• Distinctive neoplastic condition; not strictly a sarcoma
• Children and young adults, often adolescent boys
• Large mass in abdomen or pelvis, accompanied by widespread peritoneal tumor implants
• Other locations include pleura, thorax, scrotum, CNS

• 12 year old boy with stomach tumor (J Korean Surg Soc 2011;80:S80)
• 16 year old girl with pancreatic tumor (J Indian Assoc Pediatr Surg 2011;16:66)
• 25 year old man with lung mass (Lung India 2011;28:287)
• 30 year old man with intra-abdominal tumor (Int J Surg Case Rep 2011;2:293)

Greater omentum

Pancreas

Site unknown

Stomach

• Solid nests of round/oval cells surrounded by cellular desmoplastic stroma
• Also necrosis, cystic degeneration, glandular arrangements, signet ring-like cells, pseudorosette formations, rhabdoid cells, extensive areas of predominantly spindle cell morphology, carcinoid-like differentiation, adenoid cystic-like configuration

Abdominal tumor

Lung tumor

Pancreas

Stomach

Various images of unknown origin

• WT1 (C-19) (also positive in nephroblastomas)

• t(11;22)(p13;q11.2 or q12): WT1-EWS (Am J Surg Pathol 2000;24:830)
• Also t(21;22)(q22;q12): ERG-EWS

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