• First described in 2001 (Mod Pathol 2001;14:563)
• Tumor masses involve serosa of ileum, uterus or pelvic cavity
• Nodal and metastatic disease present
• One patient had tuberous sclerosis

• 16 year old girl with abdominopelvic tumor exhibiting extensive necrosis, nodal metastases and tissue invasion (Kaohsiung J Med Sci 2005;21:277)

• Sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm, moderately pleomorphic nuclei, delicate vasculature (resembles clear cell carcinoma)
• Also focal coagulative necrosis and occasional mitotic figures
• Angiolymphatic invasion present
• May have intracytoplasmic brown pigment
• No spindle cells, smooth muscle or fat

Large neoplastic cells

Abundant cytoplasm

Nodal metastasis

Large renal tumor

• HMB45, MART1 (50%)

• Keratin, EMA, S100, vimentin, muscle specific actin, desmin, chromogranin A

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