Adamantinoma-Like Ewing Sarcoma of the Parotid Gland: A Systematic Review

Adamantinoma-like Ewing sarcoma (ALES) is a rare malignant neoplasm, identified as a type of Ewing sarcoma (ES), characterized by the EWSR1::FLI1 translocation and a complex immunoprofile. Its various clinical, histopathological, and immunohistochemical features, along with a multitude of diseases in the differential diagnosis, make it a complex entity that requires special attention. Treatment involves parotidectomy alone or in combination with adjuvant chemoradiotherapy. A systematic electronic search was conducted through February 2025 in the PubMed and Scopus databases utilizing relevant Medical Subject Headings, without any time constraints. A total of 35 publications were identified using the keywords "adamantinoma-like Ewing sarcoma" AND "parotid". Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a total of 15 articles were included in this systematic review, all of which were case reports. Based on the limited information available, it appears that this tumor frequently demonstrates regression after treatment. However, given the small number of recorded cases, a clear survival rate associated with specific therapies remains uncertain. This highlights the importance of ongoing assessments and follow-ups to establish definitive outcomes for patients.