Cardiac Tumors Research Articles

Multiple Cardiac Tumors in an infant: A Tumorous Heart.

Multiple Cardiac Tumors in an infant: A Tumorous Heart.

Clinical features and surgical treatment of right cardiac system tumors

A total of 25 patients with right cardiac system tumors in the Department of Cardiac Surgery, Beijing Anzhen Hospital from January 2012 to October 2022 were retrospectively included in the study. The preoperative data, and information of surgical treatment and perioperative management on these patients were analyzed and summarized. One patient developed pulmonary embolism and died before surgery, and the other 24 patients (16 males and 8 females) received surgical treatment, with an average age of (44.7±10.2) years (24-74 years). Nine patients were diagnosed with malignant tumors. Among the 24 patients who received surgical treatment, two patients died during the perioperative period, in-situ tumor recurrence was seen in three patients within about 1 year after surgery (two patients died without surgery, and one patient died 3 months after surgery), two patients had distant metastasis, and 17 patients had a good prognosis. Right cardiac system tumors are rare, with a high malignant rate, and the clinical manifestations vary greatly. Active surgical intervention is found to be effective, and the prognosis is closely related to the pathological type and extent of tumor invasion.

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.

Clinical Features of Cardiac Calcified Amorphous Tumor: A Narrative Review.

A cardiac calcified amorphous tumor (CAT) is a non-neoplastic cardiac mass composed of calcified nodules surrounded by amorphous fibrous tissue in a context of degeneration and chronic inflammation. Although CAT cases are increasingly reported, its clinical manifestation remains unclear. We aimed to conduct a narrative review of CAT and identify its clinical characteristics. We conducted a comprehensive literature search using PubMed, with the keyword "Cardiac Calcified Amorphous Tumor" to identify relevant articles. A total of 113 articles published between 1997 and 2022 were retrieved. The clinical features allowing for assess patient background, differences with and without end-stage renal disease (ESRD), symptom-related factors, risk factors for embolism caused by CAT, and features of CAT, such as shape, location, mobility, pathology, and treatment, were statistically analyzed in 106 criteria-matched cases. The mean patient age was 60.2 ± 18.6 years, with 45 men and 61 women. Of the data collected from 21 countries, 52 patients were from Japan. Mobile CAT was more common in the chronic renal failure group. Linear, club-shaped, and spindle-shaped CAT tended to be mobile and more common in the ESRD group as well as located in the mitral valve region. The shape, mobility, location, and underlying mechanism of CAT depended on the presence or absence of ESRD. The risk of embolism was considered high because mobile CAT was more common in the ESRD group, and CAT originated in the mitral valve region. Hence, early diagnosis based on periodic examination in patients with ESRD and aggressive surgical treatment are necessary.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Lipomatous Interatrial Septum Detected During Cardioversion Evaluation for Atrial Fibrillation

Primary cardiac tumors are rare, with an estimated prevalence in autopsy series ranging from 0,001 to 0,28%. Benign tumors are the most common type, with lipoma being the second most frequent among these, following myxoma. Lipoma can pose a differential diagnostic problem with lipomatous hypertrophy of the interatrial septum (IAS). This entity is rare and often asymptomatic, requiring no surgical treatment unless it causes obstruction of surrounding anatomical structures. In practice, cardiac CT and MRI are frequently used to confirm echocardiographic suspicions, both transthoracic and transesophageal. We present the case of a 72-year-old patient referred for management of recent atrial fibrillation, with heart rate control proving difficult despite maximum dose medical treatment. We opted for electrical cardioversion to improve the patient's hemodynamic state in the short term and to prevent progression to atrial cardiomyopathy in the medium and long term. In the transesophageal echocardiogram (TEE), the patient exhibited significant thickening of the interatrial septum, measuring 22 mm, without compression of the superior vena cava, and with the fossa ovalis remaining intact. Through this case, we discuss the diagnostic and therapeutic approaches to lipomatous hypertrophy of the IAS.

Atrial Tachycardia Revealing Obstructive Left Atrial Myxoma. Case Report

Myxomas are the most common primary cardiac tumors; the most common localization is the left atrium, and may cause mitral flow obstruction so their clinical presentation could mimic severe mitral stenosis; trans thoracic echocardiography allows the diagnosis; we report a case of left atrial myxoma revealed by atrial tachycardia concomitant with dyspnea and signs of chronic right heart failure; despite surgical resection of the tumor, our patient had developed tachycardiomyopathy with severe left ventricular dysfunction,  which recovered two months later, after intensive administration of antiarrhythmic treatment.

Primary Cardiac Intimal Sarcoma: Multi-Layered Strategy and Core Role of MDM2 Amplification/Co-Amplification and MDM2 Immunostaining.

Primary cardiac tumours are relatively uncommon (75% are benign). Across the other 25%, representing malignant neoplasia, sarcomas account for 75-95%, and primary cardiac intimal sarcoma (PCIS) is one of the rarest findings. We aimed to present a comprehensive review and practical considerations from a multidisciplinary perspective with regard to the most recent published data in the specific domain of PCIS. We covered the issues of awareness amid daily practice clinical presentation to ultra-qualified management in order to achieve an adequate diagnosis and prompt intervention, also emphasizing the core role of MDM2 immunostaining and MDM2 genetic analysis. An additional base for practical points was provided by a novel on-point clinical vignette with MDM2-positive status. According to our methods (PubMed database search of full-length, English publications from January 2021 to March 2023), we identified three studies and 23 single case reports represented by 22 adults (male-to-female ratio of 1.2; male population with an average age of 53.75 years, range: 35-81; woman mean age of 55.5 years, range: 34-70) and a 4-year-old child. The tumour-related clinical picture was recognized in a matter of one day to ten months on first admission. These non-specific data (with a very low index of suspicion) included heart failure at least NYHA class II, mitral regurgitation and pulmonary hypertension, acute myocardial infarction, ischemic stroke, obstructive shock, and paroxysmal atrial fibrillation. Awareness might come from other complaints such as (most common) dyspnoea, palpitation, chest pressure, cough, asthenia, sudden fatigue, weakness, malaise, anorexia, weight loss, headache, hyperhidrosis, night sweats, and epigastric pain. Two individuals were initially misdiagnosed as having endocarditis. A history of prior treated non-cardiac malignancy was registered in 3/23 subjects. Distant metastasis as the first step of detection (n = 2/23; specifically, brain and intestinal) or during follow-up (n = 6/23; namely, intestinal, brain and bone, in two cases for each, and adrenal) required additional imagery tools (26% of the patients had distant metastasis). Transoesophageal echocardiography, computed tomography (CT), magnetic resonance imagery, and even 18F-FDG positronic emission tomography-CT (which shows hypermetabolic lesions in PCIS) represent the basis of multimodal tools of investigation. Tumour size varied from 3 cm to ≥9 cm (average largest diameter of 5.5 cm). The most frequent sites were the left atrium followed by the right ventricle and the right atrium. Post-operatory histological confirmation was provided in 20/23 cases and, upon tumour biopsy, in 3/23 of them. The post-surgery maximum free-disease interval was 8 years, the fatal outcome was at the earliest two weeks since initial admission. MDM2 analysis was provided in 7/23 subjects in terms of MDM2-positive status (two out of three subjects) at immunohistochemistry and MDM2 amplification (four out of five subjects) at genetic analysis. Additionally, another three studies addressed PCISs, and two of them offered specific MDM2/MDM2 assays (n = 35 patients with PCISs); among the provided data, we mention that one cohort (n = 20) identified a rate of 55% with regard to MDM2 amplification in intimal sarcomas, and this correlated with a myxoid pattern; another cohort (n = 15) showed that MDM2-positive had a better prognostic than MDM2-negative immunostaining. To summarize, MDM2 amplification and co-amplification, for example, with MDM4, CDK4, HMGA3, CCND3, PDGFRA, TERT, KIT, CCND3, and HDAC9, might improve the diagnosis of PCIS in addition to MDM2 immunostaining since 10-20% of these tumours are MDM2-negative. Further studies are necessary to highlight MDM2 applicability as a prognostic factor and as an element to be taken into account amid multi-layered management in an otherwise very aggressive malignancy.

Safety and outcomes of surgical treatment of atrial fibrillation in emergency surgery cases.

Recent developments in surgical devices, including left atrial appendage closure, have enabled surgeons to perform aggressive operations for atrial fibrillation (AF). However, the outcomes of AF surgery in emergent cases have not been extensively studied. The present study aimed to investigate the effectiveness of AF surgery in emergency surgery cases associated with cardiovascular events. We enrolled 18 patients who underwent various types of AF surgery due to emergencies, including acute aortic dissection (n = 6), acute myocardial infarction (n = 5), bleeding due to perforation from radiofrequency catheter ablation (n = 4), acute mitral regurgitation (n = 2), and cardiac tumor (n = 1). Four and ten patients underwent the full maze procedure and pulmonary vein isolation, respectively. Ganglionated plexi ablation was also performed in three patients as part of a combined procedure. The left atrial appendage was solely closed in four patients. There was no surgical mortality or major adverse cardiac and cerebrovascular events in our patient series. The rates of freedom of recurrence of AF or atrial tachycardia at 1 and 3years were 92.9% and 82.5%, respectively. After a mean follow-up period of 46.7 ± 25.8months, no thromboembolism events were observed in the patients. Furthermore, no cardiovascular death was recorded. The surgical procedures for AF are safe and effective in cases requiring emergency surgery.

Echocardiography-Guided Radiofrequency Ablation for Cardiac Tumors

Echocardiography-Guided Radiofrequency Ablation for Cardiac Tumors

Endomyocardial Biopsy of Heart Tumors under Cone-Beam Computed Tomography Control

Tumors of the heart are rare entities characterized by the absence of specific signs and symptoms. A biopsy is necessary for verify the diagnosis and determine the most correct treatment tactics. Endomyocardial biopsy (EMB) is a modern and relatively safe approach for biopsy of cardiac tumors. Usually this procedure performs under the control of fluoroscopy and echocardiography (intracardiac, transthoracic, transesophageal). However, this procedure is rarely used in patients with suspected cardiac malignancy.We present two clinical cases of endomyocardial biopsy of cardiac tumors by the endovascular approach under the control of cone-beam computed tomography (CBCT). The biopsy was successful in both cases and pathological diagnosis was established.

Giant Left Atrial Myxoma Revealed by Neurological Manifestation in a Young Female Patient

Cardiac myxoma is a prevalent benign neoplasm that arises inside the cardiac tissue and exhibits a gradual growth pattern. The annual incidence rate of cardiac myxoma is approximately 0.5–1 cases per 1,000,000 persons. The fragmentation of a cardiac tumor may be influenced by cerebrovascular events. Ischemic stroke is a relatively infrequent occurrence in pediatric populations and can occasionally be attributed to cardiac myxoma. In instances of ischemic stroke, neurological impairments are observed as a result of emboli or thrombi originating from the myxoma. Echocardiography is a valuable tool for promptly diagnosing myxoma and facilitating immediate surgical removal of the myxoma. We report a 17 years old female with neurological deficit weakness in the upper and lower right extremities since 2 months ago as the chief complaint with intermittent shortness of breath. Haemodynamic parameters was stable. The patient underwent brain imaging with result a lesion of the left lentiform nucleus, transthoracic echocardiography and trans esophageal echocardiography with oscillating septated mass on left atrial, attached to the anterior mitral leaflet (AML). Primary cardiac tumors are a very uncommon cause of cardiac embolic events. An atrial myxoma, a benign growth, can form on either the left or right side of the upper part of the heart. Untreated atrial myxoma can lead to adverse effects such as arrhythmia, pulmonary edema, and emboli formation that obstruct blood flow in the heart's veins. Ischemic stroke causes a specific functional impairment. Neurological symptoms in stroke can be an extra cardiac manifestation of atrial myxoma. The use of transthoracic and tran seso phageal is important in diagnosing atrial myxoma.

Rapidly growing right atrial myxoma in an older patient with advanced chronic kidney disease: a case report

BackgroundRight atrial myxoma is a rare cardiac tumor, with few reports on its growth rate.Case presentationWe found a right atrial myxoma in a male patient in his 80s with chronic kidney disease on the second echocardiogram that was performed for the creation of an arteriovenous fistula at 9 months after the initial echocardiogram, which showed no evidence of myxoma. Echocardiography revealed a mass measuring 37 mm × 28 mm in the right atrium. The patient was asymptomatic, and the tumor was surgically removed.ConclusionsTo the best of our knowledge, our patient is the oldest compared with all the patients in previously reported cases of rapidly growing right atrial tumors.

Abstract 1447: Development of advanced preclinical modeling for a comprehensive characterization of cardiac angiosarcoma biology

Abstract Cardiac angiosarcoma (CAS) is an extremely rare malignant cardiac tumor known for its propensity for early metastasis and poor prognosis. CAS frequency is around 1-2% of all angiosarcoma cases and mostly arise in the right atrium. At present, there are no effective treatments and the survival rate is less than 10% at 5 years. Furthermore, the dearth of preclinical models has limited the advancement of biological insights into CAS. To address this challenge, we developed a somatic mosaic genetically engineered mouse model (smGEMM) with the aim of accurately mirroring the biological features of CAS. This model will be a crucial instrument for acquiring in-depth insights into the disease and steering the development of impactful therapeutic strategies. To develop a CAS smGEMM, we leveraged an endothelial-specific CRE recombinase to activate a fluorescent reporter in a tissue-dependent manner and CRISPR/Cas9 technology to engineer somatic drivers of CAS tumorigenesis. Using this strategy, all endothelial cells express tdTomato fluorescent reporter and spCas9. We then administered adeno-associated viral (AAV) particles carrying selected tandem sgRNAs with two different approaches: a direct approach where AAV particles were administered by ultrasound-guided injection in the cardiac wall of the smGEMM; and an ex-vivo approach where primary endotheliocytes derived from cardiac tissue of our smGEMM were first transduced with AAV particles in vitro, and then transplanted orthotopically by ultrasound-guided injection. With the latter strategy, we successfully generated the first cardiac angiosarcoma model that faithfully recapitulates angiosarcoma features, including lung dissemination. Cell lines from primary and metastatic tumors were also established, characterized and utilized for genome-wide CRISPR Cas9 screening. Current efforts are focused on prioritizing hits for validation, aiming to glean insights that can inform potential therapeutic strategies. Citation Format: Rosalba Minelli, Luigi Perelli, Benjamin J. Bivona, Sanjana Srinivasan, Desiree Catania, Bianca E. Amador, Frederick S. Robinson, Johnathon L. Rose, Christopher T. Terranova, Alejandro Hernandez Martinez, Charles E. Deckard, Courtney N. Le, Kuo-Shun Hsu, Michael Peoples, Aparna Subramaniam, Hannah C. Beird, Ningping Feng, Joseph R. Daniele, Christopher Vellano, Joseph Marszalek, Vinod Ravi, Alexander J. Lazar, Andrew Futreal, Timothy P. Heffernan, Giannicola Genovese, Virginia Giuliani. Development of advanced preclinical modeling for a comprehensive characterization of cardiac angiosarcoma biology [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 1447.

ECG Changes in a Young Lady Candidate for In Vitro Fertilization Due to Left Ventricular Hemangioma

: Cardiac hemangioma is a rare cardiac tumor which may have a spectrum of signs and symptoms; here we would like to present a case of asymptomatic cardiac hemangioma that was discovered by an ECG.

Perinatal Results and Long-Term Follow-Up of Fetal Cardiac Tumors: A 30-Year Historical Cohort Study.

This was a 30-year retrospective cohort study that approximates closely to the natural history of cardiac tumors diagnosed in the fetus, since there was no case of pregnancy interruption. To assess morbidity and mortality in the perinatal period and at long term in fetuses diagnosed with cardiac tumor. Our secondary objective was to assess the evaluating factors of perinatal and postnatal results. This was a retrospective cohort study with 74 pregnant women with an echocardiographic diagnosis of fetal cardiac tumor at two referral centers between May 1991 and November 2021. A descriptive analysis was performed, and data were expressed as absolute (n) and relative (%) frequencies, median and interquartile range. Fisher's exact test was used to evaluate the association of echocardiographic characteristics and clinical manifestations with perinatal and postnatal results. Global survival was calculated using the Kaplan-Meier method and the curves were compared by the log-rank test. The time of follow-up, calculated in months, corresponded to the time elapsed from hospital discharge to current status (survived/ censoring or death). The level of significance was set at 5% (p<0.05). Rhabdomyoma is the most common type of cardiac tumor (85%), with a high morbidity (79.3%) and overall mortality of 17.4%. The presence of fetal hydrops was a predictor of death. The presence of fetal hydrops had an impact on mortality, and hence is an important factor in counselling and determining the prognosis. Most deaths occurred before hospital discharge.

Application of Three-Dimensional Printing Technology in the Perioperative Management of Cardiac Tumours: A Review and Analysis

Application of Three-Dimensional Printing Technology in the Perioperative Management of Cardiac Tumours: A Review and Analysis

Left atrial myxoma with cardiogenic shock following a myocardial infarction: a case report

BackgroundMyxomas are the most common primary cardiac tumor and typically originate in the left atrium. Atrial myxomas may present following complications of obstruction and emboli. If an atrial myxoma goes untreated, complications such as congestive heart failure, embolic stroke, and sudden death can occur.Case presentationA 58-year-old Caucasian male presented following a cardiac arrest. He was taken emergently to the cardiac catheterization lab and received two drug eluting stents. Following the procedure, he was found to have a left atrial mass that was intermittently obstructing the mitral valve on echocardiography. After leaving the cardiac catheterization lab, he was hypotensive and placed on multiple intravenous medications for hemodynamic support as well as an Impella device. Following medical optimization, he underwent one vessel coronary artery bypass graft as well as surgical excision of the left atrial mass, which pathology had shown to be an atrial myxoma.ConclusionThis patient’s case of cardiogenic shock following revascularization was complicated by the identification of an atrial myxoma, which, when large enough, can obstruct blood flow through the mitral valve leading to acute mitral dynamic stenosis. This condition results in circulatory collapse due to obstruction of the left ventricle in diastole as the myxoma occludes the mitral valve.

Ten-year survival after resection of a huge rhabdomyosarcoma in the pericardial cavity

BackgroundPrimary malignant cardiac tumors are exceedingly rare and cardiac rhabdomyosarcoma among them is an exceptional rarity characterized by a dismal poor prognosis.Case presentationA 48-year-old man had suffered from a persistent cough lasting for more than 6 months and computed tomography showed a huge mass in the pericardial cavity with heterogeneous content. Following referral to our department for suspected cardiac malignancy, cardiac magnetic resonance imaging revealed a lucent layer on the boundary around the mass, suggesting the feasibility of surgical resection. A baby-head-sized mass in the pericardial cavity was entirely resected and the pathological examination confirmed a tumor as a spindle cell type rhabdomyosarcoma. The patient underwent adjuvant chemotherapy and has well survived over a decade after surgery without any sign of recurrence.ConclusionRadical resection with adjuvant chemotherapy may achieve a favorable outcome for patients with massive rhabdomyosarcomas inside the pericardial cavity.

Suction thrombectomy of a uterine carcinosarcoma tumor thrombus extending into the IVC and right atrium.

Uterine carcinosarcoma is a rare, aggressive tumor with several cases in the literature reporting cardiac tumor thrombus involvement. In this case report, we describe a 72-year-old female with a history of uterine carcinosarcoma, who presented with extensive thrombus in the Inferior Vena Cava (IVC) and right atrium. The patient underwent an aspiration thrombectomy which aided in intravascular debulking of the thrombus. Histopathological analysis of the thrombus revealed tumor thrombus. In cryptic cases of tumor thrombus, thrombectomy with histopathological analysis can help confirm the diagnosis of metastatic disease and help guide oncologic staging and further therapy.