Pericardial mesothelioma

心包间皮瘤
  • 文章类型: Case Reports
    心包间皮瘤(PM)是罕见的,只有200例记录,验尸率<0.0022%。它是第三常见的心脏/心包肿瘤,在血管肉瘤和横纹肌肉瘤后面.PM发病率随着年龄的增长而增加,通常在50到70岁之间偶然诊断,男性占3:1。偶尔PM会引起胸痛,呼吸困难,咳嗽甚至吞咽困难。PM经常被误诊,只有25%的病例是死前诊断。与胸膜间皮瘤不同,石棉暴露与恶性肿瘤之间的联系不太令人信服,只有20%的病例有已知的暴露。6有三种组织学类型:上皮样,纤维(梭形细胞),和双相(混合)。诊断后的平均预期寿命为3-10个月。由于呈现的异质性和稀有性,没有标准化的管理算法,几乎没有描述诊断成像或实验室检查。我们正在介绍黄金海岸我们单位诊断的一例病例。
    Pericardial mesothelioma (PM) is rare with only 200 cases recorded, and a post-mortem prevalence of <0.0022%. It is the third most common cardiac/pericardial tumour, behind angiosarcoma and rhabdomyosarcoma. PM incidence increases with age, typically incidentally diagnosed between 50 and 70 years, with a 3:1 male predominance. Occasional PM can cause chest pain, dyspnoea, cough and even dysphagia. PMs are often misdiagnosed with only 25% of cases being antemortem diagnoses. Unlike pleural mesothelioma, the link between asbestos exposure and malignancy is less convincing, with only 20% of cases having known exposure. 6 There are three histological types: epithelioid, fibrous (spindle cell), and biphasic (mixed). The average life-expectancy post diagnosis is 3-10 months. Due to the heterogeneity of the presentation and rarity there is no standardized management algorithm, and the diagnostic imaging or laboratory investigations are scarcely described. We are presenting one of the cases diagnosed in our unit here in the Gold Coast.
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  • 文章类型: Journal Article
    犬间皮瘤是一种罕见的恶性肿瘤,主要影响体腔,如心包腔和胸膜腔。化疗在犬间皮瘤的治疗中起着至关重要的作用。我们旨在比较单药和联合化疗药物对本研究中建立的犬心包间皮瘤患者来源的原代培养物的抗肿瘤作用。我们计划为未来的研究生成异种移植模型。
    从三只经组织学诊断为心包间皮瘤的狗收集积液样品并用于原代培养。培养的细胞通过全细胞角蛋白AE1/AE3,波形蛋白的免疫染色来表征,威尔姆斯抑癌基因1(WT1),和细胞角蛋白5(CK5)。为了评估积液中细胞的致瘤特性并生成异种移植模型,将细胞悬液皮下(SC)或腹膜内(IP)注射到严重的联合免疫缺陷(SCID)小鼠中。最后,已建立的原代培养物对四种药物的化学敏感性,阿霉素,长春瑞滨,卡铂,和吉西他滨,通过单一药物治疗以及以固定浓度联合治疗卡铂,10或100μM,通过细胞活力测定评估0-1000μM范围内的不同浓度的吉西他滨。
    原代培养物被成功地产生并且通过AE1/AE3和波形蛋白的双重阳性以及WT-1和CK5的阳性染色来表征,从而证实细胞的间皮起源。在异种移植模型中,SC小鼠出现皮下肿块,而IP小鼠出现多个腹膜内结节。肿块在组织病理学上与间皮瘤一致。化学敏感性测定显示,卡铂在四种测试的单药治疗中具有最高的抗肿瘤作用。此外,与单药治疗相比,100μM卡铂联合临床相关剂量的吉西他滨显示出增强的抗肿瘤作用。
    本研究中产生的原代培养物和异种移植模型可能是犬间皮瘤的体外和体内研究的有用工具。当用作唯一药剂并与吉西他滨组合时,卡铂是对抗犬间皮瘤的高效化学治疗剂。
    UNASSIGNED: Canine mesothelioma is a rare malignant tumor that mostly affects body cavities, such as the pericardial and pleural cavities. Chemotherapy plays a crucial role in the treatment of canine mesotheliomas. We aimed to compare the antitumor effects of single-agent and combination chemotherapeutic agents on patient-derived primary cultures of canine pericardial mesothelioma established in this study. We planned to generate xenograft models for future studies.
    UNASSIGNED: Effusion samples were collected from three dogs with histologically diagnosed pericardial mesothelioma and used for primary culture. Cultured cells were characterized by immunostaining for pan-cytokeratin AE1/AE3, vimentin, Wilms\' tumor suppressor gene 1 (WT1), and cytokeratin 5 (CK5). To assess the tumorigenic properties of cells in the effusion and generate a xenograft model, the cell suspension was injected into a severe combined immunodeficient (SCID) mouse either subcutaneously (SC) or intraperitoneally (IP). Lastly, chemosensitivity of established primary cultures against four drugs, doxorubicin, vinorelbine, carboplatin, and gemcitabine, by single-agent treatment as well as combination treatment of carboplatin at a fixed concentration, either 10 or 100 μM, and gemcitabine at different concentrations ranging from 0-1000 μM was assessed by cell viability assay.
    UNASSIGNED: Primary cultures were successfully generated and characterized by dual positivity for AE1/AE3 and vimentin and positive staining for WT-1 and CK5, confirming the mesothelial origin of the cells. In the xenograft models, SC mouse developed a subcutaneous mass, whereas IP mouse developed multiple intraperitoneal nodules. The masses were histopathologically consistent with mesotheliomas. The chemosensitivity assay revealed that carboplatin had the highest anti-tumor effects among the four tested single-agent treatments. Furthermore, carboplatin at 100 μM combined with gemcitabine at clinically relevant doses demonstrated the augmented anti-tumor effects compared to single-agent treatment.
    UNASSIGNED: Primary cultures and xenograft models generated in this study could be useful tools for in vitro and in vivo studies of canine mesothelioma. Carboplatin is a highly effective chemotherapeutic agent against canine mesothelioma when used as a sole agent and in combination with gemcitabine.
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  • 文章类型: Case Reports
    一名46岁的妇女因复发性心包积液而接受了心包穿刺术和心包窗。17个月后,她出现了与缩窄性心包炎一致的体征和症状。心脏磁共振成像显示心包周围有浸润性肿块。心包经皮穿刺活检证实了心包间皮瘤的诊断。
    A 46-year-old woman underwent pericardiocentesis and pericardial window for recurrent pericardial effusion. She presented 17 months later with signs and symptoms consistent with constrictive pericarditis. Cardiac magnetic resonance imaging revealed an infiltrative mass surrounding the pericardium. A transcutaneous core needle biopsy of the pericardium confirmed the diagnosis of pericardial mesothelioma.
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  • 文章类型: Case Reports
    未经证实:原发性心包间皮瘤是一种极为罕见的疾病。预后很差,化疗或放疗的影响很小。大多数病例是在尸检时诊断的。
    未经授权:一名22岁的男子,在第二次BNT162b2COVID-19疫苗接种2个月后出现复发性心包炎和大量心包积液,行心包穿刺术和心包窗。心包病理标本提示良性间皮炎症,符合急性心包炎.四个月后,他出现了一个大的心包肿块,表现为心力衰竭,并接受了紧急心包切除术。新的病理标本免疫染色和荧光原位杂交分析显示心包间皮瘤。尽管有重症监护,患者3周后死亡。
    未经证实:在难治性复发性心包炎的鉴别诊断中,应考虑原发性心包间皮,即使先前有活检证实的心包炎,或怀疑有推定的触发因素(先前接种过COVID-19mRNA),就像这个病人的情况一样。肿瘤的诊断和鉴定包括多模态成像和实验室测试。多学科,应采用个性化护理方法。
    UNASSIGNED: Primary pericardial mesothelioma is an extremely rare disease. Prognosis is poor, with little effects of chemo- or radio-therapy. The majority of cases is diagnosed at autopsy.
    UNASSIGNED: A 22-year-old man, who presented with recurrent pericarditis and large pericardial effusion 2 months after a second BNT162b2 COVID-19 vaccine, underwent pericardiocentesis and pericardial window. Pathology specimen of pericardium revealed benign mesothelial inflammation, consistent with acute pericarditis. Four months later, he presented with a large pericardial mass manifesting in heart failure and underwent urgent pericardiectomy. A new pathology specimen immunostaining and fluorescence in situ hybridization analysis revealed pericardial mesothelioma. Despite intensive care, the patient died 3 weeks later.
    UNASSIGNED: Primary pericardial mesothelial should be considered in the differential diagnosis of refractory recurrent pericarditis, even with prior biopsy-proven pericarditis or when a putative trigger (COVID-19 mRNA prior vaccination) is suspected, as was the case in this patient. Tumour diagnosis and identification consist of multimodal imaging and laboratory tests. A multidisciplinary, individualized care approach should be performed.
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  • 文章类型: Case Reports
    该病例报告介绍了一名60岁的绅士,他有明显的吸烟史和可能的石棉暴露,他因房颤而被转诊至急诊科,室率快,有心力衰竭症状。实验室显示脑钠肽和肌钙蛋白I正常。他的超声心动图发现提示缩窄性心包炎,射血分数为60%。计算机断层扫描涉及心包肿块。左心导管插入术暗示更多的是收缩生理;然而,一些发现与限制性生理学有关。因此,做了心脏磁共振成像,确定了缩窄性心包炎的诊断。心包切除术计划采用迷宫手术治疗心房颤动。然而,在冷冻活检中发现了恶性肿瘤。因此,手术仅限于部分心包切除术,患者患有晚期浸润性肿瘤,导致缩窄性心包炎。最终病理报告确诊为恶性心包间皮瘤混合型。恶性肿瘤通常在晚期诊断,就像我们的案子一样,由于非特异性的初始演示。文献综述表明,在治疗方面缺乏既定的共识。对治疗的反应似乎也很差,只会缓解症状,尽管进行了最佳的医疗管理,但诊断后的中位生存期为6个月。
    This case report presents a 60-year-old gentleman with a significant smoking history and possible asbestos exposure who was referred to the emergency department for atrial fibrillation with a rapid ventricular rate and symptoms of heart failure. Labs showed normal brain natriuretic peptide and troponin I. His echocardiography finding suggested constrictive pericarditis with an ejection fraction of 60%. A computed tomography scan was concerning for a pericardial mass. Left and right heart catheterization hinted more toward constrictive physiology; however, some findings were concerning for restrictive physiology. Hence, cardiac magnetic resonance imaging was done, which established the diagnosis of constrictive pericarditis. Pericardiectomy was planned with a maze procedure for atrial fibrillation. However, a malignant neoplasm was seen on a frozen biopsy. Hence, surgery was limited to partial pericardiectomy, as the patient had advanced infiltrative neoplasm that had resulted in constrictive pericarditis. The final pathology report confirmed the diagnosis of malignant pericardial mesothelioma mixed type. Malignancy is usually diagnosed in an advanced stage, like in our case, due to nonspecific initial presentation. A literature review suggests that there is a lack of established consensus on treatment. The response to therapy also seems to be poor and results only in palliation of symptoms, with a median survival of six months from diagnosis despite optimum medical management.
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  • 文章类型: Case Reports
    原发性心包间皮瘤是一种极其罕见的肿瘤,病因不清楚,非特异性演示,诊断延迟,预后不良.我们介绍了一个患有心包间皮瘤的女性病例,主要表现为心脏填塞,目前在诊断和接受化疗后三年存活。学习目标:心脏填塞可能是肿瘤性疾病的第一个临床表现,包括心包间皮瘤,因此,有这种临床表现的患者应始终采用适当的诊断方法。肺间皮瘤的治疗进展使化疗方案得以调整,以适应心包间皮瘤的治疗,可以提高这种癌症患者的生存率,仍被认为预后不良。>.
    Primary pericardial mesothelioma is an extremely rare tumor, of unclear etiology, nonspecific presentation, with a delay in diagnosis, and a poor prognosis. We present the case of a woman with pericardial mesothelioma, whose main manifestation was cardiac tamponade, currently alive three years after diagnosis and undergoing chemotherapy treatment. .
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  • 文章类型: Case Reports
    Primary pericardial mesothelioma (PPM) is an extremely rare malignancy with a very poor prognosis. It poses a diagnostic challenge given its often late and non-specific presentation. This report describes a 74-year-old man who presented with central pleuritic chest pain and mild breathlessness. The patient was febrile and mildly tachycardic with crepitations in the right lung base. Blood tests revealed raised inflammatory markers and chest X-ray showed no acute pathology. Following admission, CT pulmonary angiogram showed a large left-sided mediastinal mass (approximately 110 x 70 x 85 mm) centered on the pericardium. Further post venous phase CT imaging identified possible myocardial invasion alongside suspicious liver nodules. Later, outpatient fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging highlighted further FDG avid pleural and liver lesions. CT-guided biopsy of the pericardial lesion was undertaken, with histology and immunohistochemistry indicating epitheliod-type mesothelioma. A significant malignant pericardial effusion was also identified, which ultimately required pericardial window formation. Immunotherapy was commenced utilizing dual nivolumab and ipilimumab, a novel regime for the treatment of mesothelioma. Palliative radiotherapy to the pericardial lesion will also be performed. Here, we demonstrate the diagnostic challenge of this vanishingly rare condition, which is usually diagnosed upon the development of associated complications. Early recognition gives the best chance of improved mortality, however, diagnosis requires a high index of clinical suspicion alongside prompt investigation, primarily involving cross-sectional imaging.
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  • 文章类型: Journal Article
    心脏肿块很少见,但仍然是心脏肿瘤学实践的重要组成部分。这些包括良性肿瘤,恶性肿瘤(原发性和继发性)和肿瘤样病症(例如,血栓,兰姆的后代,和心包囊肿)。多模态成像的出现使得在许多情况下能够识别心脏肿块的病因。特别是结合来自临床环境的信息。本文对流行病学进行了全面的综述,临床表现,成像,诊断,管理,和心脏肿块的结果。
    Cardiac masses are rare, but remain an important component of cardio-oncology practice. These include benign tumors, malignant tumors (primary and secondary) and tumor-like conditions (e.g., thrombus, Lambl\'s excrescences, and pericardial cyst). The advent of multimodality imaging has enabled identification of the etiology of cardiac masses in many cases, especially in conjunction with information from clinical settings. This paper provides a comprehensive review of the epidemiology, clinical presentation, imaging, diagnosis, management, and outcomes of cardiac masses.
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  • 文章类型: Case Reports
    Metastases to the heart and pericardium are much more common than primary malignant neoplasms. Primary malignant pericardial mesothelioma is a rare tumor that arises from the mesothelial cells of the pericardium. It is usually characterized by a delayed diagnosis, a low response to treatment, and a poor prognosis with an overall survival up to six months after the onset of symptoms. We report a rare case of a 32-year-old woman with primary pericardial malignant mesothelioma that was diagnosed 4 months after the onset of pericardial effusion as the first clinical manifestation.
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  • 文章类型: Case Reports
    A 6-year-old neutered male Yorkshire Terrier presented with recurrent pericardial effusion. Although clinical examinations including computed tomography were inconclusive, an exploratory thoracotomy revealed multiple small nodules and plaques on the inner surface of the pericardial sac (Day 1). A subtotal pericardiectomy was performed to prevent cardiac tamponade due to the increasing pericardial effusion, and the resected section of the pericardium was histopathologically diagnosed with mesothelioma. After surgery, chemotherapy with intrathoracic carboplatin was commenced. During the course of the treatment, a detailed follow-up ultrasonographic scan was performed to detect early lesions disseminated on the pleura, originating from the primary pericardial mesothelioma. On Day 101, the minute pleural nodules, which were disseminated lesions as predicted, were successfully imaged by ultrasonography. As the clinical stage advanced, the nodules were observed to gradually increase in size and number, implying tumor progression. These observations highlight the feasibility of ultrasonography in detecting minute disseminated lesions at an early stage, monitoring tumor progression, and thereby, predicting the prognosis of canine pericardial mesothelioma.
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