9040/3 Synovial sarcoma, NOS


Synonyms

Synovioma, NOS
Synovioma, malignant

Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It mainly affects young adults, more commonly males. The vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Histologically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur and may metastasize to the lungs, bones, and lymph nodes 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Renal synovial sarcoma (SS) is a spindle cell neoplasm that rarely displays epithelial differentiation. Most tumours are solid, but can contain areas of haemorrhage, necrosis and cyst formation
2
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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Esophagus
ICD-O-3 topography code: C15
ICD10: C15
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes
3
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Isolated cases of biphasic synovial sarcomas have been reported in the upper oesophagus. These have mostly occurred in children and young adults. Grossly, they formed polypoid intraluminal masses, and were histologically similar to synovial sarcomas in soft tissue with glandular elements that were positive for keratin and epithelial membrane antigen (EMA)
4
Click to access Pubmed
Billings SD, Meisner LF, Cummings OW, Tejada E (2000)
Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization.
Mod Pathol 13: 68-76



5
Click to access Pubmed
Bloch MJ, Iozzo RV, Edmunds LH, Brooks JJ (1987)
Polypoid synovial sarcoma of the esophagus.
Gastroenterology 92: 229-33



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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes
6
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Only a small number of primary gastric synovial sarcomas have been reported
7
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Thymus
ICD-O-3 topography code: C37
ICD10: C37
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.



Bronchus and lung
ICD-O-3 topography code: C34
ICD10: C34
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.



Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

A malignant mesenchymal neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.