• Rare (<50 cases reported) soft-tissue tumor resembling extraskeletal myxoid chondrosarcoma and chordoma
• Develops next to tendon, synovium, osseous structures in extremities
• Mean age 35 years, range 7-62 years
• Surgical excision usually adequate

• 28 year old man with mass and pain in shoulder (Turk Patoloji Derg 2011;27:173)
• 48 year old woman with mild intermittent lower abdominal distending pain (Rare Tumors 2012;4:e5)
• 60 year old woman with upper arm mass (Clin Orthop Relat Res 2008;466:1251)

• Well circumscribed lobules of large, round and eosinophilic cells, focally resembling physaliferous cells or cartilaginous cells in myxoid to densely hyaline matrix
• Also spindle cells

Presacral tumor

Upper arm

Shoulder mass

• Alcian blue at pH 2.5 (matrix), abolished with hyaluronidase predigestion
• CK 8/18, EMA, S100, vimentin, type 4 collagen around nests of cells

• Smooth muscle actin, GFAP, CK 1/10

• Trisomy 15 (one case), monosomy 1, 16, 17

• Chordoma: many multivacuolated (physaliferouslike) cells; monosomy 3, 4, 10 or 13; type 4 collagen negative (Am J Surg Pathol 1999;23:1059)
• Extraskeletal myxoid chondrosarcoma: t(9;22)+, type 4 collagen negative

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