tumors

TUMORS

Primary Benign Tumors

• Myxoma

  • Most common type of primary benign cardiac tumor in adults.

  • Rarity of cardiac tumors is noted.

  • Characteristics:

  • Primarily idiopathic; 10% genetic.

  • Usually solitary, predominantly found in females.

  • Pedunculated and typically attached to the interatrial septum (IAS).

  • 75% are located in the left atrium.

  • Myxomas have different texture compared to myocardium.

  • Risk of embolus is elevated.

  • Myxomas hinder diastolic filling and inhibit proper closure of the valve, leading to regurgitation. If mobile, they can also cause stenosis.

  • Size estimation can be done via calipers or planimetry.

  • There may be a familial link.

Signs, Symptoms, and Complications of Myxoma

• Myxomas can clinically present as:

  • Asymptomatic in certain cases.

  • Arrhythmia.

  • Arthralgia, specifically joint pain, along with clubbing of fingertips.

  • Other symptoms include chest pain, cough, dizziness, fainting, and orthopnea.

  • Embolism is a significant concern.

  • Patients may exhibit fever, flu-like symptoms, and rash.

  • Potential for heart failure.

  • Secondary stenosis and regurgitation findings.

  • Hemolytic anemia may occur.

  • Infected myxoma can be associated with additional findings, such as Raynaud's phenomenon.

Treatment of Myxoma

• If the patient qualifies as a surgical candidate, immediate surgical removal is advised.

• Complete removal of the myxoma is essential to decrease recurrence risk.

• Follow-up studies are critical to rule out recurrence.

Papillary Fibroelastoma (Papilloma)

• This is noted as the most common benign valvular tumor.

• Common locations in adults include:

  • Aortic valve.

  • Left Ventricular Outflow Tract (LVOT).

  • Anterior Mitral Valve leaflet.

• Formation:

  • Arise from thrombus attaching and growing into a dense, mobile mass.

  • Rarely exceeds 1 cm.

  • Consistency resembles chordae tendineae.

• Complications:

  • Highly mobile hence presents life-threatening complications potentially leading to embolism, stroke, valve dysfunction, arrhythmia, or sudden death.

• Treatment:

  • Surgical excision.

  • Options for valve sparing or replacement.

Lipoma

• Characteristics:

  • Encapsulated tumor formed from mature fat cells, soft in texture, may become large.

  • Typically asymptomatic, often found incidentally.

• Imaging findings:

  • Appears as an echogenic mass due to high fat content on echo.

• Seen in lipomatous hypertrophy, sparing the fossa ovalis, usually in elderly and obese patients.

• Management:

  • Rarely requires treatment; surgical removal is indicated if symptomatic.

Fibroma

• Characteristics:

  • Bulky tumor embedded in the myocardial wall, may be found in ventricles or interventricular septum (IVS).

  • Typically presents during childhood.

• Concerns:

  • Associated with ventricular arrhythmia, left ventricular outflow tract (LVOT) obstruction, heart failure, and risk of sudden death.

• Treatment options:

  • Surgical removal.

  • Heart transplant in severe cases.

Rhabdomyoma

• Characteristics:

  • Presents as a yellowish-gray tumor, multiple tumors may be present throughout the heart, particularly in the LV walls and cavity.

• Commonly diagnosed in infants, specifically within the first year of life, and 90% are diagnosed before the age of 15.

• Association:

  • Primarily associated with tuberous sclerosis, a genetic disorder.

• Symptoms:

  • Majority are asymptomatic; however, some may experience arrhythmia and LVOT obstruction.

• Management:

  • Surgical removal may be considered, but in some cases, removal may be impossible.

Primary Malignant Tumors: Sarcoma & Angiosarcoma

• Overview:

  • Primary malignant tumors of the heart are rare.

  • Most cardiac malignancies are sarcomas, with angiosarcoma being the most common subtype.

  • Usually originating in the right atrium or pericardium.

• Symptoms vary based on location and cancer grade:

  • Right Atrial Angiosarcoma can lead to obstruction of inflow and outflow, causing backup of venous return, resulting in swelling of feet, legs, abdomen, and neck veins.

  • Pericardial Angiosarcoma may cause effusion/tamponade and associated findings.

  • Symptoms include embolus risks leading to stroke, obstruction of blood flow, respiratory distress, as well as arrhythmia, hemoptysis, fever, weight loss, and fatigue.

• Treatment options:

  • Significant metastasis risk (80% metastasize), treatment approach is contingent upon age, cancer grade, metastasis, location, and tumor size.

  • Surgical removal, radiation, chemotherapy.

  • Heart transplant and auto heart transplant are also considered.

Secondary Malignant Tumors: Metastatic

• Overview:

  • Secondary (metastatic) tumors are more frequently observed than primary malignant tumors.

  • Typically associated with the terminal phase of ongoing diseases.

  • Common sources include lung, breast, renal carcinomas, or malignant melanoma.

• Pathways to reach the heart include:

  • Lymphatic system, thymoma, esophageal carcinoma.

  • Pulmonary veins leading to the left atrium from the lungs.

  • Pericardium passing into myocardium and endocardium.

  • Inferior Vena Cava (IVC) to the right heart, originating from renal, adrenal, liver, or uterine carcinoma.

• Patients may present with:

  • Pericardial effusion, tamponade, heart failure, arrhythmia.

• Management approach depends on the primary malignancy; often focuses on palliative care because of poor prognosis.

Carcinoid Heart Disease

• Source:

  • Carcinoid tumors typically arise from the ileum or appendix and secrete serotonin.

  • These tumors metastasize to the liver and deposit in the endocardial lining of the right heart, but do not affect the left heart because serotonin is inactivated in the lung.

• Echocardiographic findings:

  • Fixed, rigid tricuspid valve (TV), which does not open or close effectively.

  • Severe tricuspid regurgitation (TR) and pulmonary regurgitation (PR).

  • Possible right heart failure and rarely tricuspid stenosis (TS) or pulmonary stenosis (PS).

Extracardiac Tumors

• Definition:

  • Extracardiac tumors are located near the heart and can include:

  • Mediastinal cysts.

  • Hematomas.

  • Thymomas.

  • Teratomas.

  • Infradiaphragmatic tumors.

  • Pancreatic cysts.

  • Pleural tumors.

• Note: Pericardial involvement is common in these cases.

THROMBUS

• Formation and Characteristics:

  • Thrombus forms in areas with akinesias or dyskinesis, commonly associated with anterior myocardial infarction (MI), typically found in the apex.

  • Need to thoroughly document wall motion abnormalities (WMA), chamber size, and overall function.

• Thrombus Types:

  • Layered, single, multilobulated, and pedunculated thrombus.

• Assessment:

  • To interrogate the apex, improve resolution using high-frequency transducer and focus on the apex.

  • Utilize Color flow Doppler to check if it fills the apex. It’s essential to assess at varying depths to rule out artifacts.

  • Ensure documentation occurs in at least two different views.

  • Transesophageal echocardiography (TEE) may be indicated as necessary.

• Left Atrial Thrombus:

  • Associated with mitral stenosis (MS), left atrial enlargement, and atrial fibrillation (A-Fib).

  • Important to rule out left atrial or left atrial appendage thrombus before cardioversion.

  • TEE may be indicated to assess risk of embolization.

MISSILES

• Definition:

  • Refers to foreign bodies such as bullets, knives, nails, etc.

• Imaging Findings:

  • On echocardiography, appears as echogenic structures with strong reverberation effects.

• Assessment of Complications:

  • Rule out potential complications like pericardial effusion/tamponade, coronary artery trauma, myocardial rupture, atrial septal defects (ASD), ventricular septal defects (VSD), wall motion abnormalities, and valvular rupture leading to regurgitation.