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Etiology of soft tissue sarcoma in extremities and trunk wall

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.

Risk factors

  • 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.

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