Fetopathology and developmental pathology of the embryo and fetus: Congenital cardiac rhabdomyoma

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+ Congenital malformations of female genitalia

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+ Congenital cardiac rhabdomyoma

+ Congenital heart defects

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Congenital cardiac rhabdomyoma

Introduction

Rhabdomyoma is the most common pediatric tumor arising in the heart. It is commonly associated with tuberous sclerosis.

Clinical signs

bening hamartomatous neoplasm
occurs in fetus and neonate
tend to regress spontaneously after birth
frequent in patients with tuberous sclerosis (50 – 85%)
patiens are usually asymptomatic
complications with multiple large tumors:
- fetal hydrops
- conduction abnormalities
- outflow obstruction
- congestive heart failure
- sudden death

Macroscopic appearance

single or commonly multiple
firm pale mass
arising anywhere within the myocardium

Cardiac rhabdomyoma (74000)

Cardiac rhabdomyoma (74001)

Pictures

Cardiac rhabdomyoma: Heart, rhabdomyoma, Macro, autopsy (74000) Heart, rhabdomyoma, Macro, autopsy (74001)

Histology

Sharply circumscribed but unencapsulated nodule with characteristic spider cells. These are quite large vacuolated cells rich in glykogen with central nucleus and cytoplasmic strands extending to the plasma membrane.

Cardiac rhabdomyoma, spider cells (74038)

Pictures

Cardiac rhabdomyoma: Rhabdomyoma, myocardium, fetus, HE 40x (74038)