Three Cases of Intracardiac Leiomyomatosis with Very Long-term Follow-up.

Uterine intravascular leiomyomatosis (IVL) with extension into the right heart is uncommon, with no more than 400 cases reported in the literature since 1907. The present study aims to report three patients with intracardiac IVL surgically treated in our institution, with long-term follow-up. Three female patients in their third to fifth decades of life, with a history of difficult hysterectomy due to extensive myomatosis, presented with symptoms of right-sided heart failure. Echocardiography and computed tomography were performed, where IVL extending from the pelvis into the right heart was observed. All three patients underwent a one-stage operation under extracorporeal circulation through a right auriculotomy and inferior vena cavotomy, accessed via a sterno-laparotomy. The tumors were extirpated without complications, with ligation of the vena cava or iliac vein. The patients at 10-, 13-, and 37-year follow-up were well and alive with mild lower extremities symptoms.

Ultrasound, Histomorphologic, and Immunohistochemical Analysis of a Cardiac Tumor with Increased Purkinje Cells Detected in a Canine Fetus 42 Days into Pregnancy.

A seven-year-old healthy female Chow Chow was referred for pregnancy monitoring. Ultrasonography was used to evaluate all pregnancy and fetus parameters, and they were found to be normal. During the examination of the 42 day pregnant bitch, an unusual mass was seen in a fetus's heart. This fetus had a cardiac frequency of 273-300 beats, while the others had heart rates of 220-240 beats. Natural vaginal birth occurred at 63 days pregnant: the first two puppies were stillborn but perfectly formed, and the other three were alive and had optimal APGAR. In one of two deceased puppies, an unusual, reddish, smooth mass occupying the space in the heart was found through necroscopy. The organ was submitted for histological examination. Histopathology, immunohistochemical, and histochemical analyses all indicated a cardiac tumor with increased Purkinje cells. This type of tumor has been described in infants, swine, bearded seals, and deer but never in fetuses and neonates of dogs. To our knowledge, this is the first such case reported in veterinary medicine.

Rock on the Heart: A Case of Calcifying Fibrous Tumor Arising From the Pericardium and Literature Review.

Calcifying fibrous tumors are rare benign fibrous tumors that rarely occur in the heart. We report a 33-year-old woman who was found to have a benign pericardial tumor on health checkup, which was highly suspected to be a teratoma in clinical and imaging examination. After cardiac tumor resection, histopathological features showed scattered foci of psammoma bodies or calcification among collagenized fibrous connective tissues and foci of inflammatory cell infiltration in the interstitium. The pathological diagnosis was a calcifying fibrous tumor.

Intravascular leiomyomatosis with cardiac and pelvic involvement in a postmenopausal woman: A case report of multidisciplinary team management.

Intravascular leiomyomatosis (IVL) is a rare benign condition in which a leiomyoma, originating from the uterus, propagates through the pelvic venous system and occasionally extends into the inferior vena cava (IVC), occasionally reaching the heart. Despite its low incidence and benign nature, IVL can lead to life-threatening obstructions in the right heart's outflow tract, potentially resulting in sudden death. In this article, we present a case of a 72-year-old postmenopausal patient with IVL, who initially presented with palpitations. The diagnosis was made through echocardiography and a computerized tomography (CT) scan, revealing a tumor that extended from the uterus through the IVC and into the right ventricle. The patient was managed by a multidisciplinary team of gynecologists and cardiothoracic surgeons, who performed a single-stage surgical removal of a tumor 25 cm long. The pathological report confirmed the diagnosis of IVL. Postoperative follow-up is crucial, as IVL recurs in up to 30% of cases. This article's objective is to provide a clinical illustration of this exceedingly rare condition, with fewer than 300 reported cases, and to offer a comprehensive overview of IVL, including its clinical presentation, diagnosis, treatment, and outcomes.

Intravascular leiomyomatosis with cardiac extension, a case report.

BACKGROUND: Intravascular leiomyomatosis (IVL) is a histologically benign smooth muscle tumor arising from the uterus that can spread through the pelvic veins and, on rare occasions, extend as far as the heart via the inferior vena cava. Despite its benign characteristics, it can behave like a malignant tumor leading to significant morbidity and even mortality if left untreated. CASE PRESENTATION: The patient is a 42-year-old woman with a past medical history of uterine leiomyomas. She presented with heavy bleeding and frequent spotting; therefore, she went to her gynecologist. After further evaluation, a mass within the uterus that expanded into the pelvic veins, inferior vena cava, and right atrium was discovered. After the complete removal of the mass, the patient underwent full recovery. IVL with cardiac extension was the final diagnosis. CONCLUSION: Although IVL is rare, it must be considered in women who underwent previous hysterectomies or myomectomies and present with symptoms of right heart failure. The ideal therapy will need the aid of a multidisciplinary team and will depend on the patient's symptoms, previous operative history, the tumor's extension, and resectability.

Tuberous Sclerosis Associated With Aortic Stenosis and Endocarditis: A Case Report.

Tuberous sclerosis (TS) is a multisystem neurocutaneous disorder with an autosomal dominant pattern of inheritance. It is characterized by hamartomas that damage the skin, kidneys, lungs, heart, and central nervous system, among other organs. Rhabdomyomas, benign tumors of aberrant myocytes, are common in affected patients at birth. Depending on their size and location, these lesions might create valvopathies, which can cause heart failure or malignant arrhythmias, or they can cause obstruction of the outlet or inlet tract. Before making the diagnosis, a long time-even years-often passes. Early diagnosis can help prevent permanent irreversible complications. Differential diagnoses may include neurofibromatosis type 1, Sturge-Weber syndrome, and von Hippel-Lindau disease, among others. Diagnostic aids, such as MRI, CT scans, and genetic testing, can be useful in confirming a diagnosis of TS. Histological findings may include the presence of hamartomas, which are benign tumors composed of abnormal cells. Treatment for TS is mainly supportive and may involve medications to manage symptoms, and surgery to remove tumors. We present the case of a 23-year-old woman with TS who was admitted with macroscopic hematuria and fever, with further workup revealing tumor-like cardiac lesions associated with infective endocarditis.

Choledochoscope-guided treatment of pulmonary embolism caused by ventricular myxoma.

A 33-year male patient presented with a 6-month history of cough and shortness of breath upon physical activity. Echocardiography demonstrated right ventricular space-occupying lesions. Contrast-enhanced computed tomography of the chest showed multiple emboli in the pulmonary artery and its branches. Right ventricle tumor (myxoma) resection, tricuspid valve replacement, and clearance of the pulmonary artery thrombus were performed under cardiopulmonary bypass. Minimally invasive forceps and balloon urinary catheters were used to clear the thrombus. Clearance was confirmed by direct visualization using a choledochoscope. The patient recovered well and was discharged. The patient was prescribed oral warfarin 3 mg/day, and the international normalized ratio for prothrombin time was maintained between 2.0 and 3.0. Pre-discharge echocardiogram showed no lesion in the right ventricle or pulmonary arteries. The 6-month follow-up echocardiography indicated that the tricuspid valve was functioning well and showed no thrombus in the pulmonary artery.

Primary cardiac epithelioid angiosarcoma: A case report.

Primary cardiac angiosarcoma is an extremely rare, high-grade malignancy. Here, we describe the case of a 44-year-old male patient with a heart tumor in the left atrium wall, which caused a large amount of pericardial effusion that invaded the surrounding area and is visible on transthoracic echocardiography, computed tomography, and magnetic resonance imaging. The postoperative histopathological results confirmed this case as a primary cardiac epithelioid angiosarcoma.

A case of gastrointestinal stromal tumor metastasized to the left ventricular myocardium.

BACKGROUND: Gastrointestinal stromal tumors (GISTs), which are the most common soft tissue tumors of the gastrointestinal tract, originate from Cajal interneurons. The main metastatic sites of GISTs are the liver and intra-abdominal cavity, and metastasis to the heart is rare. CASE PRESENTATION: The patient was a 78-year-old man who was diagnosed with a rectal GIST 20 years previously. Since then, he had undergone repeated operations for metastasis. A follow-up thoracoabdominal computed tomography scan 4 months prior to the operation revealed GIST metastasis to the left ventricular myocardium. The patient wanted the tumor removed and consequently underwent an operation. The surgical findings showed a 3-cm × 3-cm mass in the lateral wall of the left ventricle. The mass was resected from the left ventricular wall in the shape of a tear drop. The left ventricular cavity was closed with a 4-0 polypropylene mattress suture and continuous suture. Postoperative histopathological findings showed nodular tumor growth consisting of bundles of spindle-shaped cells in the myocardium. The margins were negative. Immunostaining showed c-KIT (CD17) positivity and CD34 positivity, consistent with GIST metastasis. CONCLUSIONS: This case involved GIST metastasis to the heart muscle, which has rarely been reported worldwide.

Epithelioid hemangioma of the ascending aorta: A rare case.

Cardiac epithelioid hemangioma is extremely rare. Currently, there are only a few described cases of intraluminal hemangiomas of the aorta and the aortic valve and no described cases with extraluminal epithelioid hemangiomas of the ascending aorta. We now present a case of epithelioid hemangioma of the ascending aorta that was an incidental finding during the coronary artery bypass and successfully resected.

Unusual non-valvular left ventricular endocarditis presenting as multiple brain embolism.

INTRODUCTION: Masses inside the heart can cause serious and life-threatening effects to the cardiovascular system, mainly because of hemodynamic obstruction of the blood flow, either in the heart cavities themselves or remotely due to embolization. In this paper, we report a case of left ventricular tumor mass which presented with neurological symptoms due to multiple brain embolism. CASE REPORT: A 35-year-old female patient presented with right hemiparesis and dysarthria. Seven days prior to admission she had elevated body temperature and started taking antibiotics. Inflammatory markers were not elevated, and blood cultures were negative. Computerized tomography (CT) and magnetic resonance imaging (MRI) of the brain revealed multiple acute ischemic lesions. Echocardiography showed the presence of a lobular mass inside the left ventricle, which was attached to the basal segment of the lateral left ventricular wall. Based on the laboratory results and additional heart imaging (CT and MRI) the mass was primarily suspected to be a tumor. It was surgically removed. Microscopic analysis of the removed tissue revealed a non-specific endocardial inflammation with formed fresh fibrin thrombi on the surface. During the postoperative recovery intense physical rehabilitation was being performed, so the initial neurological deficit was completely withdrawn. CONCLUSION: Intracardiac masses can cause serious and potentially fatal complications that often present with dramatic clinical symptoms. Despite the comprehensive clinical, laboratory, and imaging investigations, intracardiac masses can be hard to distinguish until the definite microscopic analysis. However, with the right approach and multidisciplinary collaboration, they can be successfully managed.

Surgery for Valvular and Nonvalvular Papillary Fibroelastomas.

Papillary fibroelastomas (PFE) are benign neoplasms, mostly located on valvular surfaces with high embolic potential. This study presents a 27-year single institutional experience on surgical treatment of PFE in an adult patient- cohort with long-term follow-up. This study was approved by the institutional review board. Date and number of IRB approval: 11/23/2017, Institutional Review Board approval number A2014-0149. The need for individual patient consent was waived. We retrospectively evaluated all patients who underwent cardiac surgery for suspected space-occupying lesions in the observation period between June 1991 and June 2018 at our hospital. Clinicopathological features, imaging characteristics, surgical procedures and disease outcome were analyzed. 120 patients were diagnosed with various primary/secondary cardiac tumors and histology confirmed 21 PFEs were found in 16 patients. There was no significant age difference between patients with valvular vs nonvalvular PFEs (P = 0.26). Valvular lesions were found in aortic valve (n = 6), mitral valve (n = 2) and tricuspid valve (n = 1). Nonvalvular PFEs were found in right atrium (n = 2), left ventricle (n = 2), left atrial appendage (n = 2) and aortic wall (n = 1). Valvular lesions were significantly smaller in size compared to non-valvular lesions (P = 0.0013). Left-side PFEs were associated with a high embolization episodes (10/13 patients, 77%) not related to the size. One patient died in-hospital. All other patients were discharged out of the hospital postoperative. Follow-up was performed regularly for a median of 2.8 years (range 0.1-11 years) postoperative. Nonvalvular PFE tended to be larger in size and at least when located on the left sided heart had equally high propensity to embolize compared to valvular PFE. We strongly advocate surgical excision in all left-sided PFE.

Primary Pericardial Epithelioid Angiosarcoma: A Diagnostic Dilemma: A Case Report.

The incidence of pericardial epithelioid angiosarcoma is rare. Angiosarcoma of pericardium may coat the pericardium in a diffuse fashion. Diagnosis of an angiosarcoma is challenging and may be easily mistaken as constrictive pericarditis. Herein, a case of primary pericardial angiosarcoma is reported in a 16-year-old female. Patient presented with chest pain and dyspnea on exertion, regarded as constrictive pericarditis. Pericardectomy was performed and histopathologic examination showed pleomorphic epithelioid cells exhibiting hyperchromatic nuclei, prominent nucleoli and eosinophilic cytoplasm arranged in sheets and occasionally lined irregular vascular spaces. Moreover, immunohistochemical staining revealed that tumor cells were positive for CD31 and vimentin. The patient received chemotherapy with adriamycin, ifosfamide, and mesna. Unfortunately, the patient died of cardiac involvement and pleural metastases less than three months following the operation. Primary pericardial angiosarcoma is rare and difficult to diagnose, especially epithelioid variant. Immunohistochemical assessment is required to confirm the final diagnosis.

Multifocal precursor B-cell lymphoblastic lymphoma in an infant with cardiac involvement: A case report.

Lymphoma with cardiac involvement is a high-risk lesion, especially in children. We report a rare clinical case of multifocal precursor B-cell lymphoblastic lymphoma in a child with cardiac involvement. A 4-year-old boy presented to the Vietnam National Children's Hospital with a vague headache, but magnetic resonance imaging of the head was normal. After 1 week, the patient showed symptoms of chest pain, fatigue, dyspnea, and abdominal pain. On transthoracic echocardiography and multislice computed tomography of the thorax, a mass was detected in the right atrial wall. Abdominal ultrasound showed a small bowel intussusception, multiple nodules in the intestinal wall, and mesenteric lymph nodes. Histopathology of the bowel confirmed the diagnosis of multifocal precursor B-cell lymphoblastic lymphoma. The patient responded to 3 cycles of chemotherapy for lymphoma. Therefore, combining multiple imaging methods allowed for early diagnosis and improved treatment.

COVID-19 infection and cardiac angiosarcoma: a dangerous combination-a case report.

Background: The COVID-19 pandemic has strained all medical systems, especially in countries like Ecuador, where health services were already limited. These conditions, combined with a deadly and unusual disease, like primary heart angiosarcoma, can lead to severe outcomes. Angiosarcomas represent the most common and aggressive primary malignant heart tumor; regretfully, its clinical manifestations are vague and can be easily missed. Most patients become symptomatic when there is local invasion, embolization, or metastases, leading to late diagnosis and poor survival. High clinical awareness, adequate diagnosis, and prompt treatment are vital in these rare diseases, in which time is of paramount importance. Case presentation: We report the case of a 28-year-old female who had cough, hemoptysis, and ground-glass opacities in the CT (computed tomography). Since Ecuador is in the middle of this pandemic, she was misdiagnosed and mistreated. Primary heart angiosarcoma was diagnosed, and regretfully, the patient suffered multiple complications due to diagnosis and died. Conclusion: To this day, most cardiac angiosarcomas are found in a late-stage with distal metastasis and advanced local invasion. Sadly, this tumor is frequently missed due to its incidence and broad-spectrum of clinical symptoms. Considering that its manifestations can be misleading, misdiagnosis can occur, especially in pandemic times. Therefore, knowledge of other pathologies prevents COVID-19 from overshadowing other diagnoses, hence preventing delayed diagnosis or even misdiagnosis and consequent adverse outcomes for patients. Supplementary Information: The online version contains supplementary material available at 10.1186/s43057-021-00042-7.

Supradesnivel del segmento ST como forma de presentación atípica de un tumor cardíaco: rol diagnóstico de la multimodalidad de imagen / ST segment elevation as an atypical presentation of a cardiac tumor: diagnostic role of a multimodal imaging approach / Elevação do segmento ST como apresentação de tumor cardíaco: papel diagnóstico da multimodalidade de imagem

Los tumores cardíacos malignos son neoplasias poco frecuentes que pueden presentarse de diversas formas, lo que dificulta su diagnóstico. La ecocardiografía y la resonancia magnética cardíaca son técnicas fundamentales para el diagnóstico, la caracterización y la evaluación de su extensión tumoral. La identificación de la línea tumoral es esencial al iniciar un tratamiento oncológico dirigido. Si bien el "estándar de oro" para este fin es el estudio anatomopatológico (obtenido por biopsia o resección quirúrgica), en los casos en que esto no es posible, la resonancia magnética cardíaca es la técnica no invasiva que proporciona un mejor abordaje diagnóstico. El tratamiento de elección es la resección quirúrgica y el pronóstico, en general, es malo. Presentamos el caso de una paciente con un tumor cardíaco de presentación clínica atípica, en la que un abordaje imagenológico multimodal aporta información clave y complementaria para el diagnóstico y la caracterización tisular.

Malignant cardiac tumors are rare neoplasms that can present in various forms, making their diagnosis difficult. Echocardiography and cardiac magnetic resonance imaging are fundamental techniques for the diagnosis, characterization, and evaluation of tumor extension. Identification of the tumor line is essential when initiating targeted cancer therapy. Although the "gold standard" for this purpose is the pathological study (obtained by biopsy or surgical resection), in cases where this is not possible, cardiac resonance is the non-invasive technique that provides a better diagnostic approach. The treatment of choice is surgical resection and the prognosis is generally poor. We present the case of a patient with an atypical clinical presentation, in which a multimodal approach provides key and complementary information for tumor diagnosis and tissue characterization.

Os tumores cardíacos malignos são neoplasias raras que podem se apresentar de várias formas, dificultando seu diagnóstico. A ecocardiografia e a ressonância magnética cardíaca são técnicas fundamentais para o diagnóstico, caracterização e avaliação da extensão tumoral. A identificação da linha do tumor é essencial ao iniciar a terapia direcionada do câncer. Embora o "padrão ouro" para esse fim seja o estudo patológico (obtido por biópsia ou ressecção cirúrgica), nos casos em que isso não seja possível, a ressonância cardíaca é a técnica não invasiva que proporciona melhor abordagem diagnóstica. O tratamento de escolha é a ressecção cirúrgica e o pronóstico geralmente é ruim. Apresentamos o caso de uma paciente com apresentação clínica atípica, em que a abordagem multimodal fornece informações essenciais e complementares para o diagnóstico do tumor e caracterização do tecido.

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.

Giant Right Coronary Artery Aneurysm Resembling Cardiac Tumor: A Case Report.

BACKGROUND: Coronary artery aneurysms are not uncommon vascular anomalies, but giant coronary artery aneurysms mimicking cardiac tumors are extremely rare and easily misdiagnosed as tumors. The management and prognosis are not well defined. CASE REPORT: We describe a case of a 50-year-old woman, who had a transthoracic echocardiogram that suggested a cardiac tumor in the right ventricle adjacent to the tricuspid valve and without ventricular wall akinesia. Her medical history was unremarkable, and she had no obvious symptoms. A transesophageal echocardiogram and computed tomography angiogram examination suggested the tumor-like structure was a giant coronary aneurysm, originating from the distal portion of right coronary artery. The patient underwent surgical intervention and recovered uneventfully. Histopathology of the excised aneurysm showed fibrous tissue hyperplasia with collagenization and calcification in the wall of the artery. CONCLUSION: Giant coronary artery aneurysms resembling heart tumors are extremely rare. Differentiating them from cardiac tumors can sometimes be difficult. This rare case emphasizes the importance of combining multiple imaging methods, which helps make accurate diagnosis. For treatment, surgical intervention may be the appropriate therapeutic strategy for the rare lesion.