Features
• Commonly affects hand and wrist
• Usually flexor surfaces (hand, forearam, knee, lower leg, ankle)
• Proximal type may be seen in pelvis, peritoneum, genital tract
• Ages 20-40
• Male predilection
• Biphasic epithelioid and spindle cells
• Single cells, cords, nests
• Epithelioid round to polygonal cells
• Dense cytoplasm
• Rhabdoid cytoplasmic inclusions may be present
• Plump spindle cells may be admixed
• Nuclei may be bland to anaplastic
• Multinucleation
• Coarse chromatin
• Prominent nucleoli
• Multinucleated giant tumor cells
• Mitosis present, including atypical mitotic figures
• Necrosis present
• Fibrillary stromal fragments
• IHC: positive for vimentin, cytokeratin, EMA, may have CD34, CD99, calretinin, SMA; negative for CD31; loss of INI1
• Zeppa P et al. Diagn Cytopathol. 1999 Dec;21(6):405-8.
• Cardillo M et al. Cancer. 2001 Aug 25;93(4):246-51.