Clinicopathological analysis of primary cardiac rhabdomyoma in 26 fetuses and infants
Zhu Qing
He Chenyu
Shang Jianfeng
Chen Dong
Abstract:Objective To investigate clinicopathological features,diagnosis and differential diagnosis of primary cardiac rhabdomyoma.Methods Twenty-six cases of cardiac rhabdomyoma of fetal autopsy and infant surgical resection were analyzed through general visual observation,hematoxylin-eosin staining and immunohistochemical staining at Department of Pathology,Beijing Anzhen Hospital,Capital Medical University from 2010 to 2017.Results In 24 cases of fetal autopsy,7 cases(29.2%) had single tumor,17 cases(70.8%) had multiple tumors;11 cases(45.8%) had rhabdomyoma in left ventricle,2 cases(8.3%) had rhabdomyoma in right ventricle,11 cases (45.8%) had tumors in both side;2 cases(8.3%) with brain nodules were diagnosed of tuberous sclerosis complex;10 cases(41.7%) had septal tumor.Two samples of surgical resection came from a 5 months old infant and an 1 month old infant;the 5 months old infant had a tumor nodule in pulmonary artery and right ventricular outflow tract.Hematoxylin-eosin staining showed eosinophilic or vacuolar cells,small to medium nucleus with obvious nucleolus;there were spider-like cells but no mitotic figure was observed.Immunohistochemical staining showeddesmin(+),myoglobin(+),vimentin(+),Ki-67(1%),S-100(-),CD31(-),CD34(-),myogenic differentiation 1 (-),myosin(-),smooth muscle actin(-).Conclusions Cardiac rhabdomyoma is common in fetus and infant;it can be diagnosed by echocardiography during gestation period;the complication of tuberous sclerosis complex is rare.Immunohistochemical test and genetic test are useful in diagnosis of cardiac rhabdomyoma.
Keywords:Congenital heart diseaseCardiac rhabdomyomaTuberous sclerosisPathology
Publication Date:2017-01-01
Online Publishing Date:2026-09-14(First online date of this platform, not the publication date of the document)
Pages:4( 1805-1808 )
