/rhabdomyosarcoma with mixed embryonal and alveolar features

rhabdomyosarcoma with mixed embryonal and alveolar features

Rare Disease

MONDO:0002863

Also Known As

mixed alveolar rhabdomyosarcomamixed embryonal rhabdomyosarcoma and alveolar rhabdomyosarcomamixed type alveolar rhabdomyosarcomamixed type rhabdomyosarcomarhabdomyosarcoma with mixed embryonal and alveolar features

Definition

A rhabdomyosarcoma composed of embryonic and alveolar components. It is characterized by the presence of spindle cells with myoblastic differentiation, a myxoid stroma, and fibrous septa. These tumors were previously considered variants of alveolar rhabdomyosarcoma. The lack of PAX3-FOXO1 fusions in most of these tumors suggests that are biologically and clinically related to embryonal rhabdomyosarcoma.