Adamantinoma-like Ewing sarcoma of the salivary glands: a case report and systematic literature review

Abstract

Adamantinoma-like Ewing sarcoma (ALES) of the salivary glands is an exceedingly rare malignancy defined by the t(11,22) EWSR1::FLI1 fusion, with complex epithelial differentiation. To identify features that can allow for better recognition of this disease entity, we reviewed all published reports of molecularly confirmed ALES of the salivary glands and explored epidemiological, clinical, radiological, pathological, and therapeutic characteristics of a population of 21 patients including a single newly reported patient from our group. We searched the English-language literature indexed in PubMed, Medline, Scopus, and Web of Science using the keyword ‘Adamantinoma-like Ewing sarcoma’ published up to June 2022. The median age at diagnosis was 46 years, and a slight female sex predilection was observed. Most tumors originated in the parotid gland (86%) and presented as a painless palpable mass with a median diameter of 3.6 cm. Metastatic dissemination was reported only in one patient (5%), and after a median follow-up of 13 months the 1-year overall survival rate was 92%. Salivary gland ALES were frequently misdiagnosed at presentation (62% of cases) and were pathologically characterized by the presence of highly monomorphic small round blue cells with infiltrative pattern and positive immunostaining for CD99 and high- and low-molecular weight cytokeratins. Epidemiological and clinical features of salivary gland ALES raise questions on the incorporation of this malignancy in the Ewing sarcoma family tumor group.

Adamantinoma-like ewing sarcoma arising in the pancreatic tail: a case report of a rare entity and review of the literature

“…Among these, ALES arising from the salivary glands is the most common. A latest literature summarizes 21 cases with salivary gland ALES [ 9 ]. Median age at diagnosis was 46 years, and ALES shows a male: female ratio of 1:1.6.…”

“…This suggests that most of ALES cases have the low tendency to local and/or metastatic dissemination, which is different from classic Ewing sarcoma (ES). Although the patient in our study was receiving a ES-specific chemotherapy regimen, tolerates it well and remains disease-free by imaging 4 months after diagnosis, the single death recorded in previous study was secondary to the postoperative ES-specific regimens [ 9 ]. Thus, it is necessary to recognize and diagnose this variant of ES from prognosis and treatment point of view.…”