• Kidney, soft tissue, other sites
• Usually infants/children
• Probably represents emergence of an aggressive phenotype of various tumors (epithelioid sarcoma, intraabdominal desmoplastic round cell tumor, rhabdomyosarcoma, melanoma, carcinoma)
• Early metastases to lung, liver, lymph nodes
• Very aggressive, poor response to therapy, usually fatal

• 1.5 year old girl with extrarenal tumor (Indian J Pathol Microbiol 2011;54:819)
• 25 year old woman with vulvar mass (Case Rep Med 2011;2011:162709)
• 36 year old man with lung tumor (Case Rep Pulmonol 2011;2011:323584)

• Solid sheets of large cells with deep eosinophilic cytoplasm, possible laterally displaced nucleus, prominent nucleoli
• Myxoid, hyalinized, pseudoalveolar areas

Vulvar tumor (loss of INI1, ER+)

Lung tumor

Abdominal mass

• Vimentin, keratin, EMA

• S100, muscle markers; loss of INI1

• Prominent intermediate filaments

• Characterized by genetic loss of SMARCB1 (SNF5, INI-1), a component of the SWI/SNF chromatin remodeling complex (Lab Invest 2010;90:724)

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