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Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of Ewing sarcoma that is defined by complex epithelial differentiation, including expression of cytokeratin and p40 and frequent keratin pearl formation. In recent years, ALES has been increasingly recognized in the head and neck, where it can mimic a wide range of small round blue cell tumors and basaloid carcinomas. However, there has been persistent controversy regarding whether ALES is best classified and managed as a sarcoma or carcinoma. This review summarizes the characteristic clinical, pathologic, immunophenotypic, and molecular features of ALES with an emphasis on differential diagnosis and tumor classification.

Soft Tissue Special Issue: Adamantinoma-Like Ewing Sarcoma of the Head and Neck: A Practical Review of a Challenging Emerging Entity