vitalwiki

Synovial sarcoma

ORPHA:3273· ICD-10 C49.9

Definition

A rare soft tissue sarcoma characterized by palpable, slow-growing, deep-seated swellings, with or without pain or tenderness, affecting mostly children and young adults. Patients may have pain or joint contractures that precede swelling. Contrary to its name, lesions do not originate from intra-articular synovium, but from primitive mesenchymal cells. Majority of the lesions arise in the extremities, predominantly in the lower extremity, however can also occur in the head and neck region, trunk, lungs and pleura. Metastasis to the lungs, bone and regional lymph nodes can be observed. Increasing size, age and tumor grade are reported as negative predictive factors for both local disease recurrence and metastasis.

Prevalence
Unknown
Inheritance
Not applicable
Age of onset
All ages