Soft tissue limb and trunk sarcomas: diagnosis, treatment and follow-up

This review focuses on epidemiology, aetiology, clinical presentation, diagnosis, management, prognosis and follow-up of soft tissue sarcomas (STS) involving limbs and trunk. Any patient with a suspected STS should be referred to a specialized sarcoma centre and managed by a multidisciplinary group. The standard treatment is surgical excision followed by adjuvant radiotherapy (RT). Radiotherapy is recommended in patients with intermediate-or high-grade tumors, >5 cm of diameter or <5 cm. RT may be indicated in low grade, deep and large-size STS and/or in absence of adequate margins, after discussion within a multidisciplinary group. Neoadjuvant radiotherapy and chemotherapy should be taken into consideration for patients with borderline resectable tumors. In selected cases, amputation may be the only curative option. Isolated limb perfusion is a pre-operative treatment that may allow for amputation to be avoided. Adjuvant chemotherapy should be considered only in selected cases. Regular follow-up with clinical examination, ultrasound (US) or magnetic resonance imaging (MRI) to exclude local recurrences and chest-X-ray or chest computed tomography (CT) to exclude metastatic disease is recommended. For metastatic disease, doxorubicin is the first-line standard therapy. Second-line agents include trabectedin, ifosfamide, dacarbazine and the combination of gemcitabine-plus-docetaxel. Surgical resection of local recurrences or lung metastases should be evaluated in selected cases.