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Primary malignant pericardial mesothelioma (PMPM) is a rare pericardial malignant tumor. Most manifestations of PMPM are localized or diffuse masses surrounding the heart. The prognosis of diffuse PMPM is poor due to the difficulty of surgical resection. Although the edge of localized PMPM is clear and can be easily resected, the diagnosis of this disease is difficult. Timely diagnosis and proper treatment are key to a good prognosis. Here, we report a patient with localized PMPM and describe the method for the diagnosis of this disease.

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Malignant pericardial mesothelioma (MPM) is extremely rare, and peritoneal dissemination has not yet been reported. There is no consensus regarding appropriate pharmacological treatment for MPM, including immune checkpoint inhibitors (ICIs). We herein report a 36-year-old man with MPM diagnosed by peritoneal dissemination and treated with an ICI. Cytology of the ascites revealed malignant peritonitis, and a re-evaluation of a pericardial biopsy performed at the previous hospital led to a diagnosis of MPM. The patient was treated with nivolumab and showed a clinical response despite several complications, such as renal dysfunction and performance status deterioration. This case report provides suggestive information for the diagnosis and ICI therapy of a rare type of mesothelioma.

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Case 1: Sixty-seven-year-old male. Presented with exertional dyspnea, raising suspicion for constrictive pericarditis. His condition rapidly progressed to a state of shock, necessitating emergency surgery under percutaneous cardiopulmonary support. Pericardiectomy using the waffle procedure was performed. However, there was limited improvement in hemodynamics, and he died on the 17th postoperative day. Postoperative pathological examination diagnosed epithelioid malignant pericardial mesothelioma. Case 2: Sixty-nine-year-old female. Presented with exertional dyspnea, raising suspicion for constrictive pericarditis. While being transported for surgical intervention, she went into shock, followed by cardiopulmonary resuscitation and initiation of percutaneous cardiopulmonary support. Waiting for the improvement of multiple organ failure, pericardiectomy using the waffle procedure was conducted. However, there was limited improvement in hemodynamics, and she died on the 2nd postoperative day. Postoperative pathological examination diagnosed sarcomatoid malignant pericardial mesothelioma.

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Background: Malignant mesothelioma (MM) is a rare cancer with poor prognosis. It is less common that two serosal cavities are involved when the patient seeks medical attention firstly. The current first-line chemotherapy for advanced MM is a combination with cisplatin and pemetrexed. However, nedaplatin, a second-generation platinum-based antitumor agent, has the similar therapeutic effects as cisplatin but lower toxicity and higher water solubility. To our knowledge, this is the first case of co-existing pericardial and pleural MM treated with nedaplatin and pemetrexed and responding well. Case Description: A 33-year-old woman, who had worked in a kiln for more than 10 years, suffered from dyspnea and chest tightness for 6 days. Chest computed tomography (CT) showed a massive pericardial effusion. She was diagnosed tuberculous pericarditis and received 6 months antituberculosis treatment (rifampicin, isoniazide, pyrazinamide, ethambutol). But it was ineffective and she was re hospitalized again due to massive pleural effusion and pericardial effusion. She was diagnosed with co-existing pericardial and pleural MM finally based on pleural biopsy and cytology of pericardial effusion. She was responding well excitedly to chemotherapy with nedaplatin and pemetrexed with high tolerance. Bone marrow toxicity or recurrent massive pericardial or pleural effusion were not observed during chemotherapy. However, she gave up chemotherapy and has survived for 22 months, from the onset symptoms. Conclusions: In terms of clinical tolerance and less adverse reactions, we suggest that chemotherapy of nedaplatin with pemetrexed may be a more appropriate treatment in advanced MM. Further clinical trials are warrant.

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BACKGROUND: Primary malignant pericardial mesothelioma (PMPM) is an extremely rare malignant tumor, and it is difficult to diagnose definitively before death. We present a case in which PMPM was diagnosed at autopsy. We consider this case to be highly suggestive and report it here. CASE SUMMARY: A 78-year-old male presented with transient loss of consciousness and falls. The transient loss of consciousness was considered to result from complications of diastolic dysfunction due to pericardial disease, fever with dehydration, and paroxysmal atrial fibrillation. Ultrasound cardiography (UCG) and computed tomography showed cardiac enlargement and high-density pericardial effusion. We considered pericardial disease to be the main pathogenesis of this case. Cardiac magnetic resonance imaging and gadolinium contrast-enhanced T1-weighted images showed thick staining inside and outside the pericardium. Pericardial biopsy was considered to establish a definitive diagnosis, but the patient and his family refused further treatment and examinations, and the patient was followed conservatively. We noticed a thickening of the pericardium and massive changes in the pericardium on UCG over time. We performed an autopsy 60 h after the patient died of pneumonia. Giemsa staining of the autopsy tissue showed an epithelial-like arrangement in the pericardial tumor, and immunostaining showed positive and negative factors for the diagnosis of PMPM. Based on these findings, the final diagnosis of PMPM was made. CONCLUSION: PMPM has a poor prognosis, and early diagnosis and treatment are important. The temporal echocardiographic findings may provide a clue for the diagnosis of PMPM.

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Malignant pericardial mesothelioma (MPM) is a rare tumour that arises from the mesothelial cells of the pericardium. No standard treatment has been established owing to a poor treatment response; therefore, MPM has a poor prognosis. We herein report a rare case of MPM in a 70-year-old man that was diagnosed immunohistopathologically using cell block sections of pericardial fluid and in which long-term survival for more than 3 years was achieved with only periodic pericardial drainage. Immunohistopathological staining investigations, especially BRCA1-associated protein 1 (BAP1) immunostaining using cell block sections of pericardial effusion, are effective in making a diagnosis of MPM. Well-differentiated papillary mesothelioma (WDPM) with BAP1 loss progresses to MPM in the long term, showing that BAP1 loss may induce phenotypical evolution of WDPM. BAP1 loss may also progress to malignant mesothelioma in situ and then to invasive mesothelioma. BAP1 immunohistochemistry should be considered for the early diagnosis of MPM.

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Background: Primary malignant pericardial mesothelioma (PMPM) is a highly malignant tumor originating in the pericardium serosum with clinical manifestations presenting as constrictive pericarditis, with pericardial tamponade and heart failure. Malignant pericardial mesothelioma is rare and has a poor prognosis, with an average survival time of 6-10 months. Case Description: Herein, we report the case of a 57-year-old female who developed chest tightness and panic for no obvious reason. She was diagnosed with tuberculous pericarditis via multiple examinations including positron emission tomography/computed tomography (PET/CT), pleural biochemical routine, tuberculin purified protein derivative (PPD) test, T cell spot (T-SPOT) test, and echocardiography, and was experienced intermittent relief after anti-tuberculosis treatment. On 21 July, 2020, pericardiectomy was performed due to poor therapeutic effect, and the postoperative pathological diagnosis was malignant mesothelioma. After discussing treatment plans and considering the prognosis, the patient opted for palliative care. Subsequently, her symptoms gradually worsened, with chest tightness, shortness of breath, palpitations at rest, frequent arrhythmias, heart failure, cardiogenic shock, and multiple plasma chamber effusions. This case showed that the most common misdiagnosis of PMPM is tuberculous pericarditis, which needs to be differentiated from pleural mesothelioma with pericardial metastasis. Conclusions: The diagnosis of PMPM is usually made by pathologic surgery or histopathological examination to determine the specific disease location. In addition, pericardiocentesis fluid exfoliation cytology, imaging and echocardiography can assist diagnosis. Due to the lack of effective treatment for PMPM, timely surgery and postoperative adjuvant chemotherapy are needed to improve the quality of life of patients and prolong their survival time.

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Primary malignant pericardial mesothelioma is an extremely rare disease. Malignant disease of the pericardium is an infrequent cause of cardiac tamponade. Hence, cardiac tamponade in the context of primary malignant mesothelioma of the pericardium is an uncommon clinical scenario. A 67-year-old male patient, an ex-smoker, complaining of progressive lethargy was referred to a hospital for investigation of persistent pericardial effusion. The pericardial fluid cytology was categorized as class Ⅲ. Thereafter, he was referred to our hospital for further evaluation. Fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed FDG accumulation in the pericardium and mediastinal lymph node. Surgical biopsy of the pericardium was performed through a subxiphoid approach for a definitive diagnosis. Histopathological examination revealed diffuse infiltration of the pericardium by a malignant tumor consisting of epithelioid cells with large round nuclei and prominent nucleoli, arranged in a tubular papillary pattern. Finally, the patient was diagnosed with primary malignant pericardial mesothelioma of epithelioid type. The patient died 6 weeks after admission. This diagnosis must be considered in patients having unexplained massive pericardial effusion. Furthermore, we should consider prompt cytological analysis and FDG PET to arrive rapidly at a definitive diagnosis to administer combination chemotherapy that may provide clinical benefit. .

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We report a case of a 64-year-old woman with a history of radiation therapy for breast cancer 27 years ago who developed malignant pericardial mesothelioma. Since 3 years ago, the recurrent bloody pericardial effusion was getting worse, which caused general edema and nocturnal dyspnea. She had a thickened pericardium and the right ventricular pressure curve showed a dip-and-plateau pattern. We diagnosed constrictive pericarditis and performed a pericardiectomy and waffle procedure on the thickened epicardium without cardiopulmonary bypass. The post-operative histology confirmed malignant pericardial mesothelioma and she died on the 17th postoperative day. Pericardial malignant mesothelioma is a rare disorder but very aggressive. This fatal disease may be considered in a patient with recurrent bloody pericardial effusion who has a history of thoracic radiation therapy.

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INTRODUCTION: Primary malignant pericardial mesothelioma is a rare tumor that is very difficult to diagnose. Furthermore, it is a lethal disease, because patients usually have progressed at the time of referral. PRESENTATION OF THE CASE: We report a 44-year-old man with primary malignant pericardial mesothelioma. He was referred to our hospital for the diagnosis and treatment of a massive pericardial effusion and huge tumor. Pericardiocentesis was performed, but we could not obtain definitive diagnosis, and the cardiac tamponade continued along with the signs/symptoms. He required surgical intervention for the diagnosis and treatment. After surgery, his signs/symptoms improved. He received adjuvant therapy, although he died 7 months after surgery. DISCUSSION: Primary malignant pericardial mesothelioma is a rare tumor. The most common signs and symptoms are related to constriction of the heart by the tumor and/or effusion. Even if the pericardial fluid specimen obtained by pericardiocentesis is negative for malignant cells, primary malignant pericardial mesothelioma should be included in the differential diagnosis. Because the malignancy is usually advanced at the time of diagnosis, it has been difficult to cure. Radiation and chemotherapy have been used in addition to surgery, but have been minimally effective. CONCLUSION: The outcome of our patient with pericardial malignant mesothelioma was dismal. The indications for surgical intervention should be carefully considered except for critical cases requiring alleviation of immediate life-threating conditions.

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Sudden cardiac death is an unexpected clinical condition that typically occurs due to a cardiac cause, generally within 1 h of symptom onset, in people with known or unknown cardiac disease. Primary malignant pericardial mesothelioma, as a cause of sudden death, is an uncommon consequence of a rare disease. Herein, we present a case of cardiac tamponade due to a primary pericardial mesothelioma. Cytological, histopathology and gross post-mortem findings, in a previously asymptomatic 46-old-year man, are reported. The medical literature regarding this topic is also reviewed.

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Primary malignant pericardial mesothelioma is a rare cardiac neoplasm. The authors evaluated risk factors, clinical presentation, and outcomes by reviewing all biopsy-confirmed cases at one institution. The use of multimodality imaging, detailed hemodynamic assessment for the presence of an effusive-constrictive profile, and cytology evaluation can support the diagnosis. (Level of Difficulty: Advanced.).

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Primary malignant pericardial mesothelioma (PMPM) is an aggressive tumor that originates from the mesothelial cells of the pericardium. PMPM with extensive atrial infiltration and bone metastasis is extremely rare. The diagnosis and staging of PMPM based on anatomical imaging may be difficult when concurrent pericardial and pleural effusions are present. A 28-year-old man presented with progressive chest pain. Concurrent pericardial and pleural effusions were identified on computed tomography. On echocardiography, mild thickening and adhesions of the pericardium with the right ventricle and atrium were observed. 18F-fluorodeoxyglucose (FDG) metabolism imaging revealed increased accumulation in the pericardium and adjacent right atrium. Ring-shaped radioactivity aggregation and bone destruction in the sacrum were demonstrated on 18F-FDG and 99mTc-methyl diphosphonate imaging. The diagnosis of PMPM was subsequently confirmed by pathology. The patient survived for >1.5 years with comprehensive treatment.

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A 37-year-old female smoker without a history of exposure to asbestos was referred to our hospital with persistent pericardial effusion. Chest computed tomography imaging examination revealed an irregular thickened pericardium with large amounts of pericardial effusion and a small pleural effusion. Fluorodeoxyglucose (FDG) positron emission tomography imaging demonstrated intrapericardial FDG accumulation. Blood tests revealed an increase in serum mesothelin levels. Examination of a surgically resected specimen revealed a grayish-white thickening of the pericardium, with a straw-colored mucinous pericardial effusion. Histopathological examination confirmed the diagnosis of epithelioid malignant mesothelioma. Although the patient's condition temporarily improved, with decreased levels of serum mesothelin during chemotherapy with carboplatin and pemetrexed, she succumbed to cardiac tamponade 18 months after the initial onset of the symptoms. Primary malignant pericardial mesothelioma (PMPM) is an extremely rare and refractory disorder. Thus, an early definitive diagnosis and timely treatment are crucial for the management of PMPM.

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A 67-year-old man was admitted with right heart failure. He had severe peripheral edema of his lower limbs. The heart failure was treated by diuretics, but after 3 months, he was re-admitted with facial edema and pleural effusion. At this time, the pericardium thickened diffusely and rapidly, constricting the heart. Pericardiectomy was performed to alleviate symptoms of heart failure. The thickened pericardium firmly adhered to the epicardium, from which it was inseparable. A partial pericardiectomy was done. The diagnosis was not confirmed during operation, but primary malignant pericardial mesothelioma was diagnosed on immunohistological examination with carletinin. The patient died from massive pleural effusion and heart failure on the 22nd postoperative day. Primary malignant pericardial mesothelioma is an extremely rare pathology, which is difficult to diagnose and has a poor prognosis. However, this pathology is the disease which we should always mention as a cause of constrictive pericarditis.

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We report on a 21-year-old man with fever, dyspnea, and pleuritic chest pain. An electrocardiography showed ST elevation in multiple lead and thoracic echocardiography revealed moderate pericardial effusion. He was initially diagnosed with acute pericarditis, and treated with nonsteroidal anti-inflammatory drugs and colchicines with clinical and laboratory improvement. After 1 month of medication, his symptoms recurred. An echocardiography showed constrictive physiology and the patient was treated with steroid on the top of current medication. The patient had been well for 7 months until dyspnea and edema developed, when an echocardiography showed marked increased pericardial thickness and constriction. Pericardial biopsy was performed and primary malignant pericardial mesothelioma was diagnosed. Malignancy should be considered in the differential diagnosis of recurrent pericarditis.

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We report on a 21-year-old man with fever, dyspnea, and pleuritic chest pain. An electrocardiography showed ST elevation in multiple lead and thoracic echocardiography revealed moderate pericardial effusion. He was initially diagnosed with acute pericarditis, and treated with nonsteroidal anti-inflammatory drugs and colchicines with clinical and laboratory improvement. After 1 month of medication, his symptoms recurred. An echocardiography showed constrictive physiology and the patient was treated with steroid on the top of current medication. The patient had been well for 7 months until dyspnea and edema developed, when an echocardiography showed marked increased pericardial thickness and constriction. Pericardial biopsy was performed and primary malignant pericardial mesothelioma was diagnosed. Malignancy should be considered in the differential diagnosis of recurrent pericarditis.

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We report on a 21-year-old man with fever, dyspnea, and pleuritic chest pain. An electrocardiography showed ST elevation in multiple lead and thoracic echocardiography revealed moderate pericardial effusion. He was initially diagnosed with acute pericarditis, and treated with nonsteroidal anti-inflammatory drugs and colchicines with clinical and laboratory improvement. After 1 month of medication, his symptoms recurred. An echocardiography showed constrictive physiology and the patient was treated with steroid on the top of current medication. The patient had been well for 7 months until dyspnea and edema developed, when an echocardiography showed marked increased pericardial thickness and constriction. Pericardial biopsy was performed and primary malignant pericardial mesothelioma was diagnosed. Malignancy should be considered in the differential diagnosis of recurrent pericarditis.

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