Al-Bitar Ahmad, Sandouk Mahdi
Faculty of Medicine, Damascus University, Damascus, Syrian Arab Republic.
Faculty of Dentistry, Damascus University, Damascus, Syrian Arab Republic.
Case Rep Oncol. 2025 Mar 25;18(1):515-522. doi: 10.1159/000545490. eCollection 2025 Jan-Dec.
Ewing sarcoma (ES) is an aggressive primary bone cancer, primarily affecting children and adolescents, accounting for 5% of cancers in this demographic. ES most commonly affects males during adolescence. It primarily targets flat and long bones such as the pelvis, femur, tibia, and fibula, and less frequently, the mandible and maxilla.
This case report details a 14-year-old Arab female with maxillary ES, highlighting diagnostic and therapeutic challenges. The patient presented with jaw pain and swelling, initially evaluated via radiography and biopsy, revealing classic histopathological features (small round blue cells, Homer-Wright rosettes) and immunohistochemical markers (CD99, NKX2.2, FLI1 positivity). Imaging identified a localized maxillary mass (T4a N0 M0). Multimodal therapy included neoadjuvant chemotherapy (VDC/IE regimen), en bloc resection, adjuvant chemotherapy, and dental rehabilitation.
Craniofacial ES often mimics benign odontogenic lesions, delaying diagnosis; this case achieved timely intervention within 7 months. Unlike axial ES, jaw tumors require balancing oncologic control with functional preservation, emphasizing conservative surgery and multidisciplinary rehabilitation. The patient's outcome aligns with studies showing superior survival for craniofacial ES (82% 5-year survival) compared to pelvic sites.
尤因肉瘤（ES）是一种侵袭性原发性骨癌，主要影响儿童和青少年，占该人群癌症的5%。ES在青春期最常影响男性。它主要侵犯扁平骨和长骨，如骨盆、股骨、胫骨和腓骨，较少侵犯下颌骨和上颌骨。
本病例报告详细介绍了一名患有上颌骨ES的14岁阿拉伯女性，突出了诊断和治疗方面的挑战。患者表现为颌部疼痛和肿胀，最初通过X线摄影和活检进行评估，显示出典型的组织病理学特征（小圆蓝细胞、霍纳-赖特玫瑰花结）和免疫组化标志物（CD99、NKX2.2、FLI1阳性）。影像学检查发现一个局限性上颌骨肿块（T4a N0 M0）。多模式治疗包括新辅助化疗（VDC/IE方案）、整块切除、辅助化疗和牙齿修复。
颅面部ES常模仿良性牙源性病变，导致诊断延迟；本病例在7个月内实现了及时干预。与轴位ES不同，颌骨肿瘤需要在肿瘤控制和功能保留之间取得平衡，强调保守手术和多学科康复。患者的预后与研究结果一致，显示颅面部ES的生存率（5年生存率82%）高于骨盆部位。
