Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up

Author(s): The ESMO/European Sarcoma Network Working Group


Extraskeletal Ewing sarcoma is covered by other ESMO Guidelines: in general, the same principles for these tumours in children apply to adults. This is also the case for embryonal and alveolar rhabdomyosarcoma, which are exceedingly rare in adults. On the other hand, pleomorphic rhabdomyosarcoma is viewed as a high-grade adult-type soft tissue sarcoma. Gastrointestinal stromal tumours are covered by the dedicated ESMO Clinical Practice Guidelines. Kaposi's sarcoma is excluded.


Adult soft tissue and visceral sarcomas (excluding gastrointestinal stromal tumour) are rare tumours, with an estimated incidence averaging 4–5/100 000/year in Europe 

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