Cardiac tumours in children

Abstract

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor.

Febrile Illness in an Infant With an Intracardiac Inflammatory Myofibroblastic Tumor

“…These are differentiated from IMTs based on histology and extent of invasion into the myocardium. 9,10 IMTs appear histologically similar to myosarcomas but can be differentiated on the basis of their having less cellular pleomorphism, atypia, and fewer mitotic figures 1,10 and not invading into the myocardium. ALK-1 is positive in 35% of IMTs, 11 although a negative result does not rule out the diagnosis because several intracardiac IMTs have previously stained negative.…”

Klinikinė Perinatalinio Periodo Širdies Rabdomiomų Patirtis Vilniaus Perinatologijos Centre

“…TSK1 ir TSK2 genų mutacijos lemia ląstelėse esančio baltymo mTOR ("mammalian target of rapamycin") aktyvumo slopinimą ir gerybinių navikų (hamartomų) atsiradimą įvairiose organų sistemose. Histologinių tyrimų metu nustatyta, kad širdies rabdomiomą sudaro patognomoninės "voro tinklo" pavidalo ląstelės su citoplazma ir branduoliu centre, su link ląstelės sienelės išsidės-čiusiomis miofibrilėmis [12]. Priklausomai nuo rabdomiomų dydžio, skaičiaus ir lokalizacijos, tumorai gali trukdyti kraujui nutekėti iš kairiojo arba dešiniojo širdies skilvelio, trikdyti miokardo kontrakciją bei atsipalaidavimą ir sukelti širdies aritmijas (ekstrasistoliją, pirmalaikį sujaudinimą ar supraventrikulinę tachikardiją) [13].…”

Surgical treatment of cardiac tumors - an overview and presentation of interesting cases

“…Patients with fibroma may become symptomatic with heart failure, ventricular arrhythmias and sudden death, although one third of the patients remain asymptomatic and tumour is detected incidentally [12,28]. In symptomatic cases, the tumour can be enucleated from the ventricular wall, when the tumour is very large, and complete resection seems impossible, partial resection may result in good palliation [25,29,30]. Exceptionally, cardiac transplantation may be required when large and extensive tumour involvement precludes complete excision [12,29].…”

“…Rhabdomyomas are located more often in the ventricle with both sides equally involved rather than in the atrial wall. They can be small or protrude largely into the cardiac cavities [12,25,26]. Whether rhabdomyomas refractory rhythm disturbances) are those who require surgical excision ( fig.…”