Skip to
  1. Homepage
  2. Rare diseases
  3. Search
Simple search

Simple search

(*) mandatory field


Other search option(s)

Synovial sarcoma

Synonym(s) Synovialosarcoma
Prevalence Unknown
Inheritance Not applicable
Age of onset All ages
  • C49.9
  • C0039101
MeSH -
  • 10042863


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

(*) Required fields.

Attention: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Detailed information

Clinical practice guidelines
  • DE (2011)
  • EN (2010)
Get Acrobat Reader
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.