Primary Pericardial Tumors Research Articles

Pericardial Disease in Patients with Cancer: Clinical Insights on Diagnosis and Treatment

Pericardial disease is increasingly recognized in cancer patients, including acute pericarditis, pericardial effusion, and constrictive pericarditis, often indicating a poor prognosis. Acute pericarditis arises from direct tumor involvement, cancer therapies, and radiotherapy. Immune checkpoint inhibitor (ICI)-related pericarditis, though rare, entails significant mortality risk. Treatment includes NSAIDs, colchicine, and corticosteroids or anti-IL1 drugs in refractory cases. Pericardial effusion is the most frequent manifestation, primarily caused by lung cancer, followed by breast cancer, lymphoma, leukemia, gastrointestinal tumors, and melanoma. Chemotherapy, immunotherapy, and radiotherapy may also cause fluid accumulation in the pericardial space. Symptomatic relief for pericardial effusion may require pericardiocentesis, prolonged catheter drainage, or a pericardial window. Instillation of intrapericardial cytostatic agents may reduce recurrence. Constrictive pericarditis, though less common, often develops from radiotherapy and requires multimodality imaging for diagnosis, with pericardiectomy as the definitive treatment. Primary pericardial tumors are rare, with metastases being more frequent. Patients with cancer and pericardial disease generally have poor survival, emphasizing the need for early detection. A multidisciplinary approach involving hematologists, oncologists, and cardiologists is crucial to tailoring pericardial disease treatment to a patient’s clinical status, thereby improving the quality of life and prognosis.

Primary pericardial tumor presenting with cardiac tamponade: a case report

Primary pericardial tumor presenting with cardiac tamponade: a case report

Pericardial Disease in Patients with Cancer

Pericardial effusion can develop in patients with acute pericarditis or in association with a wide variety of systemic diseases and is characterized as transudative, exudative, pyopericardium, or hemopericardium. Large effusions are usually related to tuberculous or neoplastic effusions. Primary pericardial tumors are rare, with the vast majority of cases resulting from secondary tumors. Pericardial effusion may be present in 7% to 53% of patients with cancer and is correlated with an advanced stage of the disease. The main types [...]

Abstract 14362: A Case of a Primary Malignant Pericardial Myxoid Sarcoma in a Young Adult

Introduction: Primary pericardial malignant tumors are rare clinical entities with an incidence of 0.001%. The symptoms depend on the location and size of the mass, typically causing chest pain, dyspnea, or syncope. We present the case of a young man with atypical chest pain, rapidly progressing to cardiac arrest from a primary pericardial myxoid chondrosarcoma. Case Presentation: A 31-year-old man presented with atypical chest pain. Subsequently, he had a cardiac arrest with pulseless electrical activity. CT chest revealed a 9.5 x 10 cm epicardial mass (Figure 1a, 1b) found to be a myxoid sarcoma on CT-guided biopsy. The morphology was suggestive of a high-grade extra-skeletal myxoid chondrosarcoma. Cardiac MRI had normal function and no myocardial invasion. The staging MRI did not show any other primary source. Due to the large size of the mass, a decision was made to perform surgical resection. Most of the tumor was excised but had positive margins from the part adherent to the inferior vena cava. In the absence of clear guidelines, multiple chemotherapy regimens including doxorubicin, ifosfamide, and etoposide were tried. The initial response was complicated by eventual recurrence and metastasis to the chest wall and mediastinum (Figure 1c, 1d). Further, genetic testing with next-generation sequencing demonstrated MAP3K1 gene mutation. As MAP3K1 mutations have shown sensitivity to MEK inhibition, targeted chemotherapy with trametinib was initiated in combination with pazopanib, a multikinase inhibitor. With the progression of metastatic disease despite treatment, the patient decided to pursue palliative care. Discussion: Malignant pericardial myxoid chondrosarcoma is a rare tumor that may lead to fatal outcomes in young adults. Despite numerous surgical and chemotherapeutic interventions, they have a guarded prognosis. Increased clinician awareness and a wide differential while evaluating cardiac masses are key for early detection and timely treatment.

Primary Pericardial Tumors

Primary pericardial tumors are rare and may be classified as benign or malignant. The most common benign lesions are pericardial cysts and lipomas. Mesothelioma is the most common primary malignant pericardial neoplasm. Other malignant tumors include a wide variety of sarcomas, lymphoma, and primitive neuroectodermal tumor. When present, signs and symptoms are generally nonspecific. Patients often present with dyspnea, chest pain, palpitations, fever, or weight loss. Although the imaging approach usually begins with plain radiography of the chest or transthoracic echocardiography, the value of these imaging modalities is limited. Cross-sectional imaging, on the other hand, plays a key role in the evaluation of these lesions. Computed tomography and magnetic resonance imaging allow further characterization and may, in some cases, provide diagnostic findings. Furthermore, the importance of cross-sectional imaging lies in assessing the exact location of the tumor in relation to neighboring structures. Both benign and malignant tumors may result in compression of vital mediastinal structures. Malignant lesions may also directly invade structures, such as the myocardium and great vessels, and result in metastatic disease. Imaging plays an important role in the detection, characterization, and staging of pericardial tumors; in their treatment planning; and in the posttreatment follow-up of affected patients. The prognosis of patients with benign tumors is good, even in the few cases in which surgical intervention is required. On the other hand, the length of survival for patients with malignant pericardial tumors is, in the majority of cases, dismal.

A rare cause of pericardial disease

Among cardiovascular diseases, pericardial disease has specific characteristics. Its etiology, diagnosis and medical management are not as well understood as in coronary and valvular heart disease. In most cases, its cause is benign, although the proportion decreases with more severe clinical presentation.The authors present the case of a 35-year-old man with no relevant past medical history, who went to the emergency department with what appeared to be an idiopathic case of acute pericarditis. However, over the following five months, there was an unfavorable evolution to constrictive pericarditis, requiring pericardiectomy. The final diagnosis was only made following surgery – a rare case of a primary pericardial tumor, a mesothelioma.

Causa rara de doença pericárdica

Among cardiovascular diseases, pericardial disease has specific characteristics. Its etiology, diagnosis and medical management are not as well understood as in coronary and valvular heart disease. In most cases, its cause is benign, although the proportion decreases with more severe clinical presentation.The authors present the case of a 35-year-old man with no relevant past medical history, who went to the emergency department with what appeared to be an idiopathic case of acute pericarditis. However, over the following five months, there was an unfavorable evolution to constrictive pericarditis, requiring pericardiectomy. The final diagnosis was only made following surgery – a rare case of a primary pericardial tumor, a mesothelioma.

Intrapericardial Hemangioma:A Case Report

Hemangiomas of the pericardium are very rare primary pericardial tumours. Very few cases of pericardial hemangioma have been reported in the literature till date. We are reporting a case of a 40 years old male who presented with the complaints of chest discomfort and palpitation on exertion, of 3 months duration. ECHO and Colour Doppler ECHO which were done, revealed a large heterogeneous mass which measured 7.6 × 7.1cms, which was possibly attached to the roof and the anterior surface of the left atria, which was possibly a myxoma. Based on the radiological findings, a diagnosis of left atrial myxoma was considered. However, intra operatively, an encapsulated mass was seen within the pericardium, which was successfully excised and sent for histopathology. The histopathological examination revealed an intrapericardial hemangioma. This case has been reported on account of its extreme rarity.

Primary Pericardial Sarcoma: A Case Report and a Brief Review

There are very few cases of primary pericardial sarcomas reported in the English literature. Pericardial tumors, like other cardiac tumors, are most likely to be metastatic in nature and are an extension of primary tumors from the surrounding structures. Sarcomas are the most common primary pericardial tumors. Surgical eradication of the tumor is considered to be the treatment of choice. We are presenting a case of a primary pericardial, high-grade pleomorphic undifferentiated sarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates.

Primary malignant mesothelioma of the pericardium

Primary malignant pericardial mesothelioma is an extremely rare tumor. One of the largest autopsy series gave an incidence of primary pericardial tumors of 0.0022%, of which mesothelioma is the most common type. A male predominance of the disease has been described, and the majority of cases occur in the fourth to seventh decades of life. Unlike peritoneal and pleural mesothelioma, there has been no definite correlation between asbestos exposure and pericardial disease. Malignant pericardial mesothelioma carries a poor prognosis with few successful treatment strategies and little benefit from radiation and chemotherapy. We report a case of a 66-year-old man who presented with shortness of breath, right shoulder pain, and peripheral edema of the lower limbs. A large pericardial effusion was seen on echocardiography, which was drained but the patient died the following day. A malignant tumor was found on autopsy and a final diagnosis of primary malignant pericardial mesothelioma was made following histopathological examination.

A Rare Case with Primary Undifferentiated Carcinoma of Pericardium

A primary pericardial tumor is very rare. A 77-year-old woman was admitted to our hospital with chief complaint of exertional dyspnea due to large amount of pericardial effusion. She was finally diagnosed as pericardial undifferentiated carcinoma without definite histopathologial, immunochemistry feature. Despite palliative radiation therapy, the patient died of multiple organ failure. The prognosis of primary pericardial undifferentiated carcinoma is known to be very poor, especially in old people.

Pericardium--radiologic diagnosis

Evaluation of the pericardium using the capabilities of computed tomography (CT) and magnetic resonance imaging (MRI) remains one of the last requests to the radiologists within the spectrum of cardiac diagnostics. New technical developments in CT and MRI improve diagnostic accuracy in diagnosing pericardial disease and help to define adequate therapeutic management. The purpose of this article is to review the diagnostic possibilities of the radiologist in pericardial diseases with emphasis on CT and MRI. The anatomy of the normal pericardium including pericardial recessus and sinuses is reviewed followed by a brief discussion of congenital abnormalities. Particular attention is paid to acquired pericardial diseases including the potential characterization of pericardial effusions. Pericardial thickening and pericardial constrictions are discussed and the differentiation between pericardial constriction and restrictive cardiomyopathy is highlighted because of the therapeutic implications. Finally a brief review of primary and metastatic pericardial tumours is given.

The role of pericardiectomy in pericardial disorders

Pericardiectomy today is an accepted therapeutic concept in patients with different pericardial disorders. The postoperative outcome of patients is mainly influenced by two factors. First, diagnosis has to be established early to avoid myocardial deterioration and secondary organ failure, especially of liver and kidneys. Second, the whole accessible surface of atria and ventricles has to be freed from diseased and calcified pericardium. To achieve this, we prefer a total median sternotomy for the surgical approach. In selected cases of acute pericardial effusion or as a palliative procedure, a small infrasternal incision or anterolateral thoracotomy is used for pericardiocentesis and creation of a pericardio-pulmonary window. Between January 1969 and March 1990 we treated 187 patients with different pericardial disorders. Mortality was 4.8% overall, and was especially low (2.8%) among the 106 patients with acute and chronic pericardial effusion. Out of 67 patients with constrictive pericarditis, four died during hospital stay (5.9%). The prognosis is still poor for patients with primary or secondary malignant pericardial tumours, in whom surgery is mostly restricted to palliative resection, and a special group with constrictive and mostly calcified epicarditis, for whom no surgical option is available.

In vivo diagnosis of pericardial mesothelioma

According to the literature, primary pericardial tumors are extremely rare, rarer than cardiac tumors. By 1980, 117 reports on this pathology had been published in the world literature, most of which were based on autopsy data. Difficulties of in vivo diagnosis of pericardial mesothelioma are due to the absence of pathognomonic symptoms. The presence of hemorrhagic effusion in the absence of isoinflammatory manifestations and the results of cytological study of the exudate allow suspecting the tumor.Here is our observation.

Primary Pericardial Tumor

To the Editor.— Recently we reported, inThe Journal(230:722, 1974), a case of primary pericardial tumor masquerading as constrictive pericarditis. Since there has been a lot of interest concerning the histologic diagnosis of this pericardial tumor, we thought it might be of interest to indicate that this was a primary mesothelioma of the pericardium (Figure).

Primary Pericardial Tumor Masquerading as Constrictive Pericarditis

WE REPORT an unusual clinical course and pathologic findings in a patient with primary pericardial tumor that masqueraded as constrictive pericarditis. Of approximately 44 case reports of primary pericardial tumors reviewed, only seven were diagnosed before death. The diagnosis in this patient was established antemortem by thoracotomy after cardiac catheterization disclosed findings of pericardial constriction. Report of a Case A 41-year-old man sought medical advice when he noted increasing abdominal distention, gastrointestinal upset, and constipation. He denied shortness of breath, dyspnea on exertion, cough, chest pain, night sweats, or fever. Because of a nonpulsatile epigastric mass, hepatomegaly, and ascites on physical examination, an exploratory laparotomy was performed, during which hypotension and cyanosis developed. Because of a lack of transmitted cardiac pulsations and a downward distention of the diaphragm, a transdiaphragmatic pericardiocentesis was performed and yielded 1,800 ml of sanguineous fluid with reactive mesothelial cells, but no tumor cells. The blood

Primary pericardial tumor masquerading as constrictive pericarditis

WE REPORT an unusual clinical course and pathologic findings in a patient with primary pericardial tumor that masqueraded as constrictive pericarditis. Of approximately 44 case reports of primary pericardial tumors reviewed, only seven were diagnosed before death. The diagnosis in this patient was established antemortem by thoracotomy after cardiac catheterization disclosed findings of pericardial constriction. <h3>Report of a Case</h3> A 41-year-old man sought medical advice when he noted increasing abdominal distention, gastrointestinal upset, and constipation. He denied shortness of breath, dyspnea on exertion, cough, chest pain, night sweats, or fever. Because of a nonpulsatile epigastric mass, hepatomegaly, and ascites on physical examination, an exploratory laparotomy was performed, during which hypotension and cyanosis developed. Because of a lack of transmitted cardiac pulsations and a downward distention of the diaphragm, a transdiaphragmatic pericardiocentesis was performed and yielded 1,800 ml of sanguineous fluid with reactive mesothelial cells, but no tumor cells. The blood