Rhabdomyosarcoma with TFCP2 Rearrangement

**Semantic type:** Neoplastic Process

**Definition:** An aggressive, high-grade rhabdomyosarcoma characterized by TFCP2 rearrangement that results either in TFCP2-FUS or TFCP2-EWSR1 fusion gene formation. It affects young adults and arises from bone and less frequently soft tissue. It predominantly affects the craniofacial bones. Histologically, it usually shows a biphasic pattern with spindle and epithelioid cell areas. It is associated with poor prognosis.

**Synonyms:** - Epithelioid/Spindle Cell Rhabdomyosarcoma - Epithelioid/Spindle Cell Rhabdomyosarcoma with TFCP2 Rearrangement - TFCP2-RMS