In most cases, the cause is unknown. Many patients have experienced a trauma (blow/stab) to the tumor area before the diagnosis is made, however it has not been possible to link this to etiology and pathogenesis.
- In rare cases, sarcomas develop in irradiated areas.
- Lymphangiosarcoma can occur in lymphedematous extremities (Stewart-Treves syndrome).
- Genetic disposition for sarcoma development has been reported for Li-Fraumeni syndrome where a genetic mutation in the tumor suppressor gene P53 is of importance.
- Patients with neurofibromatosis (von Recklinghausens disease) have a 10% higher risk for developing soft tissue sarcoma in one of many benign nerve tumors.