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Synovial sarcoma

Orpha number ORPHA3273
Synonym(s) Synovialosarcoma
Prevalence Unknown
Inheritance
  • Sporadic
Age of onset Variable
ICD-10
  • C49.9
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 302851001
  • 63211008

Summary

Synovial sarcoma (SS) is a rare and aggressive soft tissue tumor, which accounts for 7-8 % of all human malignant sarcomas. Although this tumor generally affects adults, about 30% of reported cases occur in children and adolescents. Despite its name, SS does not appear to be of synovial origin, but rather from multipotent stem cells that differentiate into mesenchymal and/or epithelial structures. Clinically, SS appear as deep-seated, painless, slowly growing masses. Most frequently the tumor affects lower and upper extremities, especially in periarticular regions of large joints. The etiology of the disease remains unknown. Almost all SS cells are characterized by the presence of a translocation involving chromosomes X and 18 [t(X;18)(p11.2;q11.2)]. This translocation is specific to SS and constitutes an excellent tool to diagnose this malignancy. The optimal approach to treatment of SS remains undefined. Complete surgical removal of the primary tumor is the mainstay of treatment. Adjuvant radiotherapy appears to be beneficial in the treatment of microscopic residual disease after surgery. The role of chemotherapy needs to be clarified.

Expert reviewer(s)

  • Dr Antonio RUGGIERO

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Detailed information

Clinical practice guidelines
  • DE (2011)
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