Primary cardiac tumors in pediatrics

pp. 270-274

Abstract

Objectives

Our purpose was to communicate the incidence of primary cardiac tumors diagnosed in the Children' s Hospital of Buenos Aires city from March 1979 to December 2000. A total of 14216 clinic reports were reviewed. 

Results 

Intracardiac tumor was diagnosed in 26 patients (p). Eigh­teen patients (out of a total of 26) were male (69%). In 17/26 (65%) the age ofpresentation was under one year. The most frequent reasons for consultation were: murmur in 14/26 p (54%) and arrhythmia 5/26 (19.2%). Only 5/26 p (19%) presented increased cardiac size in the chest X-ray exam. In 16/26 p (61 %) the tumor was multiple and was located in the right ventricle in 61 %; in 14 p it was localized in the left ventricle (53%) and 5 pin atrial chamber (19.2%). Fourteen were classified as rhabdomyomas based on their echocardiographic characteristics. Ten of these were asso­ciated with tuberous sclerosis, 3 fibrous were tumors (11.5%), 4 were myxomas, 1 was a rhabdomyosarcoma and 4 were non-classifiable. Two patients were diagnosed in utero by fetal echocardiography. Global mortality was 15% (4 p): 2 with myxoma, 1 with a rhabdomyoma and 1 with a rhabdomyosarcoma. In 7 p confirmation was obtained by histopathology (3 fibrous tumor and 1 myxoma surgically treated, 1 rhabdomyoma and 1 rhabdomyosarcoma post mortem). Among those with rhabdomyomas 11/14 p re­mained in follow-up for 1 to 6 years (mean follow-up: 3.5 years). All ofthem were asymptomatic and in 3 p a regres­sion of the tumoral mass was observed. 

Conclusions 

Primary cardiac tumors in Pediatrics have low incidence. The usual symptom at presentation is the unspecific mur­mur. Echocardiography is the elective diagnostic method. There was a remarkable predominance of male patients in our population. Rhabdomyoma was the most frequent tu­mor and its multiple presentation was the most prevalent. In half ofthe cases in our series, it was associated with tu­berous sclerosis. It has a benign evolution as well as a ten­dency to regression.