Adamantinoma-Like Ewing Sarcoma

**Semantic type:** Neoplastic Process

**Definition:** An exceedingly rare variant of Ewing sarcoma with epithelial differentiation that resembles carcinoma. Morphologically, it is characterized by the presence of nests of basaloid cells, tumor cell palisading, myxoid or hyalinized stroma, focal keratin pearl formation, and high-grade features with minimal pleomorphism. It usually affects the head and neck region.

**Synonyms:** - ALES