成人における横隔膜浸潤を伴う原発性胸壁ユーイング肉腫：一括切除およびポリプロピレンメッシュ再建

Chest-wall Ewing sarcoma (CWES) is uncommon in adults and often abuts vital thoracic structures, making R0 resection challenging despite gains with multimodal therapy. A 46-year-old Arab male presented with year-long right-sided chest pain, weight loss, and a firm mass over the lower right ribs. CXR showed a lateral pleural-based opacity; computed tomography (CT) demonstrated a chest-wall lesion involving right ribs 9-11 with diaphragmatic contact. CT-guided core biopsy confirmed Ewing sarcoma. After 4 cycles of neoadjuvant multi-agent chemotherapy, he underwent composite resection of ribs 9-11 with partial diaphragmatic excision and polypropylene (Prolene) mesh reconstruction stabilized by two wires. Pathology revealed a small round blue cell tumor with broad necrosis; margins were negative laterally, posteriorly, and at cartilaginous/diaphragmatic edges, but the anterior margin was positive (R1). IHC showed diffuse membranous CD99, nuclear FLI1, diffuse vimentin, and focal NSE; molecular confirmation of EWSR1 rearrangement was recommended. This adult CWES with diaphragmatic invasion illustrates the need for multimodal care: induction chemotherapy to address micrometastases and facilitate resection, followed by aggressive local surgery and prosthetic reconstruction. The R1 anterior margin justifies adjuvant systemic therapy with consideration of postoperative radiotherapy to optimize local control. Definitive diagnosis relies on integrated histology, immunophenotype, and molecular testing. Adult CWES may require combined rib and diaphragmatic resection with prosthetic repair. Margin-negative surgery remains pivotal; when margins are positive, tailored adjuvant therapy is essential to mitigate local and micrometastatic risks.