Features

General

• Commonly affects distal extremities
• Ages 20-40
• Female predilection
• Also called melanoma of soft parts

Architecture

• Cellular smears
• Biphasic with epithelioid and spindle cells
• Nested fascicular architectural pattern
• Single cells and clusters

Cellular features

• Polygonal to spindle cells
• Granular to clear cytoplasm
• Intranuclear cytoplasmic inclusions
• May have melanin rarely

Nuclear features

• Nuclei may be pleomorphic
• Multinucleation with wreath-like nuclei
• Vesicular chromatin
• Prominent nucleoli

Background

• Tigroid background may be seen
• Mitosis and necrosis rare

Ancillary Studies

• IHC: positive for S100, HMB45, Mart1; negative for cytokeratin
• Molecular: t(12;22) EWS/ATF-1

Reference

• Zeppa P et al. Diagn Cytopathol. 1999 Dec;21(6):405-8.
• Cardillo M et al. Cancer. 2001 Aug 25;93(4):246-51.