[Individualised Multimodal Therapy of Retroperitoneal Soft Tissue Sarcomas]

Retroperitoneal soft tissue sarcomas make up a diverse group of malignant tumours of mesenchymal origin with diverse histo- and molecular pathology. Liposarcoma and leiomyosarcoma are the predominant subentities - followed by the substantially less frequent solitary fibrous tumors, malignant peripheral nerve-sheath tumors and unclassified pleomorphic sarcomas. The biological behaviour of retroperitoneal soft tissue sarcomas is highly variable, depending on the histopathological subtype. A differentiated therapeutic approach is therefore essential. There is only marginal therapeutic evidence that multimodal therapies improve patients' outcomes, yet neoadjuvant and adjuvant strategies should be discussed, depending on tumour topography, histopathology and patient-specific factors. There has been intense debate in the last 10 years about the appropriate surgical strategy. The core issue is whether long-term follow-up is improved after more radical, compartmental resection that includes adjacent organs and tissues that appear not to be infiltrated, as judged by clinical and radiological evidence. This procedure should then be generally recommended as standard of care. The available data appears to answer this question - nonetheless a differentiated approach is mandatory. Therefore entities exhibiting low local recurrence rates and/or more benign biology should be resected with the aim of organ preservation, while tumours with high local recurrence rates and unfavourable biological behaviour should undergo more radical, compartimental resection. A balanced approach should be considered in entities with high metachronic systemic dissemination rates.