Treatment of soft tissue sarcomas including GIST - Update 2021

Was ist neu? Neoadjuvante und adjuvante Chemotherapie Zur Risikoabschatzung einer perioperativen Chemotherapie kann der Sarculator" eingesetzt werden. Unter Einbeziehung von histologischem Subtyp, Tumorgro ss e, -grading und Patientenalter hilft er, das individuelle Rezidivrisiko zu bestimmen. Palliative Systemtherapie Zunehmende Hinweise aus Subgruppenanalysen prospektiver Studien und retrospektiven Studien sprechen dafur, dass Patienten mit bestimmten Sarkom-Subtypen auch bezuglich des Gesamtuberlebens von subtypspezifischen Kombinationstherapien schon in der ersten Therapielinie profitieren konnen. Die Therapieplanung in der metastasierten Situation sollte nicht nur nach patientenindividuellen Gesichtspunkten, sondern auch Sarkom-Subtyp-spezifisch erfolgen. Gastrointestinaler Stromatumor Mit Avapritinib steht ab sofort eine hochwirksame Therapie fur GIST mit der bislang als therapierefraktar angesehenen PDGFRA-Exon-18-D842V-Mutation zur Verfugung. Ripretinib ist eine neue Therapieoption fur Patienten, die alle derzeit zugelassenen Substanzen bereits erhalten haben. Durch eine 3-jahrige adjuvante Therapie kann bei Patienten mit hohem Rezidivrisiko etwa die Halfte aller Todesfalle in den ersten 10 Jahren Nachbeobachtung vermieden werden. Abstract Soft tissue sarcomas are rare tumors that represent a major challenge due to varying clinical presentations and often interdisciplinary treatment concepts. Gold standard for the treatment of localized resectable soft tissue sarcomas is complete surgical removal. So far, multimodality treatment does not represent a clininal standard. However, several newer analyses and studies suggest that a subgroup of patients seems to derive an overall survival benefit from perioperative chemotherapy. In metastatic soft tissue sarcoma systemic therapy is the treatment of choice. Most active drugs are the anthracyclines and ifosfamide. Combination chemotherapy has improved both response rate and progression-free survival at the costs of increased toxicity in comparison to single agent therapy but without impact on overall survival in first-line therapy. In pretreated patients, treatment options consist of trabectedin, pazopanib, gemcitabine plus docetaxel or DTIC, and eribulin. Recent data have shown that histiotype-specific treatment options including targeted therapy represent a major improvement for several sarcoma subtypes. In GIST, imatinib is the gold standard for patients with advanced or metastatic disease. In imatinib refractory or intolerant patients, sunitinib in an individualized treatment schedule is recommended. Regorafenib has been approved for third-line therapy. Recently, avapritinib has been approved for treatment of patients with the so far resistant D842V mutation in the PDGFRA exon 18. Ripretinib has shown very promising activity in forth and further lines of therapy and is already approved in the US.The use of adjuvant imatinib therapy in patients with completely resected localized GIST with a high risk of recurrence has significantly improved overall survival with a treatment duration of 3 years. These results have now been confirmed with a 10 years follow-up analysis.