Hastanemizde Primer Kardiyak Tümör Tanısı ile Takipli 0-18 Yaş Arası Hastaların 5 Yıllık Verilerinin Değerlendirilmesi

Abstract

Yıldırım, S., Evaluation of 5-year data of patients between 0-18 years old with the diagnosis of primary cardiac tumor in our hospital, Hacettepe University Faculty of Medicine, Pediatrics Specialist Thesis, Ankara, 2020. Primary cardiac tumors are rare in childhood. Rhabdomyoma is the most common primary cardiac tumor; coexistence of it with tuberous sclerosis is common. Everolimus can be used in complicated cases, although it may regress spontaneously. While teratoma and fibroma are other benign tumors, angiosarcoma is the most common malignant tumor. In our study, 24 (43.6%) girls, 31 (56.4%) boys with a diagnosis of primary cardiac tumors in our hospital, 47 (85.5%) of the total 55 patients were rhabdomyoma, 3 (5.5 %) were teratoma, 2 (3.6%) were fibroma, 1 (1.8%) was papillary fibroelastoma, 1 (1.8%) was angiosarcoma and 1 (1.8%) was primary yolk sac tumor. Thirty-one (56.4%) of the patients had intrauterine diagnosis. It was seen that 80% of patients with rhabdomyomas were diagnosed under the age of 1, 24 (51%) had multiple rhabdomyomas, 33 (70.2%) were accompanied by tuberous sclerosis, the most common cause of admission to the hospital was intrauterine cardiac mass, 33 (70%) patients were asymptomatic, the most common symptom was murmur, 16 (34%) had a spontaneous regression in their masses, 14 patients underwent everolimus treatment, and 11 patients had treatment responses. Six of 8 tumors other than rhabdomyoma were symptomatic, 5 patients underwent surgery, 3 patients underwent both medical and surgical treatment. The most common complication was arrhythmia. Only the patient with angiosarcoma died. Currently, 31 (56.3%) patients continue their cardiology outpatient follow-up. Our outpatient 5-year primary cardiac tumor incidence was calculated as 0.086%. Our study has shown that with the development of technology the rate of diagnosis of primary cardiac tumors has increased during intrauterine life and when they are asymptomatic. The 34% spontaneous regression rate in patients with rhabdomyomas indicates that follow-up with ECO is sufficient unless there is a hemodynamic problem. Rhabdomyoma may be the first sign of tuberous sclerosis, and although there is no diagnosis of tuberous sclerosis, rhabdomyoma patients should be followed up clinically in this regard. Due to the fact that all patients with multiple rhabdomyomas accompany with tuberous sclerosis, a closer follow-up of these patients may be beneficial. As the most common complication is arrhythmia, rhythm evaluation should be performed in patients with cardiac tumors. Our series also includes a primary cardiac yolk sac tumor which is the sixth case of the literature and it also contributes greatly to the literature in terms of number of patients.