Synovial Sarcoma:

Definition / General

Usually a deep seated mass present for years around large joints (80% in
knee and ankle) in young adults (age 20-40), 60% male; only 10% actually
involve the joint
Represent 10% of adult soft tissue tumors
5 year survival is 50 - 70%; 10 year survival 40%; recurs locally, 10 - 15%
metastasize to lung and pleura, bone, regional nodes
Rarely radiation associated (Mod Pathol 2002;15:998)
Minute (< 1 cm) tumors of hands and feet: 2/3 female, median age 29
years, 2/3 monophasic, 40% have microcalcifications; EMA+, keratin+;
have clinically favorable course if completely excised
Poor prognostic factors: high histologic grade (based on MIB1 index
and necrosis, (Hum Pathol 2001;32:257), SYT-SSX1 vs. SYT-SSX2
gene fusion

Micro Description

Biphasic, monophasic or undifferentiated

Biphasic have spindle cells resembling synoviocytes and plump epithelial
cells forming glands / cords
Monophasic lack the epithelial cells
Spindle cells are arranged in plump fascicles with hyalinization and distinct
lobulation accompanied by mast cells, occasional osseous or cartilaginous
metaplasia, focal whorling
May have hemangiopericytomatous vascular pattern
Poorly differentiated histology predicts poor outcome

___staghorn vessels___

biphasic tumor

POSITIVE:Cytokeratin 7, 8 / 18, 19 [both components], AE1 / AE3 (70%

of monophasic fibrous, 46% of poorly differentiated), EMA (epithelial areas,
100% of monophasic fibrous, 92% of poorly differentiated), CD99 / O13
(Ewings / PNET marker, 90-100% of monophasic fibrous or poorly
differentiated), vimentin (spindle cells), CEA, BCL2 (both components,
90% of monophasic fibrous or poorly differentiated) ( Am J Surg Pathol
2002;26:1434)
Also TLE1 (97%, Am J Surg Pathol 2007;31:240), CD57 (neural marker in
72%), E-cadherin (50%), S100 (30-40%), c-kit (children, Am J Surg Pathol
2002;26:486), nuclear beta-catenin
Mucin in spindle cell areas, PAS positive in epithelium, reticulin highlights
biphasic pattern

Negative Stains

CD34 (94% monophasic fibrous, 100% poorly differentiated), desmin (98%

monophasic fibrous, 100% poorly differentiated), myogenin (Am J Surg
Pathol 2001;25:1150), h-caldesmon, CD141, WT1, FLI-1

Note: normal synovium is cytokeratin negative

Differential Diagnosis

Fibrous variant resembles other sarcomas

Metastatic adenocarcinoma: if primarily epithelial component
MPNST: usually negative for CK7 and CK19;negative for SYT-SSX fusion
products