Identification and clinical course of 166 pediatric cardiac tumors

Abstract

The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.

A large ventricular fibroma requiring surgical resection in a symptomatic 3-month-old infant

“…Fibromas are usually isolated single tumours, most commonly located in the free ventricular wall or the inter-ventricular septum. 6 Architecturally, the fibroblasts and collagen matrices that make up fibromas as seen in our patient's specimen are similar to that of purkinje cells 7 (Fig 1d). This is most evident in that patients with cardiac fibromas are pre-disposed to arrhythmias at a higher rate compared to other cardiac tumours.…”

An unusual cause of shortness of breath and palpitations

“…Seventy five percent of all primary cardiac tumours are benign, and other types include papillary fibroelastoma, fibroma, lipoma, and rhabdomyoma 2. The diagnosis is confirmed by histological examination.…”

Everolimus for cardiac rhabdomyomas in children with tuberous sclerosis. The ORACLE study protocol (everOlimus for caRdiac rhAbdomyomas in tuberous sCLErosis): a randomised, multicentre, placebo-controlled, double-blind phase II trial

“…5,7 Currently, there is no standard pharmacological treatment for this disease and in severe cases, surgery is indicated but with a high risk of mortality. 6,27 Everolimus is an mTOR inhibitor and has been proved effective in reducing astrocytomas associated with TSC. 28 The mechanism of the disease is the same in both pathologies (astrocytoma and cardiac rhabdomyoma), so the benefit would be similar to astrocytomas.…”

“…This system will provide allocation concealment and assure blinding to the patient and investigator in the centre. 27 Regression of neonatal cardiac rhabdomyoma in 2 months through low-dose everolimus therapy: a report of three cases 3 0.3-0.67 mg/m 2 /day Implementation Enrolment of each patient will start by the signing of the informed consent by the parents and the consent assessment of the child if older than 8 years. This will be followed by incorporation of the patient to the database and the randomisation process.…”