A RARE CASE OF EXTRASKELETAL EWING SARCOMA TUMOR DEVELOPING IN AN UNCOMMON LOCATION—FLOOR OF THE MOUTH

Abstract

<h3>Introduction</h3> Ewing sarcomas (ESs) are uncommon malignant neoplasms that usually arise in long bones in young adults. They represent a family of high-grade tumors characterized by small round cells and include ES of bone and peripheral primitive neuroectodermal tumors. ES is poorly differentiated and may arise in bones or soft tissues; primitive neuroectodermal tumors tend to arise in soft tissues. Extraskeletal ES (EES) is very rare, particularly in the head and neck. Neoadjuvant chemotherapy improves survival; however, surgery remains an important treatment option. <h3>Case Description</h3> A 43-year-old Caucasian man presented with an otherwise asymptomatic slow-growing lump in the right submandibular/sublingual area. Magnetic resonance imaging confirmed the presence of a large mass above the mylohyoid, with suspicious characteristics. A fine-needle aspiration biopsy of the mass was suggestive of EES. The case was discussed in the head and neck and regional sarcoma MDTs. A larger sample was requested for further analysis, which confirmed the diagnosis. The patient started treatment with neoadjuvant chemotherapy and might require surgery at a later date. <h3>Discussion</h3> This case highlights the challenges faced when dealing with rare tumors. When is it reasonable to suspect malignancy in the absence of sinister symptoms? What technique offers appropriate diagnostic samples without compromising future resection? What tests allow prompt diagnosis? What management regime is indicated when specific data are lacking? We believe that such cases must be reported, to add to the knowledge of the scientific community, so that in the future optimal management can be offered in these challenging circumstances.