Clinical case of angiosarcoma of pericardium and pleura

Primary heart tumors are an extremely rare pathology, previously detected only by autopsy data and detected in 0.001%-0.03% of cases. Primary angiosarcomas of the pericardium are even more rare and are presented in the literature with isolated descriptions. The article describes a clinical case of epithelioid angiosarcoma of the pericardium and pleura of high malignancy in a 57-year-old man. The diagnosis was confirmed by immunohistochemical examination and positron emission tomography. For today, chemotherapeutic treatment of angiosarcomas localized in the chest cavity is a palliative treatment method. In this patient, the most effective scheme was the one containing anthracyclines (doxorubicin). Despite the ongoing therapy, the patient died 15 months after the diagnosis was made, with increasing signs of respiratory and heart failure. Thus, when patients, especially young and middle-aged men, show signs of hydropericardium without established etiological prerequisites, it is necessary to remember about the possibility of developing a primary malignant tumor of the heart and / or pericardium as the cause of this condition and include it in the standard diagnostic search.

Surgical Treatment of Heart Mixomas: the Analysis of 10 Years’ Experience

Introduction. Primary cardiac tumors are rare, and the autopsy detection rate does not exceed 0.003%. Approximately 80% of primary heart tumors are benign, 50% of them are myxomas. Diagnosis of cardiac tumors at present in the presence of transesophageal echocardiography is not difficult in most cases. Surgical treatment of mixomas became possible after the introduction of open-heart surgery under cardiopulmonary bypass into clinical practice. After verification of the tumor, its surgical removal is indicated. In the practice of large cardiac surgery clinics, the experience of radical treatment of heart neoplasms is relatively small; therefore, the analysis of tactical approaches and results of operations in this pathology is of absolute interest.Aim of study. To present an analysis of 10 years’ experience in surgical treatment of cardiac mixomas. Material and methods The results of treatment of 58 patients with myxomas who underwent surgical removal of the neoplasm of the heart are presented.Results. Hospital mortality after surgical removal of myxoma was 1.7%, 1 patient died. All patients, we have the long-term period data about, noted a significant improvement in well-being, a decrease or disappearance of dyspnea at rest and/or during exertion, and no recurrence of myxoma was noted. Conclusion. The data obtained indicate, on the whole, good immediate results and high quality of life of patients in the long-term period operated on for heart mixomas.

Interactions Networks for Primary Heart Sarcomas

Personalized medicine incorporates genetic information into medical practice so as to optimize the management of chronic diseases. In rare diseases, such as heart cancer (incidence 0.0017–0.33%), this may be elusive. Ninety-five percent of the cases are due to secondary involvementwith the neoplasm originating in the lungs, breasts, kidney, blood, or skin. The clinical manifestations of heart tumors (benign or malignant) include heart failure, hypertension, and cardiac arrhythmias of varying severity, frequently resulting in blood vessel emboli, including strokes. This study aims to explain the pathophysiology and contribute to a P4 medicine model for use by cardiologists, pathologists, and oncologists. We created six gene/protein heart-related and tumor-related targets high-confidence interactomes, which unfold the main pathways that may lead to cardiac diseases (heart failure, hypertension, coronary artery disease, arrhythmias), i.e., the sympathetic nervous system, the renin-angiotensin-aldosterone axis and the endothelin pathway, and excludes others, such as the K oxidase or cytochrome P450 pathways. We concluded that heart cancer patients could be affected by beta-adrenergic blockers, ACE inhibitors, QT-prolonging antiarrhythmic drugs, antibiotics, and antipsychotics. Interactomes may elucidate unknown pathways, adding to patient/survivor wellness during/after chemo- and/or radio-therapy.

Echocardiographic Diagnostics of Heart Tumors

The article focuses on ultrasound diagnosis of cardiac tumors (CT). In recent time, the frequency of detecting cardiac neoplasm has been growing. Correct diagnosis at an early stage of the process would allow timely treatment. Before the introduction of two-dimensional echocardiography (EchoCG), life-time diagnosis of CT was very rare. This article describes major echocardiographic criteria for most common benign, malignant, and metastatic CTs. The article is illustrated with original echocardiographic images.

The Question of Relapse of a Cardiac Myxoma: an Unusual Case from Practice

Various studies demonstrate that recurrence of cardiac myxoma (CM) occurs in 2.1–5.3% of cases. In cases of treatment of familial myxoma, the need for reoperations can reach 20–25%. It should be taken into account that among all benign cardiac tumors, CM is detected in up to 80% of cases, and the need for its surgical removal is beyond doubt. The aim. To present a case that is of particular interest due to an unusual multiple myxomas lesion of the heart of multi-chamber localization. Material and methods. This article presents an unusual case of detecting multiple myxomas of multi-cameral heart lesions 6 years after removal of myxoma of the left atrium of typical localization. The distinguishing feature of the course of the disease lies in the fact that in this case, a careful study of the life history did not reveal the characteristic signs of familial myxomas and Carney complex which is most often characteristic of multi-chamber lesions of the heart by a tumor process. At the same time, during the surgical removal of the myxoma of the left ventricle, the myxomas of the left and right atrium were revealed, which began to grow. A technical feature of the removal of the left ventricular myxoma of the apical localization was the use of video cameras and instruments for thoracoscopic surgery, which greatly facilitated revi-sion and removal of the hard-to-reach neoplasm. Our experience in surgical treatment of CM equals to 868 operations. Wherein, repeated operations performed in 12 patients were due to recurrence or appearance of a tumor in place other than the primary localization. Conclusions. CM recurrence is one of infrequent complications in the postoperative period, which, however, requires some vigilance and annual Echo monitoring. In the vast majority of cases, CM was represented by the so-called sporadic myxomas, which are more often localized in the left atrium, and rarely recur. If CM is suspected, it is necessary to exclude the possibility of “myxoma syndrome”, which has the frequency of 0.8%, according to our data. The use of the instruments for thoracoscopic surgery can greatly facilitate the removal of hard-to-reach heart tumors localization.

Fibroma of the interventricular septum with bilateral obstruction of the ventricular outflow/tracts

Heart fibroma accounts for about 5% of all primary neoplasms and is registered in children in 80% of cases. The ventricles or interventricular septum (IVS) are most often affected; in half of cases, the tumor has intracavitary growth. The clinical picture of the disease and its prognosis depend on the size and location of the tumor. The most unfavorable is the defeat of the IVS, since it causes obstruction of the output tract of one of the ventricles. Being localized in IVS, can involve a conducting system of heart, thereby increasing risk of sudden death.A retrospective study of a case of cardiac fibroma in a child aged 4.5 months, confirmed by echocardiography, magnetic resonance imaging and successfully operated on in the Central Federal District Center of Penza, is presented. A large tumor, localized in the IVS, caused atypical obstruction of the outflow tracts of both ventricles simultaneously. In the domestic and foreign literature, hemodynamic disturbances of only one of the ventricles are described, and we did not find a single case with simultaneous obstruction of both tracts. The possibility of MRI in the diagnosis of heart tumors has been shown.

Valve dysfunction in patients with cardiac myxomas: mechanism of damage and methods of correction

The aim – to analyze the frequency and nature of lesions of the valve apparatus, to evaluate the results of surgical correction of the affected valves in heart myxomas. Materials and methods. In one-centre study, during period from 01.01.1969 to 01.10.2020, 962 patients with morphologically verified primary heart tumors were observed. Myxomas of the heart were found in 856 (89.0 %) patients. The pathology of the valve apparatus in combination with cardiac myxomas was noted in 70 (8.1 %) patients. The volume and nature of valve damage differed and depended on size, consistency and localization of myxoma, the degree of its mobility, and the presence of areas of pronounced fibrosis and calcification on the tumor. Results. Surgical correction of lesions of valve structures was performed in 65 patients. In 5 patients, it was possible to remove tumor tissue from the valve apparatus, followed by prevention of disease recurrence without damaging valve and subvalvular structures. Surgical techniques included both plastic manipulation and valve replacement. The features of the long-term postoperative period in such patients include progressive valve dysfunction manifested by the progression of insufficiency due to further deformation of the valve apparatus. Conclusions. Alongside with myxoma removal, it is necessary to conduct a thorough examination of the valve apparatus and correction of valve damage by various methods, including plastic surgery and valve replacement, depending on the nature and severity of the damage. Key words: cardiac tumors, myxoma, valve structures, surgical treatment.

Voluminous Intrapericardial Lipoma Mimicking Pericardial Effusion

Lipomas are rare primary heart tumors and may involve the endocardium, myocardium, or pericardium. Signs and symptoms depend on the tumor location and size. The intrapericardial lipoma we report has massive dimensions and mimics a pericardial effusion. A 38-year-old male complained of dyspnea and precordial pain. On physical examination, heart sounds were diminished. The patient had received extensive medication for a clinically suspected pericardial effusion due to heart failure. A voluminous mass resembling fat within the pericardial sac was revealed by transesophageal echocardiography and a computed tomography scan. The tumor was removed successfully by a subxiphoid surgical approach. The diagnosis of a 635 gram intrapericardial lipoma was confirmed by pathological examination. After surgery, the patient recovered well and was completely asymptomatic at a follow-up at 90 days. No medications were being taken since. The diagnosis of a pericardial effusion should be secured by imaging exams to avoid unnecessary medications. Cardiac lipomas can be readily recognized by their typical features on radiologic imaging. The surgical pathology examination confirms the diagnosis and rules out malignancy criteria.

Echocardiography and Newer Imaging Techniques in Diagnosis and Long-Term Follow-Up of Primary Heart Tumors in Children

Background: Primary heart tumors (PHTs) in the pediatric population are very rare and do not manifest any characteristic symptoms. Methods: A retrospective analysis of 61 cases was undertaken. Data from three centers for the years 2003–2018 were gathered. The tumors’ clinical course, location, number, hemodynamic, treatment, and follow-up were evaluated. Echocardiography was complemented with magnetic resonance imaging, computer tomography, and histopathological examination. Results: Out of 61 PHT diagnoses, 56 (91.8%) were circumstantial including all 16 (26.2%) prenatal tumors. The reasons for cardiological consultations were arrhythmia, syncopes, lowered physical performance, and murmurs. Only five patients (8.2%) were suspected of tumors based on previous symptoms of sclerosis tuberosa. Rhabdomyoma was the most frequently found PHT (60.7%). The tumors were predominantly located in the ventricles (49.1%) and intraventricular septum (14.9%) and tended to be single (70.5%). About 37.7% of patients suffered from coexistent multi-organ problems, two (3.28%) from congenital heart defects and one (1.64%) from Carney’s syndrome. Tumor resection was performed on 26 (42.7%) patients, of which 16 (61.5%) underwent total and 10 (38.5%) partial tumor resection. During the follow-up (mean 4.3 years), 54 patients (88.5%) have improved or were stable, while seven (11.5%) died. Conclusions: Primary pediatric heart tumors are diagnosed completely circumstantially, and the most common is rhabdomyoma, although arrhythmia may suggest fibroma. Diagnosis of a heart tumor in children is not synonymous with fatal prognosis, and most of them require only constant observation. Life-saving operation allows improvement, while the prognosis for malignant tumors in children is definitely unfavorable.

Ventricular tachycardia: beginning and ending fate of a benign invasive cardiac lipoma

Cardiac lipomas are rare benign heart tumors. Their clinical manifestations primarily depend on their dimension and location. We describe a case of a 14-year-old boy complaining of palpitations. ECG showed non-sustained ventricular tachycardia (NSVT). Echocardiography and cardiac MRI showed a large apical mass suggestive of a cardiac lipoma. The patient underwent open-heart surgery which detected a pericardial mass, partially attached to the myocardium, and crossed by the distal segment of LAD. This critical anatomical relationship prevents its complete resection. The biopsy confirmed mature fat tissue. Postoperatively, Propranolol was started for NSVT episodes. After 8 months, he presented with VT recurrence. A subcutaneous ICD (S-ICD) was implanted. Two months later, he was admitted for an electrical storm with six appropriate shocks. Since then, amiodarone was added. Lipomas are not always benign and their surgery might be very difficult and unhelpful. A multidisciplinary team is crucial for their diagnosis and management.