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Aggressive Malignant Mesothelioma In A Patient Without Previous Asbestos Exposure

机译:没有石棉暴露的患者中侵袭性恶性间皮瘤

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We describe an 80-year-old male with a history of idiopathic lung fibrosis who was feeling generally unwell over a few weeks. Tissue biopsy from a rapidly growing ulceratedskin lesion on the abdomen showed a malignant epithelioid tumour positive for epithelial, mesothelial and embryonal tumour markers. The patient deteriorated rapidly and passed away. A post mortem examination was carried out and revealed thickened right pleura with multiple intra and extra-thoracic metastatic deposits. Histology of the pleural thickening and the multiple deposits were identical to that seen in the skin. The diagnosis of malignant epithelioid mesothelioma with rhabdoid features was made on histology. This is a rare case of malignant metastatic mesothelioma with ectopic hCG production and rhabdoid morphology in a patient without previous asbestos exposure. Introduction Malignant mesothelioma is an uncommon, pleural tumour which accounted for 2% of all cancer deaths in the UK in 2014 (1). Approximately seven new cases are reported every day in the UK and the incidence is increasing (1). A majority of pleural mesotheliomas are related to asbestos exposure with a long latency period (2). Malignant mesotheliomas uncommonly give rise to clinically detected metastases with a small number of distant subcutaneous metastasis cases reported (3). Only a handful of malignant mesothelioma cases showing rhabdoid morphology have been reported (4). Malignant mesothelioma with ectopic hCG (human chorionic gonadotropin) production is rare though described in literature (5, 6). We report a case of metastatic malignant mesothelioma presenting with a skin lesion in a patient without previous exposure to asbestos.We also report malignant mesothelioma associated with ectopic production of hCG and rhabdoid morphology, both of which are rare features and have not been reported together before. Case report An 80-year-old retired banker with multiple medical comorbidities including idiopathic lung fibrosis was admitted to hospital for feeling generally unwell in the last few weeks. He was a non-smoker and had no previous history of exposure to asbestos. He was under the care of the respiratory physicians for his lung condition and had been on an anti-fibrotic agent, Pirfenidone for the last two years. He remained clinically stable whilst on this agent. He was last seen in the respiratory clinic one month prior to feeling unwell when he was recruited for a pharmacokinetics drug trial. This trial lasted for one week where he was on both Pirfenidone and Nintedanib. Both drugs were stopped when his blood test results showed an increase in inflammatory markers. He was briefly admitted for pneumonia and then discharged. Three days after his discharge, he became progressively poorly and was readmitted.During this admission, he was found to have massive right pleural effusion and a chest drain was inserted. The pleural fluid was sent for cytology and the result was reported as blood only. An ulcerated skin lesion (figure 1) was identified on his abdomen which the patient noticed only a few weeks ago.
机译:我们描述了一位有特发性肺纤维化病史的80岁男性,几周后总体感觉不适。腹部溃疡性皮肤病变迅速生长的组织活检显示,上皮,间皮和胚胎肿瘤标志物阳性的恶性上皮样肿瘤。病人迅速恶化并去世。进行尸检后发现右胸膜增厚,并伴有胸内和胸外转移性沉积物。胸膜增厚和多次沉积的组织学与皮肤中观察到的相同。根据组织学诊断为具有横纹肌样特征的恶性上皮样间皮瘤。在没有石棉暴露的患者中,这是罕见的恶性转移性间皮瘤伴异位hCG产生和横纹肌样形态。简介恶性间皮瘤是一种罕见的胸膜肿瘤,占2014年英国所有癌症死亡的2%(1)。在英国,每天大约有7例新病例报告,而且发病率正在增加(1)。大多数胸膜间皮瘤与石棉接触有关,潜伏期较长(2)。恶性间皮瘤通常会引起临床检测到的转移,并报告了少数远处皮下转移病例(3)。据报道只有少数恶性间皮瘤病例呈横纹肌形态(4)。尽管文献中有描述,但异位间皮瘤伴异位hCG(人绒毛膜促性腺激素)的产生很少见(5、6)。我们报道了一例转移性恶性间皮瘤患者在未曾接触石棉的情况下出现皮肤病变;我们还报道了恶性间皮瘤与异位产生hCG和类横纹肌形态有关,这两者都是罕见的特征,之前尚未一起报道过。病例报告一名患有多种疾病(包括特发性肺纤维化)的80岁退休银行家因过去几周的总体不适而入院。他是不吸烟者,以前没有接触过石棉的历史。他的肺部疾病正在呼吸内科医师的照顾下,并且在过去两年中一直服用抗纤维化药吡非尼酮。在使用该药物时,他保持临床稳定。当他被招募参加药物动力学药物试验时,他在感到不适之前一个月才在呼吸系统诊所见过。该试验持续了一个星期,在那里他同时服用了吡非尼酮和Nintedanib。当他的血液检查结果显示炎性标志物增加时,两种药物都停止使用。他因肺炎短暂入院,然后出院。出院三天后,他逐渐恶化并重新入院。在入院期间,他被发现患有右胸腔积液,并插入了胸腔引流管。胸膜液被送去进行细胞学检查,结果仅报道为血液。仅在几周前,患者腹部发现了溃疡性皮肤病变(图1)。

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