处。
    Primary hepatic angiosarcoma (PHA), a rare soft tissue tumor, accounts for only 2% of all liver malignancies. Pathologically challenging, PHA is difficult to be distinguished from other malignancies with ultrasound, Computed Tomography (CT), or Magnetic Resonance Imaging (MRI). Due to late diagnosis and resistance against traditional chemotherapy and/or radiotherapy, only 3% of PHA patients can survive up to two years after diagnosis. To our best knowledge, this case report presents the first case of an advanced diffuse PHA with ruptured hemorrhage that has been effectively treated with TACE and Anlotinib plus Camrelizumab. So far, the patient has received 10 cycles of treatment and is faring well. Latest MRI results show that the tumor has shrunk by 56% and can be assessed as a partial response (PR). This case report includes our experience in treating such a advanced malignancy, and we hope that larger studies on advanced PHA can better quantify the potential benefit.
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  • 文章类型: Case Reports
    原发性脾或肝血管肉瘤是极罕见和侵袭性恶性肿瘤,预后不良。主要治疗方法是手术切除和化疗。我们报道了一个50岁女性的血管肉瘤病例,她出现瘀伤,疲劳,瘀斑,和肝脾肿大.在出现脾出血和死亡之前,她接受了多激酶抑制剂舒尼替尼治疗4周。最近的研究已经证明了免疫疗法在血管肉瘤中的临床益处。此外,测序技术已经展示了涉及血管肉瘤的各种分子畸变,这为精确匹配的靶向治疗提供了机会,例如VEGF/VEGFR轴和PI3K/Akt/mTor通路的抑制剂。
    Primary splenic or hepatic angiosarcomas are ultra-rare and aggressive malignancies associated with poor prognosis. The mainstay treatments are surgical resection and chemotherapy. We report a case of angiosarcoma in a 50-year-old woman who presented with bruising, fatigue, ecchymosis, and hepatosplenomegaly. She was treated with the multi-kinase inhibitor sunitinib for 4 weeks before developing a splenic hemorrhage and succumbing. Recent studies have demonstrated the clinical benefit of immunotherapies in angiosarcomas. Additionally, sequencing techniques have showcased the diverse molecular aberrations involved in angiosarcomas, which offer opportunities for precision-matched targeted therapies such as inhibitors of the VEGF/VEGFR axis and PI3K/Akt/mTor pathway.
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  • 文章类型: Case Reports
    原发性肝血管肉瘤(PHA)是一种散发性和侵袭性的肝脏肿瘤,起源于间充质细胞,占所有原发性肝肿瘤的不到2%。已知它与几种环境和工业致癌物有关;然而,在75%的案例中,病因仍不清楚。患者通常表现为非特异性症状和实验室检查结果。影像学在诊断中的作用有限。我们在此介绍一例52岁的男性,有乙型肝炎相关肝硬化病史,他被转诊到我们医院进行肝移植评估。磁共振成像(MRI)显示肝脏IV段有两个5和6mm的小结节性病变,分类为肝脏成像报告和数据系统(LI-RADS)类别3。在多学科肿瘤会议上讨论了患者,计划在三个月内进行MRI随访。三个月后,MRI描绘了测量的8.5cm的病变大小的显著增加。进行了超声引导下的切开活检,经病理证实诊断为PHA。在这份报告中,我们的目标是强调PHA的MRI特征,并强调这种罕见实体的快速和致命的进展。关键词:肝脏,肝血管肉瘤,磁共振成像。
    Primary hepatic angiosarcoma (PHA) is a sporadic and aggressive tumour of the liver that originates from mesenchymal cells and represents less than 2% of all primary liver tumours. It is known to be associated with several environmental and industrial carcinogens; however, in 75% of cases, aetiology remains unclear. Patients generally present with nonspecific symptoms and laboratory findings. Imaging has a limited role in the diagnosis.  We herein present a case of a 52-year-old man with a history of hepatitis B-related cirrhosis who was referred to our hospital for liver transplantation assessment. Magnetic resonance imaging (MRI) revealed two small nodular lesions of 5 and 6 mm in segment IV of the liver, categorised as Liver Imaging Reporting and Data System (LI-RADS) category 3. The patient was discussed at a multidisciplinary tumour meeting, and an MRI follow-up in three months was planned. Three months later, MRI depicted a substantial increase in the lesion size measured 8.5 cm. An ultrasound-guided tru-cut biopsy was performed, and the diagnosis of PHA was confirmed by pathology. In this report, we aim to highlight PHA\'s MRI features and underline this rare entity\'s rapid and fatal progression.
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