Ewing’s Sarcoma of the Mandible with Extensive Bony Metastasis in a Young Male: A Case Report

Abstract

Ewing’s Sarcoma Family of Tumours (ESFTs) comprises locally aggressive, undifferentiated small round blue-cell tumours of neuroectodermal origin, predominantly seen in adolescents. These tumours arise from non random chromosomal translocations involving the Ewing’s Sarcoma (EWS) gene. ESFTs in the mandible are uncommon. A 14-year-old male patient presented with a painless swelling in the right jaw. Plain radiographs, ultrasonography, contrast-enhanced Computed Tomography (CT), and Magnetic Resonance Imaging (MRI) revealed a large, partially well-defined, highly vascular soft tissue mass in the right mandibular region. It measured approximately 80×82×75 mm (anteroposterior×transverse×craniocaudal), with both solid and cystic components. The mass was destroying the underlying right temporomandibular joint, head, ramus and posterior body of the right hemimandible, including extensive sunray-type bony spiculations. There were multiple lytic lesions in the skull and facial bones, as well as, altered marrow signal intensity in the calvarium, bilateral humeri and clavicles, and cervical and thoracic vertebrae, indicating a locally aggressive tumour with extensive metastasis into the bones and marrow. The diagnosis was confirmed by histopathology, which showed fibrocollagenous tissue infiltrated by sheets of small round cells with scanty eosinophilic cytoplasm and prominent nuclei. Management typically involves a combination of chemotherapy, radiation and surgery. However, metastatic Ewing’s Sarcoma has a poor prognosis.

Keywords

• head and neck radiology
• jaw swelling
• mandibular sarcoma
• primitive neuroendocrine tumour