Malignant mesothelioma

恶性间皮瘤
  • 文章类型: Journal Article
    背景:原发性肝内间皮瘤(PIHMM)的报道很少。其典型的临床表现,放射学特征和病理学尚未定义。这里,我们旨在总结其诊断和治疗。
    方法:对浙江大学医学院附属第一医院3例PIHMM病例进行回顾性分析,并复习现有文献,探讨PIHMM的临床病理特点及预后。
    结果:根据我们的案例系列和文献,PIHMM的平均年龄为59.7(41-83)岁.大多数患者表现为非特异性症状,如腹痛,发烧,减肥和虚弱。在成像方面,PIHMM通常表现为固体,非均质软组织肿块,边缘不规则,动脉期边缘显着增强。免疫组织化学标记物,如calretinin,细胞角蛋白(CK)5/6,D2-40,WT-1,间皮素CK和波形蛋白可能有助于诊断。3年无复发生存率(RFS)为51.85%,术后3年总生存率(OS)为83.33%,术后3年总生存率为100%。
    结论:PIHMM只能通过仔细的术后病理来诊断,由于其非特异性临床表现,血清学指标或影像学特征。免疫组织化学染色对于区分该肿瘤与其他肝肿瘤非常有用。手术是治疗的主要手段。
    BACKGROUND: Primary intrahepatic mesothelioma (PIHMM) has been rarely reported. Its typical clinical presentation, radiological features and pathology have not been defined. Here, we aimed to summarize its diagnosis and treatment.
    METHODS: We conducted a retrospective analysis of three cases of PIHMM in the First Affiliated Hospital of Zhejiang University School of Medicine and reviewed the current literature to investigate the clinical and pathological characteristics and prognosis of PIHMM.
    RESULTS: Based on our case series and the literature, the mean age of PIHMM was 59.7 (41-83) years. Most patients present with nonspecific symptoms such as abdominal pain, fever, weight loss and weakness. On imaging, PIHMM usually presented as a solid, heterogeneous soft tissue mass with irregular margins and significant enhancement of the margins in the arterial phase. Immunohistochemical markers such as calretinin, cytokeratin (CK)5/6, D2-40, WT-1, mesothelin CK and vimentin may be useful for diagnosis. The 3-year relapse-free survival rate (RFS) was 51.85%, the 3-year overall survival (OS) rate was 83.33% and the 3-year postoperative overall survival rate was 100%.
    CONCLUSIONS: PIHMM can only be diagnosed by careful postoperative pathology, because of its nonspecific clinical presentations, serological indicators or imaging features. Immunohistochemical staining is very useful to distinguish this tumor from other liver tumors. Surgery is the mainstay of treatment.
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  • 文章类型: Letter
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  • 文章类型: Case Reports
    我们报告了一例69岁的女性,其胸膜间皮瘤出现在后纵隔,最大直径为25厘米。她患有慢性咳嗽,胸部X光检查发现胸腔积液。对积液的检查显示高透明质酸水平,和间皮瘤被怀疑。胸部计算机断层扫描显示纵隔巨大肿块,导致呼吸衰竭和心脏受压的快速进展。死亡前无法获得足够的组织样本。患者在初次就诊后约1个月死亡,并进行了病理尸检。诊断为恶性胸膜间皮瘤。恶性胸膜间皮瘤与巨大的后纵隔肿块,如在这种情况下是相当罕见的;然而,它是该疾病的一种快速发展形式,在本文中被报道为纵隔肿瘤的重要鉴别诊断。
    We report a case of a 69-year-old woman with pleural mesothelioma presenting in the posterior mediastinum with a maximum diameter of 25 cm. She had a chronic cough and a pleural effusion was noted on chest X-ray. The examination of the effusion showed high hyaluronic acid levels, and mesothelioma was suspected. A chest computed tomography scan showed a huge mediastinal mass, which caused rapid progression of respiratory failure and compression of the heart. Sufficient tissue samples could not be obtained before death. The patient died approximately 1 month after the initial visit, and a pathological autopsy was performed. The diagnosis of malignant pleural mesothelioma was made. Malignant pleural mesothelioma with a huge posterior mediastinal mass such as in this case is considerably rare; however, it is a rapidly progressing form of the disease and is reported here as an important differential diagnosis for mediastinal tumours.
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  • 文章类型: Case Reports
    恶性间皮瘤(MM)是一种罕见的侵袭性肿瘤,存在于胸膜和腹膜中。已报道了几例心包和阴道膜睾丸中的MM。此外,原发性发生在中庭极为罕见。这种肿瘤的视觉外观与常见的心房粘液瘤相似,这使得临床医生和放射科医生诊断和治疗这种疾病具有挑战性。
    一名18岁女性出现胸痛症状,呼吸急促,咳嗽,并咳痰7天。对患者进行了超声心动图检查,显示心房肿块。粘液瘤是鉴别诊断之一。肿瘤是一个有尖端的椭圆形肿块,切割表面像果冻一样,类似于粘液瘤。手术后,活检肿瘤的病理检查证实为上皮型MM。术后随访期间,未观察到肿瘤复发。
    源自中庭的MM被认为极为罕见。因此,临床医生很容易将心房MM误诊为粘液瘤。此外,为了确认诊断,组织病理学活检,组织形态学特征,免疫组织化学,分子基因检测是必需的.因此,MM的临床诊断和治疗具有挑战性。
    UNASSIGNED: Malignant mesothelioma (MM) is a rare and aggressive tumor that is found in the pleura and peritoneum. A few cases of MM in the pericardium and tunica vaginalis testis have been reported. Moreover, primary occurrence in the atrium is extremely rare. The visual appearance of this tumor is similar to that of a common atrial myxoma, which makes it challenging for clinicians and radiologists to diagnose and treat this disease.
    UNASSIGNED: An 18-year-old woman presented with symptoms of chest pain, shortness of breath, cough, and expectoration for 7 days. Echocardiography was performed on the patient, which revealed an atrial mass. Myxoma was one of the differential diagnoses. The tumor was an elliptical mass with tips, and the cut surface was jelly-like, similar to myxoma. After surgery, a pathologic examination of the biopsied tumor confirmed epithelial-type MM. During postoperative follow-up, no recurrence of the tumor was observed.
    UNASSIGNED: MM originating in the atrium is considered to be extremely rare. Consequently, clinicians can easily misdiagnose atrial MM as a myxoma. Moreover, to confirm the diagnosis, histopathologic biopsy, histomorphological characterization, immunohistochemistry, and molecular genetic testing are required. Therefore, clinical diagnosis and treatment of MM are challenging.
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  • 文章类型: Case Reports
    诊断恶性腹膜上皮样间皮瘤(MPM或MPeM)的临床医生由于该疾病的低发病率而历来面临挑战。以及患者通常存在的模糊症状。新的技术进步,特别是在免疫细胞化学中,提供了更清晰的诊断路径。此外,恶性间皮瘤必须与癌区分开来。这是通过组织学完成的,免疫细胞化学,以及仔细纳入患者的临床病史。在这种情况下,我们介绍了一名无症状的73岁非吸烟女性,没有石棉接触史.常规腹部疝修补术后,她被诊断为MPM。随后的检查显示肺浸润,成功活检和切除,显然是腺癌.仔细审查由此产生的病理,以及免疫细胞化学的解释,支持患者同时发生两个独立的恶性过程的观点。这个案例强调了两个相似的罕见,然而不同的癌症,以及流行病学,症状学,组织学,免疫细胞化学,和预后。
    Clinicians diagnosing malignant peritoneal epithelioid mesothelioma (MPM or MPeM) have historically had challenges due to the low incidence of the disease, as well as the often vague symptomatology that patients present with. Newer advances in technology, specifically in immunocytochemistry, have provided a clearer path to diagnosis. Additionally, malignant mesotheliomas must be differentiated from carcinomas. This is done via histology, immunocytochemistry, as well as a careful incorporation of the patient\'s clinical history. In this case, we present an asymptomatic 73-year-old non-smoker female with no past medical history of asbestos exposure. She was diagnosed with MPM following a routine abdominal hernia repair. Subsequent workup revealed a lung infiltrate that was successfully biopsied and resected, evidently found to be adenocarcinoma. A careful review of the resulting pathology, as well as the interpretation of immunocytochemistry, supported the notion that the patient had two independent malignant processes occurring at once. This case underscores the rarity of two similar, yet distinct cancers, as well as epidemiology, symptomatology, histology, immunocytochemistry, and prognosis.
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  • 文章类型: Case Reports
    影像学上结节性软组织胸膜增厚高度考虑恶性肿瘤,其中胸膜恶性间皮瘤和转移性疾病有区别。我们介绍了一名71岁男性的病例,该患者在胸膜穿刺术后反复出现左胸腔积液,导致呼吸急促急性恶化。他是前吸烟者,以前曾接触过石棉。进行的计算机断层扫描显示,半胸腔和半膈的左侧胸膜增厚,伴有复杂的胸腔积液。18F-2-脱氧-d-葡萄糖全身PET扫描显示,在多个胸膜肿块中,左半胸广泛摄取。影像学表现及临床病例均为典型的恶性间皮瘤。然而,病理结果提示小细胞肺癌。我们需要认识到常见疾病实体的这种非典型表现。即使所有临床和影像学发现都指向某种诊断,组织病理学评估不容忽视。
    Nodular soft tissue pleural thickening on imaging is highly suggestive of malignancy, of which pleural malignant mesothelioma and metastatic disease are differentials. We present the case of a 71-year-old male who presented with acute worsening of shortness of breath associated with a recurrent left pleural effusion post-pleurocentesis. He was an ex-smoker with previous asbestos exposure. Computed tomography performed demonstrated left-sided pleural thickening in the hemithorax and hemidiaphragm with complex pleural effusion. 18F-2-deoxy-d-glucose whole body PET scan revealed extensive uptake throughout the left hemithorax in multiple pleural masses. The imaging findings and clinical case were typical of malignant mesothelioma. However, histopathology results revealed small cell lung cancer. We need to be cognisant of this atypical presentation of a common disease entity. Even when all clinical and imaging findings point towards a certain diagnosis, histopathological assessment cannot be ignored.
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  • 文章类型: Case Reports
    腹膜恶性间皮瘤是一种罕见的肿瘤,预后不良。我们特此报告一例20岁男性,首次活检诊断为腋窝淋巴结转移。他出现腹痛和腋窝淋巴结肿大,无石棉接触史。CECT显示腹膜增厚和腹水。腹水细胞学表现为反应性形态。恶性间皮瘤转移沉积的诊断是在组织病理学上做出的,并通过免疫组织化学证实。肿瘤细胞对CK5/6,钙视网膜素,D2-40和WT1,TTF1、CK20和CD3为阴性。该病例报告具有两个重要的亮点-(i)腋窝淋巴结转移的异常表现,导致无石棉暴露史的年轻男性的诊断困境,以及(ii)IHC在腹膜恶性间皮瘤中的确证诊断作用。
    Peritoneal malignant mesothelioma is an uncommon neoplasm with a poor prognosis. We hereby report a case of a 20-year-old male, first diagnosed on biopsy with axillary lymph node metastasis. He presented with abdominal pain and axillary lymphadenopathy, with no history of asbestos exposure. CECT showed peritoneal thickening and ascites. Ascitic fluid cytology showed reactive morphology. The diagnosis of metastatic deposits of malignant mesothelioma was made on histopathology and confirmed by immunohistochemistry. Tumor cells were immune-reactive for CK 5/6, calretinin, D2-40, and WT1 and negative for TTF1, CK 20, and CD 3. This case report has two important highlights-(i) unusual presentation with axillary lymph node metastasis leading to diagnostic dilemma in a young male with no asbestos exposure history and (ii) confirmatory diagnostic role of IHC in Peritoneal malignant mesothelioma.
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  • 文章类型: Case Reports
    阴道睾丸的恶性间皮瘤是一种罕见的,高侵袭性泌尿生殖道恶性肿瘤,临床表现无特异性。这种疾病的报告病例有限。因此,早期的术前诊断是困难的。目前的研究提出了1例恶性间皮瘤的阴道睾丸和文献复习。52岁男子入住温州医科大学附属萧山医院(杭州,中国)于2022年12月接受了右睾丸和附睾的根治性切除术,但未接受放疗或化疗。患者随访5个月,未发现复发或转移。罕见的睾丸间皮瘤对其病因和诊断提出了挑战,术前很少实现。睾丸鞘膜恶性间皮瘤预后差,对放疗或化疗不敏感,需要密切的术后随访。这种情况在临床实践中很少见;因此,它需要报告,以帮助临床医生决策有关诊断和治疗。
    Malignant mesothelioma of the tunica vaginalis testis is a rare, highly invasive urogenital malignant tumor with no specific clinical manifestations. Reported cases of this disease are limited. Therefore, an early preoperative diagnosis is difficult. The current study presents a case of malignant mesothelioma of the tunica vaginalis testis and a literature review. A 52-year-old man was admitted to Xiaoshan Affiliated Hospital of Wenzhou Medical University (Hangzhou, China) in December 2022 and underwent radical resection of the right testicle and epididymis but did not undergo radiotherapy or chemotherapy. The patient was followed up for 5 months, and no recurrence or metastasis was found. The rarity of testicular mesothelioma poses a challenge to its etiology and diagnosis, which is rarely achieved preoperatively. Malignant mesothelioma of the testicular tunica vaginalis has a poor prognosis and is not sensitive to radiotherapy or chemotherapy, requiring close postoperative follow-up. This condition is rare in clinical practice; therefore, it needs to be reported to aid clinicians\' decision-making regarding diagnosis and treatment.
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  • 文章类型: Case Reports
    恶性腹膜间皮瘤(MPM)是间皮瘤的一种罕见亚型。间皮瘤有三种主要的组织学亚型:上皮样,肉瘤样,和双相(混合)。危险因素包括石棉暴露,以前的辐射,和一些种系突变。治疗包括手术切除易行肿瘤或细胞减灭术和腹腔热化疗。我们介绍了一名34岁的男性,他表现出体重减轻,盗汗,胸膜炎性胸痛,腹部影像学发现腹水伴腹膜结节。他有结核接触史,但没有石棉接触史.经过漫长而有趣的诊断过程,他随后被诊断为双相MPM.诊断挑战不仅源于肿瘤的稀有性,还源于缺乏危险因素,无法进行一些特殊的实验室调查,除了结核病暴露史的潜在误导性影响,病例的最高鉴别诊断。这是一个非常具有挑战性和完全意想不到的诊断双相性腹膜间皮瘤的结核暴露的病例报告。宪法症状,但没有石棉接触史.它强调了诊断过程以及早期诊断对提高此类恶性肿瘤总体生存率的重要性。
    Malignant peritoneal mesothelioma (MPM) is a rare subtype of mesothelioma. There are three main histological subtypes of mesothelioma: epithelioid, sarcomatoid, and biphasic (mixed). Risk factors include asbestos exposure, previous radiation, and some germline mutations. Treatment includes surgical resection of amenable tumors or cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. We present a 34-year-old male who presented with weight loss, night sweats, and pleuritic chest pain and was found to have ascites with peritoneal nodularity on abdominal imaging. He had a history of tuberculosis contact, but no history of asbestos exposure. After a long challenging and interesting diagnostic process, he was subsequently diagnosed with biphasic MPM. The diagnostic challenge stems from not only the rarity of the tumor but also from the absence of risk factors, the unavailability of some special laboratory investigations, in addition to the potentially misleading effect of tuberculosis exposure history, a top differential diagnosis in the case. This is a case report of a really challenging and totally unexpected diagnosis of biphasic peritoneal mesothelioma in a patient with tuberculosis exposure, constitutional symptoms, but no history of asbestos exposure. It highlights the diagnostic process as well as the importance of early diagnosis to improve the overall survival of such malignancies.
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  • 文章类型: Journal Article
    背景:石棉肺含量被认为是将恶性间皮瘤(MM)因果归因于先前石棉暴露的最可靠工具。然而,缺乏与健康个体相比MM患者肺部石棉负担的研究。这项研究旨在提供这样的比较,调查,还有,石棉肺负荷随性别和时间趋势的差异。
    方法:使用扫描电子显微镜结合能量色散谱(SEM-EDS),对福尔马林固定的肺碎片评估了石棉肺含量,并在2005年至2023年之间死于暴力原因的健康受试者(对照)。
    结果:发现石棉和石棉体(ABs),分别,在73.7%和43.2%的病例以及28%和22%的对照中;在MM病例中,最典型的石棉类型是青石棉和铁石棉,而在对照组中,它是透闪石-阳起石石棉。与对照组相比,MM病例中石棉纤维和AB的浓度在统计学上显着较高。平均石棉纤维宽度也明显高于对照组。患有MM的男性和女性显示出相似的石棉和ABs浓度,但是女性的温石棉浓度更高,与男性相比,纤维宽度显着降低。时间趋势表明,从2011年开始MM肺石棉浓度下降。
    结论:结果表明肺部石棉负荷与MM风险之间存在相关性。温石棉的浓度不同,MM男性和女性的石棉纤维宽度不同可能反映了肺部微环境对吸入石棉反应的性别差异。最后,这项研究提供了禁止石棉使用效果的第一个病理证据,表明2011年后石棉肺含量显著下降。
    Asbestos lung content is regarded as the most reliable tool for causal attribution of malignant mesothelioma (MM) to previous asbestos exposures. However, there is a lack of studies on asbestos burden in lungs of MM patients in comparison with healthy individuals. This study aims to provide such a comparison, investigating, as well, differences in asbestos lung burden with sex and time trends.
    Asbestos lung content has been assessed on formalin-fixed lung fragments using scanning electron microscopy coupled with energy dispersion spectroscopy (SEM-EDS) on individuals deceased from MM (cases) and healthy subjects without any lung disease who died from violent causes (controls) between 2005 and 2023.
    Asbestos and asbestos bodies (ABs) were found, respectively, in 73.7% and 43.2% of cases and in 28 and 22% of controls; in MM cases the most represented asbestos types were crocidolite and amosite, whereas in controls it was tremolite-actinolite asbestos. The concentration of both asbestos fibers and ABs was statistically significantly higher in MM cases compared to controls. The mean asbestos fibers width was also significantly higher in cases than controls. Males and females with MM showed similar asbestos and ABs concentrations, but females had higher concentrations of chrysotile, and significantly lower fibers width compared to males. Time trends show that MM lung asbestos concentrations decreased starting in 2011.
    The results suggest a correlation between asbestos burden in lungs and MM risk. The different concentration of chrysotile, as well as the different width of asbestos fibers in MM males and females might reflect a sex difference in response of the lung microenvironment to inhaled asbestos. Finally, this study provides the first pathological evidence of the effect of the ban of asbestos use, demonstrating a significant decrease of asbestos lung content after 2011.
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