Epidemiology
An uncommon low-grade sarcoma, typically found in the hands, feet, and distal parts of the limbs in adults. In the hands and feet it can be confused with chronic inflammatory lesions, especially if it grows slowly and causes little pain.
Basic features
It may look inflammatory or cyst-like and grow slowly, which can delay the diagnosis if it is not biopsied. It often shows alterations of the TGFBR3 and OGA (formerly MGEA5) genes, which help to confirm it.
Usual treatment
Wide or carefully planned resection aims to achieve clear margins without sacrificing function unnecessarily.
Outcomes and prognosis
Local recurrence is the main concern and is common; spread to distant sites (metastasis) is uncommon but possible.
