Primary Chest Wall Ewing Sarcoma With Diaphragmatic Invasion in an Adult: En‐Bloc Resection and Polypropylene Mesh Reconstruction

TL;DR

A rare case of adult chest wall Ewing sarcoma involving the diaphragm was treated with surgery and chemotherapy, highlighting the need for combined approaches to manage this aggressive cancer.

Contribution

This case report highlights the surgical and therapeutic management of adult chest wall Ewing sarcoma with diaphragmatic invasion, emphasizing multimodal treatment strategies.

Findings

En-bloc resection of ribs and partial diaphragm was performed with polypropylene mesh reconstruction.

R1 anterior margin necessitated adjuvant systemic therapy and possible postoperative radiotherapy.

Integrated histology, immunophenotype, and molecular testing confirmed Ewing sarcoma diagnosis.

Abstract

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.

Adult CWES/PNET may necessitate combined rib and diaphragmatic resection with prosthetic reconstruction; even after neoadjuvant therapy, an R1 margin should trigger adjuvant chemotherapy and consideration of postoperative radiotherapy to optimize local control.

Figures