cardiac rhabdomyosarcoma
Pronunciation: /ˌræbdoʊmaɪoʊsɑrˈkoʊmə/
Rare malignant cardiac tumor arising from striated muscle tissue, most commonly affecting children and young adults.
Full Definition
Cardiac rhabdomyosarcoma is an extremely rare primary malignant cardiac tumor that originates from striated muscle cells within the heart. It represents less than 5% of all primary cardiac tumors and typically affects pediatric and young adult populations. Histologically, the tumor shows pleomorphic cells with eosinophilic cytoplasm, cross-striations, and positive immunostaining for skeletal muscle markers including desmin, myogenin, and MyoD1. The tumor has a predilection for the right ventricle and interventricular septum, often causing outflow tract obstruction. Prognosis is generally poor due to the aggressive nature of the tumor and frequent metastatic spread.
Usage
Usage note: Do not confuse with the benign cardiac rhabdomyoma, which is much more common in children.
In Context
- "Immunohistochemistry confirmed the diagnosis of cardiac rhabdomyosarcoma with strong positivity for MyoD1 and myogenin." — Histopathology report
- "The cardiac rhabdomyosarcoma had extensively infiltrated the right ventricular myocardium and extended into the tricuspid valve apparatus." — Surgical pathology report