Abdominal Wall Extraskeletal Ewing's Sarcoma in an 8-Year-Old Child

Area of Science:

• Pediatric Oncology
• Surgical Oncology
• Medical Imaging

Background:

• Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a diverse group of mesenchymal neoplasms.
• Accurate diagnosis of NRSTSs can be challenging, with Ewing's sarcoma being particularly rare.
• Pediatric abdominal wall Ewing's sarcoma is exceptionally uncommon.

Observation:

• An 8-year-old female presented with a 3-month history of a progressively enlarging left iliac fossa mass.
• Imaging revealed a 4 cm exophytic mass in the left lower abdominal wall, originating from oblique muscles.
• Biopsy and karyotyping confirmed extraskeletal Ewing's sarcoma (EES) of the abdominal wall.

Findings:

• Neoadjuvant chemotherapy (VAEC-IE) was administered to downsize the tumor.
• Surgical resection involved partial removal of abdominal oblique muscles, with the transversalis fascia preserved.
• Histopathology confirmed tumor-free margins post-excision, with minimal response to chemotherapy, necessitating adjuvant radiotherapy (45 Gy).

Implications:

• Extraskeletal Ewing's sarcoma of the abdominal wall requires a multimodal treatment approach.
• Surgery, aiming for wide excision with negative margins, is the cornerstone of management.
• This case demonstrates the efficacy of a combined treatment strategy in achieving long-term disease control for this rare pediatric tumor.